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Craniocervical instability

2019-06-06T18:24:40Z

Jeff:/* Symptoms */

'''Craniocervical instability''' (CCI) is a pathological condition of increased mobility at the craniocervical junction, the area where the skull meets the spine. In CCI the ligamentous connections of the craniocervical junction can be stretched, weakened or ruptured.<ref name=":2">{{Cite journal|last=Henderson|first=Fraser C.|last2=Austin|first2=Claudiu|last3=Benzel|first3=Edward|last4=Bolognese|first4=Paolo|last5=Ellenbogen|first5=Richard|last6=Francomano|first6=Clair A.|last7=Ireton|first7=Candace|last8=Klinge|first8=Petra|last9=Koby|first9=Myles|date=2017|title=Neurological and spinal manifestations of the Ehlers–Danlos syndromes|url=https://onlinelibrary.wiley.com/doi/abs/10.1002/ajmg.c.31549|journal=American Journal of Medical Genetics Part C: Seminars in Medical Genetics|language=en|volume=175|issue=1|pages=195–211|doi=10.1002/ajmg.c.31549|issn=1552-4876}}</ref> This can lead to compression of the [[Brainstem compression|brainstem]], upper [[spinal cord]], or [[cerebellum]] and result in myelopathy, disabling neck pain and a range of other symptoms.<ref name=":11">{{Cite journal|last=Choi|first=Sung Ho|last2=Lee|first2=Sang Gu|last3=Park|first3=Chan Woo|last4=Kim|first4=Woo Kyung|last5=Yoo|first5=Chan Jong|last6=Son|first6=Seong|date=2013-4|title=Surgical Outcomes and Complications after Occipito-Cervical Fusion Using the Screw-Rod System in Craniocervical Instability|url=https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3698232/|journal=Journal of Korean Neurosurgical Society|volume=53|issue=4|pages=223–227|doi=10.3340/jkns.2013.53.4.223|issn=2005-3711|pmc=PMCPMC3698232|pmid=23826478}}</ref>

CCI usually develops as a result of physical trauma such as a car accident, an inflammation disease such as rheumatoid arthritis or a congenital disorder such as Down syndrome.<ref name=":10">{{Cite journal|last=Ashafai|first=Nabeel S.|last2=Visocchi|first2=Massimiliano|last3=Wąsik|first3=Norbert|date=2019|title=Occipitocervical Fusion: An Updated Review|url=https://www.ncbi.nlm.nih.gov/pubmed/30610329|journal=Acta Neurochirurgica. Supplement|volume=125|pages=247–252|doi=10.1007/978-3-319-62515-7_35|issn=0065-1419|pmid=30610329}}</ref> More recently, physicians have recognized an increased prevalence of CCI in patients with hereditary disorders of connective tissue such as Ehlers Danlos Syndromes (EDS).<ref name=":9">{{Cite web|url=https://www.researchgate.net/publication/302923107_Cranio-cervical_Instability_in_Patients_with_Hypermobility_Connective_Disorders|title=(PDF) Cranio-cervical Instability in Patients with Hypermobility Connective Disorders|website=ResearchGate|language=en|access-date=2019-06-03}}</ref> There have also been anecdotal reports of patients with myalgic encephalomyelitis/chronic fatigue sydrome (ME/CFS) who were diagnosed with CCI <ref>{{Cite web|url=https://forums.phoenixrising.me/index.php?threads/have-you-ruled-out-chiari-as-a-cause-of-your-cfs.56908/|title=Have you ruled out Chiari as a cause of your CFS|last=|first=|date=|website=Phoenix Rising|archive-url=|archive-date=|dead-url=|access-date=}}</ref><ref>{{Cite web|url=https://rsci.app.link/3LpJOxcTaX?_p=f3542c5bfc29c2616780177b27|website=rsci.app.link|access-date=2019-06-01}}</ref>, although no scientific publication on this subject exists.
== Symptoms ==
Characteristic symptoms of craniocervical instability include headache, neck pain and neurological abnormalities such as numbness, motor weakness, dizziness, and gait instability. <ref>{{Cite journal|last=Bobinski|first=Lukas|last2=Levivier|first2=Marc|last3=Duff|first3=John M.|date=2015-2|title=Occipitoaxial spinal interarticular stabilization with vertebral artery preservation for atlantal lateral mass failure|url=https://www.ncbi.nlm.nih.gov/pubmed/25415481|journal=Journal of Neurosurgery. Spine|volume=22|issue=2|pages=134–138|doi=10.3171/2014.10.SPINE14131|issn=1547-5646|pmid=25415481}}</ref><ref>{{Cite journal|last=O'Brien|first=Michael F.|last2=Casey|first2=Adrian T. H.|last3=Crockard|first3=Alan|last4=Pringle|first4=Jean|last5=Stevens|first5=John M.|date=2002-10-15|title=Histology of the craniocervical junction in chronic rheumatoid arthritis: a clinicopathologic analysis of 33 operative cases|url=https://www.ncbi.nlm.nih.gov/pubmed/12394902|journal=Spine|volume=27|issue=20|pages=2245–2254|doi=10.1097/01.BRS.0000029252.98053.43|issn=1528-1159|pmid=12394902}}</ref><ref>{{Cite journal|last=Young|first=Richard M.|last2=Sherman|first2=Jonathan H.|last3=Wind|first3=Joshua J.|last4=Litvack|first4=Zachary|last5=O'Brien|first5=Joseph|date=2014-8|title=Treatment of craniocervical instability using a posterior-only approach: report of 3 cases|url=https://www.ncbi.nlm.nih.gov/pubmed/24785968|journal=Journal of Neurosurgery. Spine|volume=21|issue=2|pages=239–248|doi=10.3171/2014.3.SPINE13684|issn=1547-5646|pmid=24785968}}</ref><ref>{{Cite journal|last=Botelho|first=Ricardo V.|last2=Neto|first2=Eliseu B.|last3=Patriota|first3=Gustavo C.|last4=Daniel|first4=Jefferson W.|last5=Dumont|first5=Paulo A. S.|last6=Rotta|first6=José M.|date=2007-10|title=Basilar invagination: craniocervical instability treated with cervical traction and occipitocervical fixation. Case report|url=https://www.ncbi.nlm.nih.gov/pubmed/17933321|journal=Journal of Neurosurgery. Spine|volume=7|issue=4|pages=444–449|doi=10.3171/SPI-07/10/444|issn=1547-5654|pmid=17933321}}</ref><ref>{{Cite journal|last=Henderson|first=Fraser C.|last2=Henderson|first2=Fraser C.|last3=Wilson|first3=William A.|last4=Mark|first4=Alexander S.|last5=Koby|first5=Myles|date=2018-1|title=Utility of the clivo-axial angle in assessing brainstem deformity: pilot study and literature review|url=https://www.ncbi.nlm.nih.gov/pubmed/28258417|journal=Neurosurgical Review|volume=41|issue=1|pages=149–163|doi=10.1007/s10143-017-0830-3|issn=1437-2320|pmc=PMCPMC5748419|pmid=28258417}}</ref> Patients frequently describe pain at the lower back of the head and the feeling that their head is too heavy for their neck to support (“bobble-head”).<ref name=":9" /> No particular symptom is mandatory for a diagnosis of CCI.

Other symptoms of CCI include:
* [[Muscle weakness]]<ref name=":0">{{Cite journal|last=Henderson|first=Fraser C.|last2=Francomano|first2=C. A.|last3=Koby|first3=M.|last4=Tuchman|first4=K.|last5=Adcock|first5=J.|last6=Patel|first6=S.|date=2019-01-09|title=Cervical medullary syndrome secondary to craniocervical instability and ventral brainstem compression in hereditary hypermobility connective tissue disorders: 5-year follow-up after craniocervical reduction, fusion, and stabilization|url=https://doi.org/10.1007/s10143-018-01070-4|journal=Neurosurgical Review|language=en|doi=10.1007/s10143-018-01070-4|issn=1437-2320}}</ref>, [[numbness]]<ref name=":1">{{Cite journal|last=Francomano|first=Clair A.|last2=McDonnell|first2=Nazli B.|last3=Nishikawa|first3=Misao|last4=Bolognese|first4=Paolo A.|last5=Milhorat|first5=Thomas H.|date=2007-12-01|title=Syndrome of occipitoatlantoaxial hypermobility, cranial settling, and Chiari malformation Type I in patients with hereditary disorders of connective tissue|url=https://thejns.org/view/journals/j-neurosurg-spine/7/6/article-p601.xml|journal=Journal of Neurosurgery: Spine|language=en-US|volume=7|issue=6|pages=601–609|doi=10.3171/SPI-07/12/601}}</ref><ref name=":2" /> [[paralysis]], parasthesias<ref name=":0" /><ref name=":4">{{Cite web|url=https://search.proquest.com/openview/34b3b18a8854c04ffa0fc50273d68313/1?pq-origsite=gscholar&cbl=47886|title=MRI video diagnosis and surgical therapy of soft tissue trauma to the craniocervical junction - ProQuest|website=search.proquest.com|language=en|access-date=2019-06-01}}</ref><ref name=":5">{{Cite journal|last=Rebbeck|first=Trudy|last2=Liebert|first2=Ann|date=2014-12-01|title=Clinical management of cranio-vertebral instability after whiplash, when guidelines should be adapted: A case report|url=http://www.sciencedirect.com/science/article/pii/S1356689X14000101|journal=Manual Therapy|volume=19|issue=6|pages=618–621|doi=10.1016/j.math.2014.01.009|issn=1356-689X}}</ref><ref name=":6">{{Cite journal|last=Mathers|first=K. Sean|last2=Schneider|first2=Michael|last3=Timko|first3=Michael|date=2011-06|title=Occult Hypermobility of the Craniocervical Junction: A Case Report and Review|url=https://www.jospt.org/doi/full/10.2519/jospt.2011.3305|journal=Journal of Orthopaedic & Sports Physical Therapy|language=en|volume=41|issue=6|pages=444–457|doi=10.2519/jospt.2011.3305|issn=0190-6011}}</ref>
* Poor proprioception<ref name=":7">{{Cite journal|last=Bergholm|first=Ulla|last2=Johansson|first2=Bengt H.|last3=Johansson|first3=Hakan|date=2004-01-01|title=New Diagnostic Tools Can Contribute to Better Treatment of Patients with Chronic Whiplash Disorders|url=https://doi.org/10.3109/J180v03n02_02|journal=Journal of Whiplash & Related Disorders|volume=3|issue=2|pages=5–19|doi=10.3109/J180v03n02_02|issn=1533-2888}}</ref>, impaired [[coordination]], gait changes<ref name=":0" /><ref name=":7" />
* [[Dizziness|Dizzinesss]], [[vertigo]]<ref name=":0" /><ref name=":2" /><ref name=":7" /><ref name=":4" /><ref name=":6" /><ref name=":5" /> syncope<ref name=":0" /><ref name=":1" /><ref name=":2" /><ref name=":7" />, nausea<ref name=":1" /><ref name=":2" />
* Neck stiffness, torticollis<ref name=":7" /><ref>{{Cite journal|last=Ghanem|first=Ismat|last2=El Hage|first2=Samer|last3=Rachkidi|first3=Rami|last4=Kharrat|first4=Khalil|last5=Dagher|first5=Fernand|last6=Kreichati|first6=Gabi|date=2008-03-01|title=Pediatric cervical spine instability|url=https://online.boneandjoint.org.uk/doi/full/10.1007/s11832-008-0092-2|journal=Journal of Children's Orthopaedics|volume=2|issue=2|pages=71–84|doi=10.1007/s11832-008-0092-2|issn=1863-2521|pmc=PMC2656787|pmid=19308585}}</ref> posterior scalp irritation<ref name=":7" />, facial pain, headache behind the eyes
* Apnea<ref>{{Cite journal|last=Janjua|first=M. Burhan|last2=Hwang|first2=Steven W.|last3=Samdani|first3=Amer F.|last4=Pahys|first4=Joshua M.|last5=Baaj|first5=Ali A.|last6=Härtl|first6=Roger|last7=Greenfield|first7=Jeffrey P.|date=2019-01-01|title=Instrumented arthrodesis for non-traumatic craniocervical instability in very young children|url=https://doi.org/10.1007/s00381-018-3876-9|journal=Child's Nervous System|language=en|volume=35|issue=1|pages=97–106|doi=10.1007/s00381-018-3876-9|issn=1433-0350}}</ref><ref name=":1" /><ref name=":3">{{Cite journal|last=Henderson|first=Fraser C.|last2=Austin|first2=Claudiu|last3=Benzel|first3=Edward|last4=Bolognese|first4=Paolo|last5=Ellenbogen|first5=Richard|last6=Francomano|first6=Clair A.|last7=Ireton|first7=Candace|last8=Klinge|first8=Petra|last9=Koby|first9=Myles|date=2017|title=Neurological and spinal manifestations of the Ehlers–Danlos syndromes|url=https://onlinelibrary.wiley.com/doi/abs/10.1002/ajmg.c.31549|journal=American Journal of Medical Genetics Part C: Seminars in Medical Genetics|language=en|volume=175|issue=1|pages=195–211|doi=10.1002/ajmg.c.31549|issn=1552-4876}}</ref>, dyspnea (shortness of breath)<ref name=":0" /><ref name=":1" /> and dysphagia (difficulty swallowing)<ref name=":1" /><ref name=":2" />
* Visual disturbances<ref name=":4" /> downward [[nystagmus]] (irregular eye movements)<ref name=":1" /><ref name=":7" /> tinnitus<ref>{{Cite journal|last=Montazem|first=Abbas|author-link=|author-link2=|author-link3=|author-link4=|author-link5=|date=2000|title=Secondary tinnitus as a symptom of instability in the upper cervical spine: Operative management|url=https://pdfs.semanticscholar.org/21c4/85984a6ebe07efed38cf82a2f7a49b2a644e.pdf|journal=International Tinnitus Journal|volume=|issue=|pages=|quote=|via=}}</ref><ref name=":4" />
* Fatigue<ref name=":0" /><ref>{{Cite journal|last=Henderson|first=Fraser C.|last2=Henderson|first2=Fraser C.|last3=Wilson|first3=William A.|last4=Mark|first4=Alexander S.|last5=Koby|first5=Myles|date=2018-01-01|title=Utility of the clivo-axial angle in assessing brainstem deformity: pilot study and literature review|url=https://doi.org/10.1007/s10143-017-0830-3|journal=Neurosurgical Review|language=en|volume=41|issue=1|pages=149–163|doi=10.1007/s10143-017-0830-3|issn=1437-2320|pmc=PMC5748419|pmid=28258417}}</ref><ref>{{Cite journal|last=Henderson|first=Fraser C.|last2=Wilson|first2=William A.|last3=Mott|first3=Stephen|last4=Mark|first4=Alexander|last5=Schmidt|first5=Kristi|last6=Berry|first6=Joel K.|last7=Vaccaro|first7=Alexander|last8=Benzel|first8=Edward|date=2010-07-16|title=Deformative stress associated with an abnormal clivo-axial angle: A finite element analysis|url=https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2940090/|journal=Surgical Neurology International|volume=1|doi=10.4103/2152-7806.66461|issn=2152-7806|pmc=PMCPMC2940090|pmid=20847911}}</ref> sleep disturbance<ref name=":0" /><ref name=":4" />cognitive impairment<ref name=":4" /> and memory loss.

== Risk factors and comorbidities ==
Established risk factors for CCI include physical trauma, inflammatory disease, congenital disorders, neoplasms and hereditary hypermobility connective tissue disorders.<ref name=":10" /><ref name=":12">{{Cite journal|last=Sapkas|first=George|last2=Papadakis|first2=Stamatios A|last3=Segkos|first3=Dimitrios|last4=Kateros|first4=Konstantinos|last5=Tsakotos|first5=George|last6=Katonis|first6=Pavlos|date=2011-06-02|title=Posterior Instrumentation for Occipitocervical Fusion|url=https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3139273/|journal=The Open Orthopaedics Journal|volume=5|pages=209–218|doi=10.2174/1874325001105010209|issn=1874-3250|pmc=PMCPMC3139273|pmid=21772931}}</ref>

{| class="wikitable"
|'''Cause of instability'''
|'''Example'''
|-
|Physical trauma<ref>{{Cite journal|last=Ghatan|first=Saadi|last2=Newell|first2=David W.|last3=Grady|first3=M. Sean|last4=Mirza|first4=Sohail K.|last5=Chapman|first5=Jens R.|last6=Mann|first6=Frederick A.|last7=Ellenbogen|first7=Richard G.|date=2004-8|title=Severe posttraumatic craniocervical instability in the very young patient. Report of three cases|url=https://www.ncbi.nlm.nih.gov/pubmed/16206980|journal=Journal of Neurosurgery|volume=101|issue=1 Suppl|pages=102–107|doi=10.3171/ped.2004.101.2.0102|issn=0022-3085|pmid=16206980}}</ref>
|Car accident<ref>{{Cite journal|last=Uribe|first=Juan S.|last2=Ramos|first2=Edwin|last3=Baaj|first3=Ali|last4=Youssef|first4=A. Samy|last5=Vale|first5=Fernando L.|date=2009-12|title=Occipital cervical stabilization using occipital condyles for cranial fixation: technical case report|url=https://www.ncbi.nlm.nih.gov/pubmed/19934947|journal=Neurosurgery|volume=65|issue=6|pages=E1216–1217; discussion E1217|doi=10.1227/01.NEU.0000349207.98394.FA|issn=1524-4040|pmid=19934947}}</ref><ref>{{Cite journal|last=Volle|first=E.|last2=Montazem|first2=A.|date=2001-1|title=MRI video diagnosis and surgical therapy of soft tissue trauma to the craniocervical junction|url=https://www.ncbi.nlm.nih.gov/pubmed/11209518|journal=Ear, Nose, & Throat Journal|volume=80|issue=1|pages=41–44, 46–48|issn=0145-5613|pmid=11209518}}</ref>, blow to the head.<ref>{{Cite journal|last=Mathers|first=K. Sean|last2=Schneider|first2=Michael|last3=Timko|first3=Michael|date=2011-6|title=Occult hypermobility of the craniocervical junction: a case report and review|url=https://www.ncbi.nlm.nih.gov/pubmed/21628827|journal=The Journal of Orthopaedic and Sports Physical Therapy|volume=41|issue=6|pages=444–457|doi=10.2519/jospt.2011.3305|issn=1938-1344|pmid=21628827}}</ref>
|-
|Inflammatory disease
|Rheumatoid arthritis<ref>{{Cite journal|last=O'Brien|first=Michael F.|last2=Casey|first2=Adrian T. H.|last3=Crockard|first3=Alan|last4=Pringle|first4=Jean|last5=Stevens|first5=John M.|date=2002-10-15|title=Histology of the craniocervical junction in chronic rheumatoid arthritis: a clinicopathologic analysis of 33 operative cases|url=https://www.ncbi.nlm.nih.gov/pubmed/12394902|journal=Spine|volume=27|issue=20|pages=2245–2254|doi=10.1097/01.BRS.0000029252.98053.43|issn=1528-1159|pmid=12394902}}</ref>, tuberculosis<ref>{{Cite journal|last=Chaudhary|first=Kshitij|last2=Potdar|first2=Prabodhan|last3=Bapat|first3=Mihir|last4=Rathod|first4=Ashok|last5=Laheri|first5=Vinod|date=2012-06-15|title=Structural odontoid lesions in craniovertebral tuberculosis: a review of 15 cases|url=https://www.ncbi.nlm.nih.gov/pubmed/22261632|journal=Spine|volume=37|issue=14|pages=E836–843|doi=10.1097/BRS.0b013e31824a4c8f|issn=1528-1159|pmid=22261632}}</ref>
|-
|Neoplasms
|Tumors<ref name=":12" /> such as haemangioma, aneurysmal bone cyst
|-
|Congenital
|Down’s syndrome<ref>{{Cite journal|last=Hankinson|first=Todd C.|last2=Anderson|first2=Richard C. E.|date=2010-3|title=Craniovertebral junction abnormalities in Down syndrome|url=https://www.ncbi.nlm.nih.gov/pubmed/20173525|journal=Neurosurgery|volume=66|issue=3 Suppl|pages=32–38|doi=10.1227/01.NEU.0000365803.22786.F0|issn=1524-4040|pmid=20173525}}</ref>, os odontoideum<ref>{{Cite journal|last=Zhao|first=Deng|last2=Wang|first2=Shenglin|last3=Passias|first3=Peter G.|last4=Wang|first4=Chao|date=2015-5|title=Craniocervical instability in the setting of os odontoideum: assessment of cause, presentation, and surgical outcomes in a series of 279 cases|url=https://www.ncbi.nlm.nih.gov/pubmed/25635883|journal=Neurosurgery|volume=76|issue=5|pages=514–521|doi=10.1227/NEU.0000000000000668|issn=1524-4040|pmid=25635883}}</ref>, dwarfism
|-
|Hereditary hypermobility connective tissue disorders
|Ehlers Danlos Syndromes<ref name=":0" /><ref name=":1" />
|}
It is not unusual for CCI to co-occur with other structural neurological abnormalities such as atlantoaxial instability (AAI) and chiari malformation (CM).<ref>{{Cite journal|last=Camino Willhuber|first=Gaston O.|last2=Bosio|first2=Santiago T.|last3=Puigdevall|first3=Miguel H.|last4=Halliburton|first4=Carolina|last5=Sola|first5=Carlos A.|last6=Maenza|first6=Ruben A.|date=2017-1|title=Craniocervical spinal instability after type 1 Arnold Chiari decompression: a case report|url=https://www.ncbi.nlm.nih.gov/pubmed/27258364|journal=Journal of Pediatric Orthopedics. Part B|volume=26|issue=1|pages=80–85|doi=10.1097/BPB.0000000000000346|issn=1473-5865|pmid=27258364}}</ref><ref name=":0" />

== Diagnosis ==
{{Video|id=https://youtu.be/MsYDA3SXTkg|service=youtube|dimensions=550|description=Dr. Paolo Bolognese discusses methods of imaging and measurement for diagnosis.|alignment=right|urlargs=}}
The diagnosis of CCI is based on symptom presentation, a supportive history, demonstrable neurological findings and abnormal imaging.

=== Imaging ===
CCI is typically diagnosed via a cervical MRI, whether supine or upright. If supine, a 3 Tesla MRI is preferred over a 1.5 Tesla. Most neurosurgeons prefer upright MRI with flexion and extension.<ref>{{Cite web|url=https://www.mechanicalbasis.org/diagnosis.html|title=Craniocervical instability, Atlantoaxial Instability, Myalgic Encephalomyelitis, ME, CFS|website=MEchanical Basis|language=en|access-date=2019-06-02}}</ref> According to Henderson FC, “ventral brainstem compression may exist in flexion of the cervical spine, but appear normal on routine imaging.”<ref name=":9" />

=== Measurements ===
More than twenty radiological measurements have been proposed or used in the diagnosis of CCI. However, three measurements are most commonly used: the Grabb-Oakes line, which measures focal compression; the Clivo-Axial Angle (CXA), which measures brainstem deformity by the odontoid process; and the Basion Dens Interval, which measures vertical instability (cranial settling). Acorrding to a 2013 consensus statement on the assessment of CCI a CXA of 135 degrees or less should be considered as "potential pathological."<ref>https://csfinfo.org/files/1613/9665/4797/Final_Booklet_complete.pdf</ref>

=== Traction ===
Manual traction and invasive cervical traction are often used to aid in the diagnosis of CCI. Symptomatic improvement with traction can help determine whether a patient will benefit from craniocervical fusion surgery.

== Treatment ==
{{Video|id=https://youtu.be/sEi9AlHQTJc|service=youtube|dimensions=550|description=Dr. Fraser Henderson presents the results a five-year follow-up study.|alignment=right|urlargs=}}
=== Conservative treatment ===
Treatment of CCI can include “conservative measures” such as rest, pain management, bracing with a cervical collar, or physical therapy to strengthen neck muscles.<ref>{{Cite journal|last=Mathers|first=K. Sean|last2=Schneider|first2=Michael|last3=Timko|first3=Michael|date=2011-6|title=Occult hypermobility of the craniocervical junction: a case report and review|url=https://www.ncbi.nlm.nih.gov/pubmed/21628827|journal=The Journal of Orthopaedic and Sports Physical Therapy|volume=41|issue=6|pages=444–457|doi=10.2519/jospt.2011.3305|issn=1938-1344|pmid=21628827}}</ref> Many conservative therapies have little to no supporting evidence of efficacy.

There is no evidence for the efficacy of experimental treatments for CCI such as prolotherapy and upper cervical chiropractic.

=== Surgery ===
When non-invasive treatments for CCI fail to work, occipito-cervical fusion (OCF) can be considered.<ref name=":0" /> OCF is a surgery that aims at a biomechanical stabilization of the craniocervical junction. Patients with objective radiological findings, a clinical picture supportive of the diagnosis, a positive response to traction, and who are significantly impaired may be candidates for this surgery. Different surgical procedures for OCF exist, but the current standard involves internal fixation of the upper spine by mechanical screws. Surgery typically involves using titanium hardware to fixate the occiput, axis and atlas (i.e., C0 to C2) along with rib graft or cadaver bone graft. When cervical instability is present below C2, additional vertebrae may also be fused if the patient is symptomatic.

==== Risks and complications ====
The outcome of OCF is generally favorable with most patients experiencing symptom relief post-surgery.<ref name=":0" /> The complications of OCF however can be serious<ref>{{Cite journal|last=Garrido|first=Ben J.|last2=Sasso|first2=Rick C.|date=2012-1|title=Occipitocervical fusion|url=https://www.ncbi.nlm.nih.gov/pubmed/22082624|journal=The Orthopedic Clinics of North America|volume=43|issue=1|pages=1–9, vii|doi=10.1016/j.ocl.2011.08.009|issn=1558-1373|pmid=22082624}}</ref> and occur in an estimated 10% to 33% of patients.<ref name=":10" /><ref>{{Cite journal|last=Winegar|first=Corbett D.|last2=Lawrence|first2=James P.|last3=Friel|first3=Brian C.|last4=Fernandez|first4=Carmella|last5=Hong|first5=Joseph|last6=Maltenfort|first6=Mitchell|last7=Anderson|first7=Paul A.|last8=Vaccaro|first8=Alexander R.|date=2010-7|title=A systematic review of occipital cervical fusion: techniques and outcomes|url=https://www.ncbi.nlm.nih.gov/pubmed/20594011|journal=Journal of Neurosurgery. Spine|volume=13|issue=1|pages=5–16|doi=10.3171/2010.3.SPINE08143|issn=1547-5646|pmid=20594011}}</ref><ref name=":11" /><ref>{{Cite journal|last=Ando|first=Kei|last2=Imagama|first2=Shiro|last3=Ito|first3=Zenya|last4=Kobayashi|first4=Kazuyoshi|last5=Yagi|first5=Hideki|last6=Shinjo|first6=Ryuichi|last7=Hida|first7=Tetsuro|last8=Ito|first8=Kenyu|last9=Ishikawa|first9=Yoshimoto|date=2017-6|title=Minimum 5-year Follow-up Results for Occipitocervical Fusion Using the Screw-Rod System in Craniocervical Instability|url=https://www.ncbi.nlm.nih.gov/pubmed/28525489|journal=Clinical Spine Surgery|volume=30|issue=5|pages=E628–E632|doi=10.1097/BSD.0000000000000199|issn=2380-0194|pmid=28525489}}</ref> Common complications include screw failure, wound infection, dural tear and cerebrospinal fluid leakage<ref name=":11" /> In some cases revision surgery is needed to treat infection or to remove hardware. Severe complications include meningitis and accidental injury of the vertebral artery by misplaced screws.

==== Side effects ====
OCF causes a substantial reduction in the neck’s range of motion, estimated at approximately 40% of total cervical flexion–extension.<ref name=":8">{{Cite journal|last=Ashafai|first=Nabeel S.|last2=Visocchi|first2=Massimiliano|last3=Wąsik|first3=Norbert|date=2019|editor-last=Visocchi|editor-first=Massimiliano|title=Occipitocervical Fusion: An Updated Review|url=https://doi.org/10.1007/978-3-319-62515-7_35|series=Acta Neurochirurgica Supplement|language=en|location=Cham|publisher=Springer International Publishing|pages=247–252|doi=10.1007/978-3-319-62515-7_35|isbn=9783319625157}}</ref>

==== Cost ====
OCF is estimated to cost tens of thousands of dollars, although some insurance schemes fully cover the cost of surgery depending on the country located and neurosurgeons involved.

== Dysautonomia and CCI in EDS ==
As CCI might lead to a compression of the brain stem, some researchers speculate this might cause some of the autonomic symptoms such as tachycardia and orthostatic intolerance that are frequently seen in patients with Ehlers Danlos Syndromes (EDS). In a 2007 influential paper Milhorat et al. followed-up on patients with Chiari malformation who did not improve with treatment and surgery. The authors discovered that many of these patients suffered from EDS and had other structural abnormalities at the upper spine such as CCI and cranial settling. Milhorat et al. speculated that the resulting compression of the brainstem might be the cause of the autonomic and other symptoms these patients were suffering from.<ref>{{Cite journal|last=Milhorat|first=Thomas H.|last2=Bolognese|first2=Paolo A.|last3=Nishikawa|first3=Misao|last4=McDonnell|first4=Nazli B.|last5=Francomano|first5=Clair A.|date=2007-12|title=Syndrome of occipitoatlantoaxial hypermobility, cranial settling, and chiari malformation type I in patients with hereditary disorders of connective tissue|url=https://www.ncbi.nlm.nih.gov/pubmed/18074684|journal=Journal of Neurosurgery. Spine|volume=7|issue=6|pages=601–609|doi=10.3171/SPI-07/12/601|issn=1547-5654|pmid=18074684}}</ref> In conference presentations, neurosurgeons have indicated that they think CCI can cause dysautonomia symptoms such as postural orthostatic tachycardia sydrome (POTS) <ref>[https://www.youtube.com/watch?time_continue=735&v=857Jsjsqxjw Craniocervical Instability (Dr Henderson the 2012 EDNF Confrence).] Minute 12.10. </ref> [OTHER REFERENCE NEEDED].

Henderson et al. tested this theory by following 20 CCI patients with comorbid Chiari Malformation and hereditary hypermobility connective tissue disorders for a period of 5 years after OCF-surgery. Patients were satisfied with the surgery and experienced significant improvements in some CCI-related symptoms such as vertigo, headaches, imbalance, dysarthria dizziness or frequent daytime urination. There was however only a small increase in objective outcomes such as work resumption with 60% of patients remaining unable to work or go to school. Participants attributed this to other medical problems related to EDS such as musculoskeletal pain, fatigue, gastrointestinal issues and POTS, indicating these were not significantly improved after OCF-surgery.<ref name=":0" />

== Mechanical basis theory ==
Five ME/CFS patients diagnosed with CCI report to have experienced spectacular improvements and even remission of their ME/CFS symptoms following OCF-surgery. Some of them have dual diagnosis of EDS.<ref>{{Cite web|url=https://medium.com/@jenbrea/health-update-3-my-me-is-in-remission-dd575e650f71|title=Health update #3: My ME is in remission|last=Brea|first=Jennifer|date=2019-05-20|website=Medium|access-date=2019-06-03}}</ref><ref>{{Cite web|url=https://www.mechanicalbasis.org/interviews.html|title=Craniocervical instability, Atlantoaxial Instability, Myalgic Encephalomyelitis, ME, CFS|website=MEchanical Basis|language=en|access-date=2019-06-06}}</ref> They speculate that mechanical compression of the brainstem due to CCI has the potential to cause characteristic ME/CFS symptoms such as post-exertional malaise<ref>{{Cite web|url=https://www.mechanicalbasis.org/|title=Craniocervical instability, Atlantoaxial Instability, Myalgic Encephalomyelitis, ME, CFS|website=MEchanical Basis|language=en|access-date=2019-06-03}}</ref>, although this theory is currently not supported by scientific evidence. The current literature does not suggest a connection between ME/CFS and CCI. Others have raised concerns about CCI surgery in patients with ME/CFS given the lack of research on OCF in this patient population.<ref>{{Cite web|url=https://www.s4me.info/threads/concerns-about-craniocervical-instability-surgery-in-me-cfs.9638/|title=Concerns about craniocervical instability surgery in ME/CFS|website=Science for ME|language=en-US|access-date=2019-06-03}}</ref>

== Synonyms ==
* Syndrome of Occipitoatlantialaxial Hypermobility<ref name=":1" />
* Hypermobility of the Craniocervical Junction<ref>{{Cite journal|last=Mathers|first=K. Sean|last2=Schneider|first2=Michael|last3=Timko|first3=Michael|date=2011-6|title=Occult hypermobility of the craniocervical junction: a case report and review|url=https://www.ncbi.nlm.nih.gov/pubmed/21628827|journal=The Journal of Orthopaedic and Sports Physical Therapy|volume=41|issue=6|pages=444–457|doi=10.2519/jospt.2011.3305|issn=1938-1344|pmid=21628827}}</ref>

== See also ==

* [[Brainstem compression]]
* [[Ehlers-Danlos syndrome]]
* [[Chiari malformation]]
* [[Intracranial hypertension]]
* Atlantoaxial instability (AAI)
* [[Tethered cord syndrome]]

== Learn more ==

== References ==
<references />
[[Category:Diagnoses]]

Craniocervical instability

2019-06-06T11:18:12Z

Jeff:/* Symptoms */

'''Craniocervical instability''' (CCI) is a pathological condition of increased mobility at the craniocervical junction, the area where the skull meets the spine. In CCI the ligamentous connections of the craniocervical junction can be stretched, weakened or ruptured.<ref name=":2">{{Cite journal|last=Henderson|first=Fraser C.|last2=Austin|first2=Claudiu|last3=Benzel|first3=Edward|last4=Bolognese|first4=Paolo|last5=Ellenbogen|first5=Richard|last6=Francomano|first6=Clair A.|last7=Ireton|first7=Candace|last8=Klinge|first8=Petra|last9=Koby|first9=Myles|date=2017|title=Neurological and spinal manifestations of the Ehlers–Danlos syndromes|url=https://onlinelibrary.wiley.com/doi/abs/10.1002/ajmg.c.31549|journal=American Journal of Medical Genetics Part C: Seminars in Medical Genetics|language=en|volume=175|issue=1|pages=195–211|doi=10.1002/ajmg.c.31549|issn=1552-4876}}</ref> This can lead to compression of the [[Brainstem compression|brainstem]], upper [[spinal cord]], or [[cerebellum]] and result in myelopathy, disabling neck pain and a range of other symptoms.<ref name=":11">{{Cite journal|last=Choi|first=Sung Ho|last2=Lee|first2=Sang Gu|last3=Park|first3=Chan Woo|last4=Kim|first4=Woo Kyung|last5=Yoo|first5=Chan Jong|last6=Son|first6=Seong|date=2013-4|title=Surgical Outcomes and Complications after Occipito-Cervical Fusion Using the Screw-Rod System in Craniocervical Instability|url=https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3698232/|journal=Journal of Korean Neurosurgical Society|volume=53|issue=4|pages=223–227|doi=10.3340/jkns.2013.53.4.223|issn=2005-3711|pmc=PMCPMC3698232|pmid=23826478}}</ref>

CCI usually develops as a result of physical trauma such as a car accident, an inflammation disease such as rheumatoid arthritis or a congenital disorder such as Down syndrome.<ref name=":10">{{Cite journal|last=Ashafai|first=Nabeel S.|last2=Visocchi|first2=Massimiliano|last3=Wąsik|first3=Norbert|date=2019|title=Occipitocervical Fusion: An Updated Review|url=https://www.ncbi.nlm.nih.gov/pubmed/30610329|journal=Acta Neurochirurgica. Supplement|volume=125|pages=247–252|doi=10.1007/978-3-319-62515-7_35|issn=0065-1419|pmid=30610329}}</ref> More recently, physicians have recognized an increased prevalence of CCI in patients with hereditary disorders of connective tissue such as Ehlers Danlos Syndromes (EDS).<ref name=":9">{{Cite web|url=https://www.researchgate.net/publication/302923107_Cranio-cervical_Instability_in_Patients_with_Hypermobility_Connective_Disorders|title=(PDF) Cranio-cervical Instability in Patients with Hypermobility Connective Disorders|website=ResearchGate|language=en|access-date=2019-06-03}}</ref> There have also been anecdotal reports of patients with myalgic encephalomyelitis (ME) who were diagnosed with CCI and improved markedly following craniocervical fusion surgery<ref>{{Cite web|url=https://forums.phoenixrising.me/index.php?threads/have-you-ruled-out-chiari-as-a-cause-of-your-cfs.56908/|title=Have you ruled out Chiari as a cause of your CFS|last=|first=|date=|website=Phoenix Rising|archive-url=|archive-date=|dead-url=|access-date=}}</ref><ref>{{Cite web|url=https://rsci.app.link/3LpJOxcTaX?_p=f3542c5bfc29c2616780177b27|website=rsci.app.link|access-date=2019-06-01}}</ref>, although scientific publications on this subject are currently lacking.
== Symptoms ==
Characteristic symptoms of craniocervical instability include neck pain, headache, fatigue, and dizziness. Additional neurological abnormalities such as numbness, motor weakness and gait instability are not uncommon,<ref>{{Cite journal|last=Bobinski|first=Lukas|last2=Levivier|first2=Marc|last3=Duff|first3=John M.|date=2015-2|title=Occipitoaxial spinal interarticular stabilization with vertebral artery preservation for atlantal lateral mass failure|url=https://www.ncbi.nlm.nih.gov/pubmed/25415481|journal=Journal of Neurosurgery. Spine|volume=22|issue=2|pages=134–138|doi=10.3171/2014.10.SPINE14131|issn=1547-5646|pmid=25415481}}</ref><ref>{{Cite journal|last=O'Brien|first=Michael F.|last2=Casey|first2=Adrian T. H.|last3=Crockard|first3=Alan|last4=Pringle|first4=Jean|last5=Stevens|first5=John M.|date=2002-10-15|title=Histology of the craniocervical junction in chronic rheumatoid arthritis: a clinicopathologic analysis of 33 operative cases|url=https://www.ncbi.nlm.nih.gov/pubmed/12394902|journal=Spine|volume=27|issue=20|pages=2245–2254|doi=10.1097/01.BRS.0000029252.98053.43|issn=1528-1159|pmid=12394902}}</ref><ref>{{Cite journal|last=Young|first=Richard M.|last2=Sherman|first2=Jonathan H.|last3=Wind|first3=Joshua J.|last4=Litvack|first4=Zachary|last5=O'Brien|first5=Joseph|date=2014-8|title=Treatment of craniocervical instability using a posterior-only approach: report of 3 cases|url=https://www.ncbi.nlm.nih.gov/pubmed/24785968|journal=Journal of Neurosurgery. Spine|volume=21|issue=2|pages=239–248|doi=10.3171/2014.3.SPINE13684|issn=1547-5646|pmid=24785968}}</ref><ref>{{Cite journal|last=Botelho|first=Ricardo V.|last2=Neto|first2=Eliseu B.|last3=Patriota|first3=Gustavo C.|last4=Daniel|first4=Jefferson W.|last5=Dumont|first5=Paulo A. S.|last6=Rotta|first6=José M.|date=2007-10|title=Basilar invagination: craniocervical instability treated with cervical traction and occipitocervical fixation. Case report|url=https://www.ncbi.nlm.nih.gov/pubmed/17933321|journal=Journal of Neurosurgery. Spine|volume=7|issue=4|pages=444–449|doi=10.3171/SPI-07/10/444|issn=1547-5654|pmid=17933321}}</ref><ref>{{Cite journal|last=Henderson|first=Fraser C.|last2=Henderson|first2=Fraser C.|last3=Wilson|first3=William A.|last4=Mark|first4=Alexander S.|last5=Koby|first5=Myles|date=2018-1|title=Utility of the clivo-axial angle in assessing brainstem deformity: pilot study and literature review|url=https://www.ncbi.nlm.nih.gov/pubmed/28258417|journal=Neurosurgical Review|volume=41|issue=1|pages=149–163|doi=10.1007/s10143-017-0830-3|issn=1437-2320|pmc=PMCPMC5748419|pmid=28258417}}</ref> although no particular symptom is mandatory for a diagnosis of CCI. Patients also frequently describe a constant or intermittent pain at lower back of the head and the feeling that their head is too heavy for their neck to support (“bobble-head”)<ref name=":9" />

Other symptoms of CCI include:
* [[Muscle weakness]]<ref name=":0">{{Cite journal|last=Henderson|first=Fraser C.|last2=Francomano|first2=C. A.|last3=Koby|first3=M.|last4=Tuchman|first4=K.|last5=Adcock|first5=J.|last6=Patel|first6=S.|date=2019-01-09|title=Cervical medullary syndrome secondary to craniocervical instability and ventral brainstem compression in hereditary hypermobility connective tissue disorders: 5-year follow-up after craniocervical reduction, fusion, and stabilization|url=https://doi.org/10.1007/s10143-018-01070-4|journal=Neurosurgical Review|language=en|doi=10.1007/s10143-018-01070-4|issn=1437-2320}}</ref>, [[numbness]]<ref name=":1">{{Cite journal|last=Francomano|first=Clair A.|last2=McDonnell|first2=Nazli B.|last3=Nishikawa|first3=Misao|last4=Bolognese|first4=Paolo A.|last5=Milhorat|first5=Thomas H.|date=2007-12-01|title=Syndrome of occipitoatlantoaxial hypermobility, cranial settling, and Chiari malformation Type I in patients with hereditary disorders of connective tissue|url=https://thejns.org/view/journals/j-neurosurg-spine/7/6/article-p601.xml|journal=Journal of Neurosurgery: Spine|language=en-US|volume=7|issue=6|pages=601–609|doi=10.3171/SPI-07/12/601}}</ref><ref name=":2" /> [[paralysis]], parasthesias<ref name=":0" /><ref name=":4">{{Cite web|url=https://search.proquest.com/openview/34b3b18a8854c04ffa0fc50273d68313/1?pq-origsite=gscholar&cbl=47886|title=MRI video diagnosis and surgical therapy of soft tissue trauma to the craniocervical junction - ProQuest|website=search.proquest.com|language=en|access-date=2019-06-01}}</ref><ref name=":5">{{Cite journal|last=Rebbeck|first=Trudy|last2=Liebert|first2=Ann|date=2014-12-01|title=Clinical management of cranio-vertebral instability after whiplash, when guidelines should be adapted: A case report|url=http://www.sciencedirect.com/science/article/pii/S1356689X14000101|journal=Manual Therapy|volume=19|issue=6|pages=618–621|doi=10.1016/j.math.2014.01.009|issn=1356-689X}}</ref><ref name=":6">{{Cite journal|last=Mathers|first=K. Sean|last2=Schneider|first2=Michael|last3=Timko|first3=Michael|date=2011-06|title=Occult Hypermobility of the Craniocervical Junction: A Case Report and Review|url=https://www.jospt.org/doi/full/10.2519/jospt.2011.3305|journal=Journal of Orthopaedic & Sports Physical Therapy|language=en|volume=41|issue=6|pages=444–457|doi=10.2519/jospt.2011.3305|issn=0190-6011}}</ref>
* Poor proprioception<ref name=":7">{{Cite journal|last=Bergholm|first=Ulla|last2=Johansson|first2=Bengt H.|last3=Johansson|first3=Hakan|date=2004-01-01|title=New Diagnostic Tools Can Contribute to Better Treatment of Patients with Chronic Whiplash Disorders|url=https://doi.org/10.3109/J180v03n02_02|journal=Journal of Whiplash & Related Disorders|volume=3|issue=2|pages=5–19|doi=10.3109/J180v03n02_02|issn=1533-2888}}</ref>, impaired [[coordination]], gait changes<ref name=":0" /><ref name=":7" />
* [[Dizziness|Dizzinesss]], [[vertigo]]<ref name=":0" /><ref name=":2" /><ref name=":7" /><ref name=":4" /><ref name=":6" /><ref name=":5" /> syncope<ref name=":0" /><ref name=":1" /><ref name=":2" /><ref name=":7" />, nausea<ref name=":1" /><ref name=":2" />
* Neck stiffness, torticollis<ref name=":7" /><ref>{{Cite journal|last=Ghanem|first=Ismat|last2=El Hage|first2=Samer|last3=Rachkidi|first3=Rami|last4=Kharrat|first4=Khalil|last5=Dagher|first5=Fernand|last6=Kreichati|first6=Gabi|date=2008-03-01|title=Pediatric cervical spine instability|url=https://online.boneandjoint.org.uk/doi/full/10.1007/s11832-008-0092-2|journal=Journal of Children's Orthopaedics|volume=2|issue=2|pages=71–84|doi=10.1007/s11832-008-0092-2|issn=1863-2521|pmc=PMC2656787|pmid=19308585}}</ref> posterior scalp irritation<ref name=":7" />, facial pain, headache behind the eyes
* Apnea<ref>{{Cite journal|last=Janjua|first=M. Burhan|last2=Hwang|first2=Steven W.|last3=Samdani|first3=Amer F.|last4=Pahys|first4=Joshua M.|last5=Baaj|first5=Ali A.|last6=Härtl|first6=Roger|last7=Greenfield|first7=Jeffrey P.|date=2019-01-01|title=Instrumented arthrodesis for non-traumatic craniocervical instability in very young children|url=https://doi.org/10.1007/s00381-018-3876-9|journal=Child's Nervous System|language=en|volume=35|issue=1|pages=97–106|doi=10.1007/s00381-018-3876-9|issn=1433-0350}}</ref><ref name=":1" /><ref name=":3">{{Cite journal|last=Henderson|first=Fraser C.|last2=Austin|first2=Claudiu|last3=Benzel|first3=Edward|last4=Bolognese|first4=Paolo|last5=Ellenbogen|first5=Richard|last6=Francomano|first6=Clair A.|last7=Ireton|first7=Candace|last8=Klinge|first8=Petra|last9=Koby|first9=Myles|date=2017|title=Neurological and spinal manifestations of the Ehlers–Danlos syndromes|url=https://onlinelibrary.wiley.com/doi/abs/10.1002/ajmg.c.31549|journal=American Journal of Medical Genetics Part C: Seminars in Medical Genetics|language=en|volume=175|issue=1|pages=195–211|doi=10.1002/ajmg.c.31549|issn=1552-4876}}</ref>, dyspnea (shortness of breath)<ref name=":0" /><ref name=":1" /> and dysphagia (difficulty swallowing)<ref name=":1" /><ref name=":2" />
* Visual disturbances<ref name=":4" /> downward [[nystagmus]] (irregular eye movements)<ref name=":1" /><ref name=":7" /> tinnitus<ref>{{Cite journal|last=Montazem|first=Abbas|author-link=|author-link2=|author-link3=|author-link4=|author-link5=|date=2000|title=Secondary tinnitus as a symptom of instability in the upper cervical spine: Operative management|url=https://pdfs.semanticscholar.org/21c4/85984a6ebe07efed38cf82a2f7a49b2a644e.pdf|journal=International Tinnitus Journal|volume=|issue=|pages=|quote=|via=}}</ref><ref name=":4" />
* Fatigue<ref name=":0" /><ref>{{Cite journal|last=Henderson|first=Fraser C.|last2=Henderson|first2=Fraser C.|last3=Wilson|first3=William A.|last4=Mark|first4=Alexander S.|last5=Koby|first5=Myles|date=2018-01-01|title=Utility of the clivo-axial angle in assessing brainstem deformity: pilot study and literature review|url=https://doi.org/10.1007/s10143-017-0830-3|journal=Neurosurgical Review|language=en|volume=41|issue=1|pages=149–163|doi=10.1007/s10143-017-0830-3|issn=1437-2320|pmc=PMC5748419|pmid=28258417}}</ref><ref>{{Cite journal|last=Henderson|first=Fraser C.|last2=Wilson|first2=William A.|last3=Mott|first3=Stephen|last4=Mark|first4=Alexander|last5=Schmidt|first5=Kristi|last6=Berry|first6=Joel K.|last7=Vaccaro|first7=Alexander|last8=Benzel|first8=Edward|date=2010-07-16|title=Deformative stress associated with an abnormal clivo-axial angle: A finite element analysis|url=https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2940090/|journal=Surgical Neurology International|volume=1|doi=10.4103/2152-7806.66461|issn=2152-7806|pmc=PMCPMC2940090|pmid=20847911}}</ref> sleep disturbance<ref name=":0" /><ref name=":4" />cognitive impairment<ref name=":4" /> and memory loss.

== Risk factors and comorbidities ==
Established risk factors for CCI include physical trauma, inflammatory disease, congenital disorders, neoplasms and hereditary hypermobility connective tissue disorders.<ref name=":10" /><ref name=":12">{{Cite journal|last=Sapkas|first=George|last2=Papadakis|first2=Stamatios A|last3=Segkos|first3=Dimitrios|last4=Kateros|first4=Konstantinos|last5=Tsakotos|first5=George|last6=Katonis|first6=Pavlos|date=2011-06-02|title=Posterior Instrumentation for Occipitocervical Fusion|url=https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3139273/|journal=The Open Orthopaedics Journal|volume=5|pages=209–218|doi=10.2174/1874325001105010209|issn=1874-3250|pmc=PMCPMC3139273|pmid=21772931}}</ref>

{| class="wikitable"
|'''Cause of instability'''
|'''Example'''
|-
|Physical trauma<ref>{{Cite journal|last=Ghatan|first=Saadi|last2=Newell|first2=David W.|last3=Grady|first3=M. Sean|last4=Mirza|first4=Sohail K.|last5=Chapman|first5=Jens R.|last6=Mann|first6=Frederick A.|last7=Ellenbogen|first7=Richard G.|date=2004-8|title=Severe posttraumatic craniocervical instability in the very young patient. Report of three cases|url=https://www.ncbi.nlm.nih.gov/pubmed/16206980|journal=Journal of Neurosurgery|volume=101|issue=1 Suppl|pages=102–107|doi=10.3171/ped.2004.101.2.0102|issn=0022-3085|pmid=16206980}}</ref>
|Car accident<ref>{{Cite journal|last=Uribe|first=Juan S.|last2=Ramos|first2=Edwin|last3=Baaj|first3=Ali|last4=Youssef|first4=A. Samy|last5=Vale|first5=Fernando L.|date=2009-12|title=Occipital cervical stabilization using occipital condyles for cranial fixation: technical case report|url=https://www.ncbi.nlm.nih.gov/pubmed/19934947|journal=Neurosurgery|volume=65|issue=6|pages=E1216–1217; discussion E1217|doi=10.1227/01.NEU.0000349207.98394.FA|issn=1524-4040|pmid=19934947}}</ref><ref>{{Cite journal|last=Volle|first=E.|last2=Montazem|first2=A.|date=2001-1|title=MRI video diagnosis and surgical therapy of soft tissue trauma to the craniocervical junction|url=https://www.ncbi.nlm.nih.gov/pubmed/11209518|journal=Ear, Nose, & Throat Journal|volume=80|issue=1|pages=41–44, 46–48|issn=0145-5613|pmid=11209518}}</ref>, blow to the head.<ref>{{Cite journal|last=Mathers|first=K. Sean|last2=Schneider|first2=Michael|last3=Timko|first3=Michael|date=2011-6|title=Occult hypermobility of the craniocervical junction: a case report and review|url=https://www.ncbi.nlm.nih.gov/pubmed/21628827|journal=The Journal of Orthopaedic and Sports Physical Therapy|volume=41|issue=6|pages=444–457|doi=10.2519/jospt.2011.3305|issn=1938-1344|pmid=21628827}}</ref>
|-
|Inflammatory disease
|Rheumatoid arthritis<ref>{{Cite journal|last=O'Brien|first=Michael F.|last2=Casey|first2=Adrian T. H.|last3=Crockard|first3=Alan|last4=Pringle|first4=Jean|last5=Stevens|first5=John M.|date=2002-10-15|title=Histology of the craniocervical junction in chronic rheumatoid arthritis: a clinicopathologic analysis of 33 operative cases|url=https://www.ncbi.nlm.nih.gov/pubmed/12394902|journal=Spine|volume=27|issue=20|pages=2245–2254|doi=10.1097/01.BRS.0000029252.98053.43|issn=1528-1159|pmid=12394902}}</ref>, tuberculosis<ref>{{Cite journal|last=Chaudhary|first=Kshitij|last2=Potdar|first2=Prabodhan|last3=Bapat|first3=Mihir|last4=Rathod|first4=Ashok|last5=Laheri|first5=Vinod|date=2012-06-15|title=Structural odontoid lesions in craniovertebral tuberculosis: a review of 15 cases|url=https://www.ncbi.nlm.nih.gov/pubmed/22261632|journal=Spine|volume=37|issue=14|pages=E836–843|doi=10.1097/BRS.0b013e31824a4c8f|issn=1528-1159|pmid=22261632}}</ref>
|-
|Neoplasms
|Tumors<ref name=":12" /> such as haemangioma, aneurysmal bone cyst
|-
|Congenital
|Down’s syndrome<ref>{{Cite journal|last=Hankinson|first=Todd C.|last2=Anderson|first2=Richard C. E.|date=2010-3|title=Craniovertebral junction abnormalities in Down syndrome|url=https://www.ncbi.nlm.nih.gov/pubmed/20173525|journal=Neurosurgery|volume=66|issue=3 Suppl|pages=32–38|doi=10.1227/01.NEU.0000365803.22786.F0|issn=1524-4040|pmid=20173525}}</ref>, os odontoideum<ref>{{Cite journal|last=Zhao|first=Deng|last2=Wang|first2=Shenglin|last3=Passias|first3=Peter G.|last4=Wang|first4=Chao|date=2015-5|title=Craniocervical instability in the setting of os odontoideum: assessment of cause, presentation, and surgical outcomes in a series of 279 cases|url=https://www.ncbi.nlm.nih.gov/pubmed/25635883|journal=Neurosurgery|volume=76|issue=5|pages=514–521|doi=10.1227/NEU.0000000000000668|issn=1524-4040|pmid=25635883}}</ref>, dwarfism
|-
|Hereditary hypermobility connective tissue disorders
|Ehlers Danlos Syndromes<ref name=":0" /><ref name=":1" />
|}
It is not unusual for CCI to co-occur with other structural neurological abnormalities such as atlantoaxial instability (AAI) and chiari malformation (CM).<ref>{{Cite journal|last=Camino Willhuber|first=Gaston O.|last2=Bosio|first2=Santiago T.|last3=Puigdevall|first3=Miguel H.|last4=Halliburton|first4=Carolina|last5=Sola|first5=Carlos A.|last6=Maenza|first6=Ruben A.|date=2017-1|title=Craniocervical spinal instability after type 1 Arnold Chiari decompression: a case report|url=https://www.ncbi.nlm.nih.gov/pubmed/27258364|journal=Journal of Pediatric Orthopedics. Part B|volume=26|issue=1|pages=80–85|doi=10.1097/BPB.0000000000000346|issn=1473-5865|pmid=27258364}}</ref><ref name=":0" />

== Diagnosis ==
{{Video|id=https://youtu.be/MsYDA3SXTkg|service=youtube|dimensions=550|description=Dr. Paolo Bolognese discusses methods of imaging and measurement for diagnosis.|alignment=right|urlargs=}}
The diagnosis of CCI is based on symptom presentation, a supportive history, demonstrable neurological findings and abnormal imaging.

=== Imaging ===
CCI is typically diagnosed via a cervical MRI, whether supine or upright. If supine, a 3 Tesla MRI is preferred over a 1.5 Tesla. Most neurosurgeons prefer upright MRI with flexion and extension.<ref>{{Cite web|url=https://www.mechanicalbasis.org/diagnosis.html|title=Craniocervical instability, Atlantoaxial Instability, Myalgic Encephalomyelitis, ME, CFS|website=MEchanical Basis|language=en|access-date=2019-06-02}}</ref> According to Henderson FC, “ventral brainstem compression may exist in flexion of the cervical spine, but appear normal on routine imaging.”<ref name=":9" />

=== Measurements ===
More than twenty radiological measurements have been proposed or used in the diagnosis of CCI. However, three measurements are most commonly used: the Grabb-Oakes line, which measures focal compression; the Clivo-Axial Angle (CXA), which measures brainstem deformity by the odontoid process; and the Basion Dens Interval, which measures vertical instability (cranial settling). Acorrding to a 2013 consensus statement on the assessment of CCI a CXA of 135 degrees or less should be considered as "potential pathological."<ref>https://csfinfo.org/files/1613/9665/4797/Final_Booklet_complete.pdf</ref>

=== Traction ===
Manual traction and invasive cervical traction are often used to aid in the diagnosis of CCI. Symptomatic improvement with traction can help determine whether a patient will benefit from craniocervical fusion surgery.

== Treatment ==
{{Video|id=https://youtu.be/sEi9AlHQTJc|service=youtube|dimensions=550|description=Dr. Fraser Henderson presents the results a five-year follow-up study.|alignment=right|urlargs=}}
=== Conservative treatment ===
Treatment of CCI can include “conservative measures” such as rest, pain management, bracing with a cervical collar, or physical therapy to strengthen neck muscles.<ref>{{Cite journal|last=Mathers|first=K. Sean|last2=Schneider|first2=Michael|last3=Timko|first3=Michael|date=2011-6|title=Occult hypermobility of the craniocervical junction: a case report and review|url=https://www.ncbi.nlm.nih.gov/pubmed/21628827|journal=The Journal of Orthopaedic and Sports Physical Therapy|volume=41|issue=6|pages=444–457|doi=10.2519/jospt.2011.3305|issn=1938-1344|pmid=21628827}}</ref> Many conservative therapies have little to no supporting evidence of efficacy.

There is no evidence for the efficacy of experimental treatments of CCI such as prolotherapy and upper cervical chiropractic.

=== Surgery ===
When non-invasive treatments for CCI fail to work, occipito-cervical fusion (OCF) can be considered.<ref name=":0" /> OCF is a surgery that aims at a biomechanical stabilization of the craniocervical junction. Patients with objective radiological findings, a clinical picture supportive of the diagnosis, a positive response to traction, and who are significantly impaired may be candidates for this surgery. Different surgical procedures for OCF exist, but the current standard involves internal fixation of the upper spine by mechanical screws. Surgery typically involves using titanium hardware to fixate the occiput, axis and atlas (i.e., C0 to C2) along with rib graft or cadaver bone graft. When cervical instability is present below C2, additional vertebrae may also be fused if the patient is symptomatic.

==== Risks and complications ====
The outcome of OCF is generally favorable with most patients experiencing symptom relief post-surgery.<ref name=":0" /> The complications of OCF however can be serious<ref>{{Cite journal|last=Garrido|first=Ben J.|last2=Sasso|first2=Rick C.|date=2012-1|title=Occipitocervical fusion|url=https://www.ncbi.nlm.nih.gov/pubmed/22082624|journal=The Orthopedic Clinics of North America|volume=43|issue=1|pages=1–9, vii|doi=10.1016/j.ocl.2011.08.009|issn=1558-1373|pmid=22082624}}</ref> and occur in an estimated 10% to 33% of patients.<ref name=":10" /><ref>{{Cite journal|last=Winegar|first=Corbett D.|last2=Lawrence|first2=James P.|last3=Friel|first3=Brian C.|last4=Fernandez|first4=Carmella|last5=Hong|first5=Joseph|last6=Maltenfort|first6=Mitchell|last7=Anderson|first7=Paul A.|last8=Vaccaro|first8=Alexander R.|date=2010-7|title=A systematic review of occipital cervical fusion: techniques and outcomes|url=https://www.ncbi.nlm.nih.gov/pubmed/20594011|journal=Journal of Neurosurgery. Spine|volume=13|issue=1|pages=5–16|doi=10.3171/2010.3.SPINE08143|issn=1547-5646|pmid=20594011}}</ref><ref name=":11" /><ref>{{Cite journal|last=Ando|first=Kei|last2=Imagama|first2=Shiro|last3=Ito|first3=Zenya|last4=Kobayashi|first4=Kazuyoshi|last5=Yagi|first5=Hideki|last6=Shinjo|first6=Ryuichi|last7=Hida|first7=Tetsuro|last8=Ito|first8=Kenyu|last9=Ishikawa|first9=Yoshimoto|date=2017-6|title=Minimum 5-year Follow-up Results for Occipitocervical Fusion Using the Screw-Rod System in Craniocervical Instability|url=https://www.ncbi.nlm.nih.gov/pubmed/28525489|journal=Clinical Spine Surgery|volume=30|issue=5|pages=E628–E632|doi=10.1097/BSD.0000000000000199|issn=2380-0194|pmid=28525489}}</ref> Common complications include screw failure, wound infection, dural tear and cerebrospinal fluid leakage<ref name=":11" /> In some cases revision surgery is needed to treat infection or to remove hardware. Severe complications include meningitis and accidental injury of the vertebral artery by misplaced screws.

==== Side effects ====
OCF causes a substantial reduction in the neck’s range of motion, estimated at approximately 40% of total cervical flexion–extension.<ref name=":8">{{Cite journal|last=Ashafai|first=Nabeel S.|last2=Visocchi|first2=Massimiliano|last3=Wąsik|first3=Norbert|date=2019|editor-last=Visocchi|editor-first=Massimiliano|title=Occipitocervical Fusion: An Updated Review|url=https://doi.org/10.1007/978-3-319-62515-7_35|series=Acta Neurochirurgica Supplement|language=en|location=Cham|publisher=Springer International Publishing|pages=247–252|doi=10.1007/978-3-319-62515-7_35|isbn=9783319625157}}</ref>

==== Cost ====
OCF is estimated to cost tens of thousands of dollars, although some insurance schemes fully cover the cost of surgery depending on the country located and neurosurgeons involved.

== Dysautonomia and CCI in EDS ==
As CCI might lead to a compression of the brain stem, some researchers speculate this might cause some of the autonomic symptoms such as tachycardia and orthostatic intolerance that are frequently seen in patients with Ehlers Danlos Syndromes (EDS). In a 2007 influential paper Milhorat et al. followed-up on patients with Chiari malformation who did not improve with treatment and surgery. The authors discovered that many of these patients suffered from EDS and had other structural abnormalities at the upper spine such as CCI and cranial settling. Milhorat et al. speculated that the resulting compression of the brainstem might be the cause of the autonomic and other symptoms these patients were suffering from.<ref>{{Cite journal|last=Milhorat|first=Thomas H.|last2=Bolognese|first2=Paolo A.|last3=Nishikawa|first3=Misao|last4=McDonnell|first4=Nazli B.|last5=Francomano|first5=Clair A.|date=2007-12|title=Syndrome of occipitoatlantoaxial hypermobility, cranial settling, and chiari malformation type I in patients with hereditary disorders of connective tissue|url=https://www.ncbi.nlm.nih.gov/pubmed/18074684|journal=Journal of Neurosurgery. Spine|volume=7|issue=6|pages=601–609|doi=10.3171/SPI-07/12/601|issn=1547-5654|pmid=18074684}}</ref> In conference presentations, several neurosurgeons have indicated that they think CCI can cause dysautonomia symptoms such as postural orthostatic tachycardia sydrome (POTS) <ref>[https://www.youtube.com/watch?time_continue=735&v=857Jsjsqxjw Craniocervical Instability (Dr Henderson the 2012 EDNF Confrence).] Minute 12.10. </ref> [OTHER REFERENCE NEEDED].

Henderson et al. tested this theory by following 20 CCI patients with comorbid Chiari Malformation and hereditary hypermobility connective tissue disorders for a period of 5 years after OCF-surgery. Patients were satisfied with the surgery and experienced significant improvements in some CCI-related symptoms such as vertigo, headaches, imbalance, dysarthria dizziness or frequent daytime urination. There was however only a small increase in objective outcomes such as work resumption with 60% of patients remaining unable to work or go to school. Participants attributed this to other medical problems related to EDS such as musculoskeletal pain, fatigue, gastrointestinal issues and POTS, indicating these were not significantly improved after OCF-surgery.<ref name=":0" />

== Mechanical basis theory ==
Some ME/CFS patients diagnosed with CCI report to have experienced spectacular improvements and even remission of their ME/CFS symptoms following OCF-surgery.<ref>{{Cite web|url=https://medium.com/@jenbrea/health-update-3-my-me-is-in-remission-dd575e650f71|title=Health update #3: My ME is in remission|last=Brea|first=Jennifer|date=2019-05-20|website=Medium|access-date=2019-06-03}}</ref> They speculate that mechanical compression of the brainstem due to CCI has the potential to cause characteristic ME/CFS symptoms such as post-exertional malaise<ref>{{Cite web|url=https://www.mechanicalbasis.org/|title=Craniocervical instability, Atlantoaxial Instability, Myalgic Encephalomyelitis, ME, CFS|website=MEchanical Basis|language=en|access-date=2019-06-03}}</ref>, although this theory is currently not supported by scientific evidence. Others have raised concerns about CCI surgery in patients with ME/CFS given the lack of research on OCF in this patient population.<ref>{{Cite web|url=https://www.s4me.info/threads/concerns-about-craniocervical-instability-surgery-in-me-cfs.9638/|title=Concerns about craniocervical instability surgery in ME/CFS|website=Science for ME|language=en-US|access-date=2019-06-03}}</ref>

== Synonyms ==
* Syndrome of Occipitoatlantialaxial Hypermobility<ref name=":1" />
* Hypermobility of the Craniocervical Junction<ref>{{Cite journal|last=Mathers|first=K. Sean|last2=Schneider|first2=Michael|last3=Timko|first3=Michael|date=2011-6|title=Occult hypermobility of the craniocervical junction: a case report and review|url=https://www.ncbi.nlm.nih.gov/pubmed/21628827|journal=The Journal of Orthopaedic and Sports Physical Therapy|volume=41|issue=6|pages=444–457|doi=10.2519/jospt.2011.3305|issn=1938-1344|pmid=21628827}}</ref>

== See also ==

* [[Brainstem compression]]
* [[Ehlers-Danlos syndrome]]
* [[Chiari malformation]]
* [[Intracranial hypertension]]
* Atlantoaxial instability (AAI)
* [[Tethered cord syndrome]]

== Learn more ==

== References ==
<references />
[[Category:Diagnoses]]

Craniocervical instability

2019-06-06T11:08:28Z

Jeff:/* Symptoms */ Removed "incapacitating" descriptor, as this isn't supported by literature.

'''Craniocervical instability''' (CCI) is a pathological condition of increased mobility at the craniocervical junction, the area where the skull meets the spine. In CCI the ligamentous connections of the craniocervical junction can be stretched, weakened or ruptured.<ref name=":2">{{Cite journal|last=Henderson|first=Fraser C.|last2=Austin|first2=Claudiu|last3=Benzel|first3=Edward|last4=Bolognese|first4=Paolo|last5=Ellenbogen|first5=Richard|last6=Francomano|first6=Clair A.|last7=Ireton|first7=Candace|last8=Klinge|first8=Petra|last9=Koby|first9=Myles|date=2017|title=Neurological and spinal manifestations of the Ehlers–Danlos syndromes|url=https://onlinelibrary.wiley.com/doi/abs/10.1002/ajmg.c.31549|journal=American Journal of Medical Genetics Part C: Seminars in Medical Genetics|language=en|volume=175|issue=1|pages=195–211|doi=10.1002/ajmg.c.31549|issn=1552-4876}}</ref> This can lead to compression of the [[Brainstem compression|brainstem]], upper [[spinal cord]], or [[cerebellum]] and result in myelopathy, disabling neck pain and a range of other symptoms.<ref name=":11">{{Cite journal|last=Choi|first=Sung Ho|last2=Lee|first2=Sang Gu|last3=Park|first3=Chan Woo|last4=Kim|first4=Woo Kyung|last5=Yoo|first5=Chan Jong|last6=Son|first6=Seong|date=2013-4|title=Surgical Outcomes and Complications after Occipito-Cervical Fusion Using the Screw-Rod System in Craniocervical Instability|url=https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3698232/|journal=Journal of Korean Neurosurgical Society|volume=53|issue=4|pages=223–227|doi=10.3340/jkns.2013.53.4.223|issn=2005-3711|pmc=PMCPMC3698232|pmid=23826478}}</ref>

CCI usually develops as a result of physical trauma such as a car accident, an inflammation disease such as rheumatoid arthritis or a congenital disorder such as Down syndrome.<ref name=":10">{{Cite journal|last=Ashafai|first=Nabeel S.|last2=Visocchi|first2=Massimiliano|last3=Wąsik|first3=Norbert|date=2019|title=Occipitocervical Fusion: An Updated Review|url=https://www.ncbi.nlm.nih.gov/pubmed/30610329|journal=Acta Neurochirurgica. Supplement|volume=125|pages=247–252|doi=10.1007/978-3-319-62515-7_35|issn=0065-1419|pmid=30610329}}</ref> More recently, physicians have recognized an increased prevalence of CCI in patients with hereditary disorders of connective tissue such as Ehlers Danlos Syndromes (EDS).<ref name=":9">{{Cite web|url=https://www.researchgate.net/publication/302923107_Cranio-cervical_Instability_in_Patients_with_Hypermobility_Connective_Disorders|title=(PDF) Cranio-cervical Instability in Patients with Hypermobility Connective Disorders|website=ResearchGate|language=en|access-date=2019-06-03}}</ref> There have also been anecdotal reports of patients with myalgic encephalomyelitis (ME) who were diagnosed with CCI and improved markedly following craniocervical fusion surgery<ref>{{Cite web|url=https://forums.phoenixrising.me/index.php?threads/have-you-ruled-out-chiari-as-a-cause-of-your-cfs.56908/|title=Have you ruled out Chiari as a cause of your CFS|last=|first=|date=|website=Phoenix Rising|archive-url=|archive-date=|dead-url=|access-date=}}</ref><ref>{{Cite web|url=https://rsci.app.link/3LpJOxcTaX?_p=f3542c5bfc29c2616780177b27|website=rsci.app.link|access-date=2019-06-01}}</ref>, although scientific publications on this subject are currently lacking.
== Symptoms ==
Characteristic symptoms of craniocervical instability include neck pain and neurological abnormalities such as numbness, motor weakness and gait instability<ref>{{Cite journal|last=Bobinski|first=Lukas|last2=Levivier|first2=Marc|last3=Duff|first3=John M.|date=2015-2|title=Occipitoaxial spinal interarticular stabilization with vertebral artery preservation for atlantal lateral mass failure|url=https://www.ncbi.nlm.nih.gov/pubmed/25415481|journal=Journal of Neurosurgery. Spine|volume=22|issue=2|pages=134–138|doi=10.3171/2014.10.SPINE14131|issn=1547-5646|pmid=25415481}}</ref><ref>{{Cite journal|last=O'Brien|first=Michael F.|last2=Casey|first2=Adrian T. H.|last3=Crockard|first3=Alan|last4=Pringle|first4=Jean|last5=Stevens|first5=John M.|date=2002-10-15|title=Histology of the craniocervical junction in chronic rheumatoid arthritis: a clinicopathologic analysis of 33 operative cases|url=https://www.ncbi.nlm.nih.gov/pubmed/12394902|journal=Spine|volume=27|issue=20|pages=2245–2254|doi=10.1097/01.BRS.0000029252.98053.43|issn=1528-1159|pmid=12394902}}</ref><ref>{{Cite journal|last=Young|first=Richard M.|last2=Sherman|first2=Jonathan H.|last3=Wind|first3=Joshua J.|last4=Litvack|first4=Zachary|last5=O'Brien|first5=Joseph|date=2014-8|title=Treatment of craniocervical instability using a posterior-only approach: report of 3 cases|url=https://www.ncbi.nlm.nih.gov/pubmed/24785968|journal=Journal of Neurosurgery. Spine|volume=21|issue=2|pages=239–248|doi=10.3171/2014.3.SPINE13684|issn=1547-5646|pmid=24785968}}</ref><ref>{{Cite journal|last=Botelho|first=Ricardo V.|last2=Neto|first2=Eliseu B.|last3=Patriota|first3=Gustavo C.|last4=Daniel|first4=Jefferson W.|last5=Dumont|first5=Paulo A. S.|last6=Rotta|first6=José M.|date=2007-10|title=Basilar invagination: craniocervical instability treated with cervical traction and occipitocervical fixation. Case report|url=https://www.ncbi.nlm.nih.gov/pubmed/17933321|journal=Journal of Neurosurgery. Spine|volume=7|issue=4|pages=444–449|doi=10.3171/SPI-07/10/444|issn=1547-5654|pmid=17933321}}</ref><ref>{{Cite journal|last=Henderson|first=Fraser C.|last2=Henderson|first2=Fraser C.|last3=Wilson|first3=William A.|last4=Mark|first4=Alexander S.|last5=Koby|first5=Myles|date=2018-1|title=Utility of the clivo-axial angle in assessing brainstem deformity: pilot study and literature review|url=https://www.ncbi.nlm.nih.gov/pubmed/28258417|journal=Neurosurgical Review|volume=41|issue=1|pages=149–163|doi=10.1007/s10143-017-0830-3|issn=1437-2320|pmc=PMCPMC5748419|pmid=28258417}}</ref> although no particular symptom is mandatory for a diagnosis of CCI. Patients also frequently describe a constant pain at lower back of the head and the feeling that their head is too heavy for their neck to support (“bobble-head”)<ref name=":9" />

Other symptoms of CCI include:
* [[Muscle weakness]]<ref name=":0">{{Cite journal|last=Henderson|first=Fraser C.|last2=Francomano|first2=C. A.|last3=Koby|first3=M.|last4=Tuchman|first4=K.|last5=Adcock|first5=J.|last6=Patel|first6=S.|date=2019-01-09|title=Cervical medullary syndrome secondary to craniocervical instability and ventral brainstem compression in hereditary hypermobility connective tissue disorders: 5-year follow-up after craniocervical reduction, fusion, and stabilization|url=https://doi.org/10.1007/s10143-018-01070-4|journal=Neurosurgical Review|language=en|doi=10.1007/s10143-018-01070-4|issn=1437-2320}}</ref>, [[numbness]]<ref name=":1">{{Cite journal|last=Francomano|first=Clair A.|last2=McDonnell|first2=Nazli B.|last3=Nishikawa|first3=Misao|last4=Bolognese|first4=Paolo A.|last5=Milhorat|first5=Thomas H.|date=2007-12-01|title=Syndrome of occipitoatlantoaxial hypermobility, cranial settling, and Chiari malformation Type I in patients with hereditary disorders of connective tissue|url=https://thejns.org/view/journals/j-neurosurg-spine/7/6/article-p601.xml|journal=Journal of Neurosurgery: Spine|language=en-US|volume=7|issue=6|pages=601–609|doi=10.3171/SPI-07/12/601}}</ref><ref name=":2" /> [[paralysis]], parasthesias<ref name=":0" /><ref name=":4">{{Cite web|url=https://search.proquest.com/openview/34b3b18a8854c04ffa0fc50273d68313/1?pq-origsite=gscholar&cbl=47886|title=MRI video diagnosis and surgical therapy of soft tissue trauma to the craniocervical junction - ProQuest|website=search.proquest.com|language=en|access-date=2019-06-01}}</ref><ref name=":5">{{Cite journal|last=Rebbeck|first=Trudy|last2=Liebert|first2=Ann|date=2014-12-01|title=Clinical management of cranio-vertebral instability after whiplash, when guidelines should be adapted: A case report|url=http://www.sciencedirect.com/science/article/pii/S1356689X14000101|journal=Manual Therapy|volume=19|issue=6|pages=618–621|doi=10.1016/j.math.2014.01.009|issn=1356-689X}}</ref><ref name=":6">{{Cite journal|last=Mathers|first=K. Sean|last2=Schneider|first2=Michael|last3=Timko|first3=Michael|date=2011-06|title=Occult Hypermobility of the Craniocervical Junction: A Case Report and Review|url=https://www.jospt.org/doi/full/10.2519/jospt.2011.3305|journal=Journal of Orthopaedic & Sports Physical Therapy|language=en|volume=41|issue=6|pages=444–457|doi=10.2519/jospt.2011.3305|issn=0190-6011}}</ref>
* Poor proprioception<ref name=":7">{{Cite journal|last=Bergholm|first=Ulla|last2=Johansson|first2=Bengt H.|last3=Johansson|first3=Hakan|date=2004-01-01|title=New Diagnostic Tools Can Contribute to Better Treatment of Patients with Chronic Whiplash Disorders|url=https://doi.org/10.3109/J180v03n02_02|journal=Journal of Whiplash & Related Disorders|volume=3|issue=2|pages=5–19|doi=10.3109/J180v03n02_02|issn=1533-2888}}</ref>, impaired [[coordination]], gait changes<ref name=":0" /><ref name=":7" />
* [[Dizziness|Dizzinesss]], [[vertigo]]<ref name=":0" /><ref name=":2" /><ref name=":7" /><ref name=":4" /><ref name=":6" /><ref name=":5" /> syncope<ref name=":0" /><ref name=":1" /><ref name=":2" /><ref name=":7" />, nausea<ref name=":1" /><ref name=":2" />
* Neck stiffness, torticollis<ref name=":7" /><ref>{{Cite journal|last=Ghanem|first=Ismat|last2=El Hage|first2=Samer|last3=Rachkidi|first3=Rami|last4=Kharrat|first4=Khalil|last5=Dagher|first5=Fernand|last6=Kreichati|first6=Gabi|date=2008-03-01|title=Pediatric cervical spine instability|url=https://online.boneandjoint.org.uk/doi/full/10.1007/s11832-008-0092-2|journal=Journal of Children's Orthopaedics|volume=2|issue=2|pages=71–84|doi=10.1007/s11832-008-0092-2|issn=1863-2521|pmc=PMC2656787|pmid=19308585}}</ref> posterior scalp irritation<ref name=":7" />, facial pain, headache behind the eyes
* Apnea<ref>{{Cite journal|last=Janjua|first=M. Burhan|last2=Hwang|first2=Steven W.|last3=Samdani|first3=Amer F.|last4=Pahys|first4=Joshua M.|last5=Baaj|first5=Ali A.|last6=Härtl|first6=Roger|last7=Greenfield|first7=Jeffrey P.|date=2019-01-01|title=Instrumented arthrodesis for non-traumatic craniocervical instability in very young children|url=https://doi.org/10.1007/s00381-018-3876-9|journal=Child's Nervous System|language=en|volume=35|issue=1|pages=97–106|doi=10.1007/s00381-018-3876-9|issn=1433-0350}}</ref><ref name=":1" /><ref name=":3">{{Cite journal|last=Henderson|first=Fraser C.|last2=Austin|first2=Claudiu|last3=Benzel|first3=Edward|last4=Bolognese|first4=Paolo|last5=Ellenbogen|first5=Richard|last6=Francomano|first6=Clair A.|last7=Ireton|first7=Candace|last8=Klinge|first8=Petra|last9=Koby|first9=Myles|date=2017|title=Neurological and spinal manifestations of the Ehlers–Danlos syndromes|url=https://onlinelibrary.wiley.com/doi/abs/10.1002/ajmg.c.31549|journal=American Journal of Medical Genetics Part C: Seminars in Medical Genetics|language=en|volume=175|issue=1|pages=195–211|doi=10.1002/ajmg.c.31549|issn=1552-4876}}</ref>, dyspnea (shortness of breath)<ref name=":0" /><ref name=":1" /> and dysphagia (difficulty swallowing)<ref name=":1" /><ref name=":2" />
* Visual disturbances<ref name=":4" /> downward [[nystagmus]] (irregular eye movements)<ref name=":1" /><ref name=":7" /> tinnitus<ref>{{Cite journal|last=Montazem|first=Abbas|author-link=|author-link2=|author-link3=|author-link4=|author-link5=|date=2000|title=Secondary tinnitus as a symptom of instability in the upper cervical spine: Operative management|url=https://pdfs.semanticscholar.org/21c4/85984a6ebe07efed38cf82a2f7a49b2a644e.pdf|journal=International Tinnitus Journal|volume=|issue=|pages=|quote=|via=}}</ref><ref name=":4" />
* Fatigue<ref name=":0" /><ref>{{Cite journal|last=Henderson|first=Fraser C.|last2=Henderson|first2=Fraser C.|last3=Wilson|first3=William A.|last4=Mark|first4=Alexander S.|last5=Koby|first5=Myles|date=2018-01-01|title=Utility of the clivo-axial angle in assessing brainstem deformity: pilot study and literature review|url=https://doi.org/10.1007/s10143-017-0830-3|journal=Neurosurgical Review|language=en|volume=41|issue=1|pages=149–163|doi=10.1007/s10143-017-0830-3|issn=1437-2320|pmc=PMC5748419|pmid=28258417}}</ref><ref>{{Cite journal|last=Henderson|first=Fraser C.|last2=Wilson|first2=William A.|last3=Mott|first3=Stephen|last4=Mark|first4=Alexander|last5=Schmidt|first5=Kristi|last6=Berry|first6=Joel K.|last7=Vaccaro|first7=Alexander|last8=Benzel|first8=Edward|date=2010-07-16|title=Deformative stress associated with an abnormal clivo-axial angle: A finite element analysis|url=https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2940090/|journal=Surgical Neurology International|volume=1|doi=10.4103/2152-7806.66461|issn=2152-7806|pmc=PMCPMC2940090|pmid=20847911}}</ref> sleep disturbance<ref name=":0" /><ref name=":4" />cognitive impairment<ref name=":4" /> and memory loss.

== Risk factors and comorbidities ==
Established risk factors for CCI include physical trauma, inflammatory disease, congenital disorders, neoplasms and hereditary hypermobility connective tissue disorders.<ref name=":10" /><ref name=":12">{{Cite journal|last=Sapkas|first=George|last2=Papadakis|first2=Stamatios A|last3=Segkos|first3=Dimitrios|last4=Kateros|first4=Konstantinos|last5=Tsakotos|first5=George|last6=Katonis|first6=Pavlos|date=2011-06-02|title=Posterior Instrumentation for Occipitocervical Fusion|url=https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3139273/|journal=The Open Orthopaedics Journal|volume=5|pages=209–218|doi=10.2174/1874325001105010209|issn=1874-3250|pmc=PMCPMC3139273|pmid=21772931}}</ref>

{| class="wikitable"
|'''Cause of instability'''
|'''Example'''
|-
|Physical trauma<ref>{{Cite journal|last=Ghatan|first=Saadi|last2=Newell|first2=David W.|last3=Grady|first3=M. Sean|last4=Mirza|first4=Sohail K.|last5=Chapman|first5=Jens R.|last6=Mann|first6=Frederick A.|last7=Ellenbogen|first7=Richard G.|date=2004-8|title=Severe posttraumatic craniocervical instability in the very young patient. Report of three cases|url=https://www.ncbi.nlm.nih.gov/pubmed/16206980|journal=Journal of Neurosurgery|volume=101|issue=1 Suppl|pages=102–107|doi=10.3171/ped.2004.101.2.0102|issn=0022-3085|pmid=16206980}}</ref>
|Car accident<ref>{{Cite journal|last=Uribe|first=Juan S.|last2=Ramos|first2=Edwin|last3=Baaj|first3=Ali|last4=Youssef|first4=A. Samy|last5=Vale|first5=Fernando L.|date=2009-12|title=Occipital cervical stabilization using occipital condyles for cranial fixation: technical case report|url=https://www.ncbi.nlm.nih.gov/pubmed/19934947|journal=Neurosurgery|volume=65|issue=6|pages=E1216–1217; discussion E1217|doi=10.1227/01.NEU.0000349207.98394.FA|issn=1524-4040|pmid=19934947}}</ref><ref>{{Cite journal|last=Volle|first=E.|last2=Montazem|first2=A.|date=2001-1|title=MRI video diagnosis and surgical therapy of soft tissue trauma to the craniocervical junction|url=https://www.ncbi.nlm.nih.gov/pubmed/11209518|journal=Ear, Nose, & Throat Journal|volume=80|issue=1|pages=41–44, 46–48|issn=0145-5613|pmid=11209518}}</ref>, blow to the head.<ref>{{Cite journal|last=Mathers|first=K. Sean|last2=Schneider|first2=Michael|last3=Timko|first3=Michael|date=2011-6|title=Occult hypermobility of the craniocervical junction: a case report and review|url=https://www.ncbi.nlm.nih.gov/pubmed/21628827|journal=The Journal of Orthopaedic and Sports Physical Therapy|volume=41|issue=6|pages=444–457|doi=10.2519/jospt.2011.3305|issn=1938-1344|pmid=21628827}}</ref>
|-
|Inflammatory disease
|Rheumatoid arthritis<ref>{{Cite journal|last=O'Brien|first=Michael F.|last2=Casey|first2=Adrian T. H.|last3=Crockard|first3=Alan|last4=Pringle|first4=Jean|last5=Stevens|first5=John M.|date=2002-10-15|title=Histology of the craniocervical junction in chronic rheumatoid arthritis: a clinicopathologic analysis of 33 operative cases|url=https://www.ncbi.nlm.nih.gov/pubmed/12394902|journal=Spine|volume=27|issue=20|pages=2245–2254|doi=10.1097/01.BRS.0000029252.98053.43|issn=1528-1159|pmid=12394902}}</ref>, tuberculosis<ref>{{Cite journal|last=Chaudhary|first=Kshitij|last2=Potdar|first2=Prabodhan|last3=Bapat|first3=Mihir|last4=Rathod|first4=Ashok|last5=Laheri|first5=Vinod|date=2012-06-15|title=Structural odontoid lesions in craniovertebral tuberculosis: a review of 15 cases|url=https://www.ncbi.nlm.nih.gov/pubmed/22261632|journal=Spine|volume=37|issue=14|pages=E836–843|doi=10.1097/BRS.0b013e31824a4c8f|issn=1528-1159|pmid=22261632}}</ref>
|-
|Neoplasms
|Tumors<ref name=":12" /> such as haemangioma, aneurysmal bone cyst
|-
|Congenital
|Down’s syndrome<ref>{{Cite journal|last=Hankinson|first=Todd C.|last2=Anderson|first2=Richard C. E.|date=2010-3|title=Craniovertebral junction abnormalities in Down syndrome|url=https://www.ncbi.nlm.nih.gov/pubmed/20173525|journal=Neurosurgery|volume=66|issue=3 Suppl|pages=32–38|doi=10.1227/01.NEU.0000365803.22786.F0|issn=1524-4040|pmid=20173525}}</ref>, os odontoideum<ref>{{Cite journal|last=Zhao|first=Deng|last2=Wang|first2=Shenglin|last3=Passias|first3=Peter G.|last4=Wang|first4=Chao|date=2015-5|title=Craniocervical instability in the setting of os odontoideum: assessment of cause, presentation, and surgical outcomes in a series of 279 cases|url=https://www.ncbi.nlm.nih.gov/pubmed/25635883|journal=Neurosurgery|volume=76|issue=5|pages=514–521|doi=10.1227/NEU.0000000000000668|issn=1524-4040|pmid=25635883}}</ref>, dwarfism
|-
|Hereditary hypermobility connective tissue disorders
|Ehlers Danlos Syndromes<ref name=":0" /><ref name=":1" />
|}
It is not unusual for CCI to co-occur with other structural neurological abnormalities such as atlantoaxial instability (AAI) and chiari malformation (CM).<ref>{{Cite journal|last=Camino Willhuber|first=Gaston O.|last2=Bosio|first2=Santiago T.|last3=Puigdevall|first3=Miguel H.|last4=Halliburton|first4=Carolina|last5=Sola|first5=Carlos A.|last6=Maenza|first6=Ruben A.|date=2017-1|title=Craniocervical spinal instability after type 1 Arnold Chiari decompression: a case report|url=https://www.ncbi.nlm.nih.gov/pubmed/27258364|journal=Journal of Pediatric Orthopedics. Part B|volume=26|issue=1|pages=80–85|doi=10.1097/BPB.0000000000000346|issn=1473-5865|pmid=27258364}}</ref><ref name=":0" />

== Diagnosis ==
{{Video|id=https://youtu.be/MsYDA3SXTkg|service=youtube|dimensions=550|description=Dr. Paolo Bolognese discusses methods of imaging and measurement for diagnosis.|alignment=right|urlargs=}}
The diagnosis of CCI is based on symptom presentation, a supportive history, demonstrable neurological findings and abnormal imaging.

=== Imaging ===
CCI is typically diagnosed via a cervical MRI, whether supine or upright. If supine, a 3 Tesla MRI is preferred over a 1.5 Tesla. Most neurosurgeons prefer upright MRI with flexion and extension.<ref>{{Cite web|url=https://www.mechanicalbasis.org/diagnosis.html|title=Craniocervical instability, Atlantoaxial Instability, Myalgic Encephalomyelitis, ME, CFS|website=MEchanical Basis|language=en|access-date=2019-06-02}}</ref> According to Henderson FC, “ventral brainstem compression may exist in flexion of the cervical spine, but appear normal on routine imaging.”<ref name=":9" />

=== Measurements ===
More than twenty radiological measurements have been proposed or used in the diagnosis of CCI. However, three measurements are most commonly used: the Grabb-Oakes line, which measures focal compression; the Clivo-Axial Angle (CXA), which measures brainstem deformity by the odontoid process; and the Basion Dens Interval, which measures vertical instability (cranial settling). Acorrding to a 2013 consensus statement on the assessment of CCI a CXA of 135 degrees or less should be considered as "potential pathological."<ref>https://csfinfo.org/files/1613/9665/4797/Final_Booklet_complete.pdf</ref>

=== Traction ===
Manual traction and invasive cervical traction are often used to aid in the diagnosis of CCI. Symptomatic improvement with traction can help determine whether a patient will benefit from craniocervical fusion surgery.

== Treatment ==
{{Video|id=https://youtu.be/sEi9AlHQTJc|service=youtube|dimensions=550|description=Dr. Fraser Henderson presents the results a five-year follow-up study.|alignment=right|urlargs=}}
=== Conservative treatment ===
Treatment of CCI can include “conservative measures” such as rest, pain management, bracing with a cervical collar, or physical therapy to strengthen neck muscles.<ref>{{Cite journal|last=Mathers|first=K. Sean|last2=Schneider|first2=Michael|last3=Timko|first3=Michael|date=2011-6|title=Occult hypermobility of the craniocervical junction: a case report and review|url=https://www.ncbi.nlm.nih.gov/pubmed/21628827|journal=The Journal of Orthopaedic and Sports Physical Therapy|volume=41|issue=6|pages=444–457|doi=10.2519/jospt.2011.3305|issn=1938-1344|pmid=21628827}}</ref> Many conservative therapies have little to no supporting evidence of efficacy.

There is no evidence for the efficacy of experimental treatments of CCI such as prolotherapy and upper cervical chiropractic.

=== Surgery ===
When non-invasive treatments for CCI fail to work, occipito-cervical fusion (OCF) can be considered.<ref name=":0" /> OCF is a surgery that aims at a biomechanical stabilization of the craniocervical junction. Patients with objective radiological findings, a clinical picture supportive of the diagnosis, a positive response to traction, and who are significantly impaired may be candidates for this surgery. Different surgical procedures for OCF exist, but the current standard involves internal fixation of the upper spine by mechanical screws. Surgery typically involves using titanium hardware to fixate the occiput, axis and atlas (i.e., C0 to C2) along with rib graft or cadaver bone graft. When cervical instability is present below C2, additional vertebrae may also be fused if the patient is symptomatic.

==== Risks and complications ====
The outcome of OCF is generally favorable with most patients experiencing symptom relief post-surgery.<ref name=":0" /> The complications of OCF however can be serious<ref>{{Cite journal|last=Garrido|first=Ben J.|last2=Sasso|first2=Rick C.|date=2012-1|title=Occipitocervical fusion|url=https://www.ncbi.nlm.nih.gov/pubmed/22082624|journal=The Orthopedic Clinics of North America|volume=43|issue=1|pages=1–9, vii|doi=10.1016/j.ocl.2011.08.009|issn=1558-1373|pmid=22082624}}</ref> and occur in an estimated 10% to 33% of patients.<ref name=":10" /><ref>{{Cite journal|last=Winegar|first=Corbett D.|last2=Lawrence|first2=James P.|last3=Friel|first3=Brian C.|last4=Fernandez|first4=Carmella|last5=Hong|first5=Joseph|last6=Maltenfort|first6=Mitchell|last7=Anderson|first7=Paul A.|last8=Vaccaro|first8=Alexander R.|date=2010-7|title=A systematic review of occipital cervical fusion: techniques and outcomes|url=https://www.ncbi.nlm.nih.gov/pubmed/20594011|journal=Journal of Neurosurgery. Spine|volume=13|issue=1|pages=5–16|doi=10.3171/2010.3.SPINE08143|issn=1547-5646|pmid=20594011}}</ref><ref name=":11" /><ref>{{Cite journal|last=Ando|first=Kei|last2=Imagama|first2=Shiro|last3=Ito|first3=Zenya|last4=Kobayashi|first4=Kazuyoshi|last5=Yagi|first5=Hideki|last6=Shinjo|first6=Ryuichi|last7=Hida|first7=Tetsuro|last8=Ito|first8=Kenyu|last9=Ishikawa|first9=Yoshimoto|date=2017-6|title=Minimum 5-year Follow-up Results for Occipitocervical Fusion Using the Screw-Rod System in Craniocervical Instability|url=https://www.ncbi.nlm.nih.gov/pubmed/28525489|journal=Clinical Spine Surgery|volume=30|issue=5|pages=E628–E632|doi=10.1097/BSD.0000000000000199|issn=2380-0194|pmid=28525489}}</ref> Common complications include screw failure, wound infection, dural tear and cerebrospinal fluid leakage<ref name=":11" /> In some cases revision surgery is needed to treat infection or to remove hardware. Severe complications include meningitis and accidental injury of the vertebral artery by misplaced screws.

==== Side effects ====
OCF causes a substantial reduction in the neck’s range of motion, estimated at approximately 40% of total cervical flexion–extension.<ref name=":8">{{Cite journal|last=Ashafai|first=Nabeel S.|last2=Visocchi|first2=Massimiliano|last3=Wąsik|first3=Norbert|date=2019|editor-last=Visocchi|editor-first=Massimiliano|title=Occipitocervical Fusion: An Updated Review|url=https://doi.org/10.1007/978-3-319-62515-7_35|series=Acta Neurochirurgica Supplement|language=en|location=Cham|publisher=Springer International Publishing|pages=247–252|doi=10.1007/978-3-319-62515-7_35|isbn=9783319625157}}</ref>

==== Cost ====
OCF is estimated to cost tens of thousands of dollars, although some insurance schemes fully cover the cost of surgery depending on the country located and neurosurgeons involved.

== Dysautonomia and CCI in EDS ==
As CCI might lead to a compression of the brain stem, some researchers speculate this might cause some of the autonomic symptoms such as tachycardia and orthostatic intolerance that are frequently seen in patients with Ehlers Danlos Syndromes (EDS). In a 2007 influential paper Milhorat et al. followed-up on patients with Chiari malformation who did not improve with treatment and surgery. The authors discovered that many of these patients suffered from EDS and had other structural abnormalities at the upper spine such as CCI and cranial settling. Milhorat et al. speculated that the resulting compression of the brainstem might be the cause of the autonomic and other symptoms these patients were suffering from.<ref>{{Cite journal|last=Milhorat|first=Thomas H.|last2=Bolognese|first2=Paolo A.|last3=Nishikawa|first3=Misao|last4=McDonnell|first4=Nazli B.|last5=Francomano|first5=Clair A.|date=2007-12|title=Syndrome of occipitoatlantoaxial hypermobility, cranial settling, and chiari malformation type I in patients with hereditary disorders of connective tissue|url=https://www.ncbi.nlm.nih.gov/pubmed/18074684|journal=Journal of Neurosurgery. Spine|volume=7|issue=6|pages=601–609|doi=10.3171/SPI-07/12/601|issn=1547-5654|pmid=18074684}}</ref> In conference presentations, several neurosurgeons have indicated that they think CCI can cause dysautonomia symptoms such as postural orthostatic tachycardia sydrome (POTS) <ref>[https://www.youtube.com/watch?time_continue=735&v=857Jsjsqxjw Craniocervical Instability (Dr Henderson the 2012 EDNF Confrence).] Minute 12.10. </ref> [OTHER REFERENCE NEEDED].

Henderson et al. tested this theory by following 20 CCI patients with comorbid Chiari Malformation and hereditary hypermobility connective tissue disorders for a period of 5 years after OCF-surgery. Patients were satisfied with the surgery and experienced significant improvements in some CCI-related symptoms such as vertigo, headaches, imbalance, dysarthria dizziness or frequent daytime urination. There was however only a small increase in objective outcomes such as work resumption with 60% of patients remaining unable to work or go to school. Participants attributed this to other medical problems related to EDS such as musculoskeletal pain, fatigue, gastrointestinal issues and POTS, indicating these were not significantly improved after OCF-surgery.<ref name=":0" />

== Mechanical basis theory ==
Some ME/CFS patients diagnosed with CCI report to have experienced spectacular improvements and even remission of their ME/CFS symptoms following OCF-surgery.<ref>{{Cite web|url=https://medium.com/@jenbrea/health-update-3-my-me-is-in-remission-dd575e650f71|title=Health update #3: My ME is in remission|last=Brea|first=Jennifer|date=2019-05-20|website=Medium|access-date=2019-06-03}}</ref> They speculate that mechanical compression of the brainstem due to CCI has the potential to cause characteristic ME/CFS symptoms such as post-exertional malaise<ref>{{Cite web|url=https://www.mechanicalbasis.org/|title=Craniocervical instability, Atlantoaxial Instability, Myalgic Encephalomyelitis, ME, CFS|website=MEchanical Basis|language=en|access-date=2019-06-03}}</ref>, although this theory is currently not supported by scientific evidence. Others have raised concerns about CCI surgery in patients with ME/CFS given the lack of research on OCF in this patient population.<ref>{{Cite web|url=https://www.s4me.info/threads/concerns-about-craniocervical-instability-surgery-in-me-cfs.9638/|title=Concerns about craniocervical instability surgery in ME/CFS|website=Science for ME|language=en-US|access-date=2019-06-03}}</ref>

== Synonyms ==
* Syndrome of Occipitoatlantialaxial Hypermobility<ref name=":1" />
* Hypermobility of the Craniocervical Junction<ref>{{Cite journal|last=Mathers|first=K. Sean|last2=Schneider|first2=Michael|last3=Timko|first3=Michael|date=2011-6|title=Occult hypermobility of the craniocervical junction: a case report and review|url=https://www.ncbi.nlm.nih.gov/pubmed/21628827|journal=The Journal of Orthopaedic and Sports Physical Therapy|volume=41|issue=6|pages=444–457|doi=10.2519/jospt.2011.3305|issn=1938-1344|pmid=21628827}}</ref>

== See also ==

* [[Brainstem compression]]
* [[Ehlers-Danlos syndrome]]
* [[Chiari malformation]]
* [[Intracranial hypertension]]
* Atlantoaxial instability (AAI)
* [[Tethered cord syndrome]]

== Learn more ==

== References ==
<references />
[[Category:Diagnoses]]

Craniocervical instability

2019-06-02T04:13:24Z

Jeff:/* Surgery */

'''Craniocervical instability''' (CCI), also known as the Syndrome of Occipitoatlantialaxial Hypermobility, is a structural instability of the craniocervical junction which may lead to deformation or [[Brainstem compression|compression of the brainstem]], upper [[spinal cord]], and [[cerebellum]]. CCI as well as atlanto-axial instability (AAI), instability between the first and second vertebrae, can frequently co-occur. CCI/AAI can be caused by a traumatic injury, such as whiplash or a high-impact trauma. However, there are several known conditions where CCI/AAI can happen, seemingly spontaneously, in absence of obvious trauma: Down Syndrome, dwarfism, [[rheumatoid arthritis]] (RA), [[Ehlers-Danlos syndrome|Ehlers-Danlos Syndrome]] (EDS) (itself a common co-morbidity with [[myalgic encephalomyelitis|Myalgic Encephalomyelitis]] (ME)), and other [[connective tissue]] disorders. The ligaments of the craniocervical junction, which are made up of connective tissue, become too lax to hold the skull in normal relationship with the cervical spine.

There have been anecdotal reports of people with ME who meet the [[International Consensus Criteria]] (ICC) who were ultimately diagnosed with CCI/AAI and have had their symptoms improve or remit follow craniocervical fusion surgery.<ref>{{Cite web|url=https://forums.phoenixrising.me/index.php?threads/have-you-ruled-out-chiari-as-a-cause-of-your-cfs.56908/|title=Have you ruled out Chiari as a cause of your CFS|last=|first=|date=|website=Phoenix Rising|archive-url=|archive-date=|dead-url=|access-date=}}</ref><ref>{{Cite web|url=https://www.healthrising.org/blog/2019/05/21/jennifer-brea-chronic-fatigue-mecfs-recovering-story/|title=Jennifer Brea's Amazing ME/CFS Recovering Story: the Spinal Series - Pt. II|last=Johnson|first=Cort|date=2019-05-21|website=Health Rising|language=en-GB|access-date=2019-06-01}}</ref><ref>{{Cite web|url=https://rsci.app.link/3LpJOxcTaX?_p=f3542c5bfc29c2616780177b27|website=rsci.app.link|access-date=2019-06-01}}</ref><ref>{{Cite web|url=https://www.mechanicalbasis.org/|title=Craniocervical instability, Atlantoaxial Instability, Myalgic Encephalomyelitis, ME, CFS|website=MEchanical Basis|language=en|access-date=2019-06-01}}</ref> However, thus far, any potential connection between CCI and ME remains essentially unexplored: there has been no formal research investigating the relationship between CCI and ME.

== Symptoms ==
There can be a wide range of clinical presentations. Symptoms<ref>{{Cite web|url=http://thezebranetwork.org/craniocervical-instability/|title=Craniocervical Instability|last=|first=|date=|website=The Zebra Network|archive-url=|archive-date=|dead-url=|access-date=}}</ref> of craniocervical instability and atlantoaxial instability, particularly in the context of a connective tissue disorder, can and ''may'' include:
* [[headache]]<ref name=":2">{{Cite journal|last=Henderson|first=Fraser C.|last2=Austin|first2=Claudiu|last3=Benzel|first3=Edward|last4=Bolognese|first4=Paolo|last5=Ellenbogen|first5=Richard|last6=Francomano|first6=Clair A.|last7=Ireton|first7=Candace|last8=Klinge|first8=Petra|last9=Koby|first9=Myles|date=2017|title=Neurological and spinal manifestations of the Ehlers–Danlos syndromes|url=https://onlinelibrary.wiley.com/doi/abs/10.1002/ajmg.c.31549|journal=American Journal of Medical Genetics Part C: Seminars in Medical Genetics|language=en|volume=175|issue=1|pages=195–211|doi=10.1002/ajmg.c.31549|issn=1552-4876}}</ref><ref name=":0">{{Cite journal|last=Henderson|first=Fraser C.|last2=Francomano|first2=C. A.|last3=Koby|first3=M.|last4=Tuchman|first4=K.|last5=Adcock|first5=J.|last6=Patel|first6=S.|date=2019-01-09|title=Cervical medullary syndrome secondary to craniocervical instability and ventral brainstem compression in hereditary hypermobility connective tissue disorders: 5-year follow-up after craniocervical reduction, fusion, and stabilization|url=https://doi.org/10.1007/s10143-018-01070-4|journal=Neurosurgical Review|language=en|doi=10.1007/s10143-018-01070-4|issn=1437-2320}}</ref><ref name=":5">{{Cite journal|last=Rebbeck|first=Trudy|last2=Liebert|first2=Ann|date=2014-12-01|title=Clinical management of cranio-vertebral instability after whiplash, when guidelines should be adapted: A case report|url=http://www.sciencedirect.com/science/article/pii/S1356689X14000101|journal=Manual Therapy|volume=19|issue=6|pages=618–621|doi=10.1016/j.math.2014.01.009|issn=1356-689X}}</ref>: common types include an occipital headache or occipital pain (back lower part of the head)<ref name=":4">{{Cite web|url=https://search.proquest.com/openview/34b3b18a8854c04ffa0fc50273d68313/1?pq-origsite=gscholar&cbl=47886|title=MRI video diagnosis and surgical therapy of soft tissue trauma to the craniocervical junction - ProQuest|website=search.proquest.com|language=en|access-date=2019-06-01}}</ref><ref name=":6">{{Cite journal|last=Mathers|first=K. Sean|last2=Schneider|first2=Michael|last3=Timko|first3=Michael|date=2011-06|title=Occult Hypermobility of the Craniocervical Junction: A Case Report and Review|url=https://www.jospt.org/doi/full/10.2519/jospt.2011.3305|journal=Journal of Orthopaedic & Sports Physical Therapy|language=en|volume=41|issue=6|pages=444–457|doi=10.2519/jospt.2011.3305|issn=0190-6011}}</ref>; a pressure headache: an impairment of [[cerebropinsal fluid]] (CSF) flow causes intracranial pressure which would be aggravated by “valsalva maneuvers” such as yawning, laughing, crying, coughing, sneezing or straining; headache behind the eyes<ref name=":6" />; and a feeling that head is too heavy for the neck to support (feeling like a “bobble-head”).
* [[Dysautonomia]]<ref name=":0" /><ref name=":3">{{Cite journal|last=Henderson|first=Fraser C.|last2=Austin|first2=Claudiu|last3=Benzel|first3=Edward|last4=Bolognese|first4=Paolo|last5=Ellenbogen|first5=Richard|last6=Francomano|first6=Clair A.|last7=Ireton|first7=Candace|last8=Klinge|first8=Petra|last9=Koby|first9=Myles|date=2017|title=Neurological and spinal manifestations of the Ehlers–Danlos syndromes|url=https://onlinelibrary.wiley.com/doi/abs/10.1002/ajmg.c.31549|journal=American Journal of Medical Genetics Part C: Seminars in Medical Genetics|language=en|volume=175|issue=1|pages=195–211|doi=10.1002/ajmg.c.31549|issn=1552-4876}}</ref> (e.g., [[heat intolerance]], [[orthostatic intolerance]]<ref name=":1">{{Cite journal|last=Francomano|first=Clair A.|last2=McDonnell|first2=Nazli B.|last3=Nishikawa|first3=Misao|last4=Bolognese|first4=Paolo A.|last5=Milhorat|first5=Thomas H.|date=2007-12-01|title=Syndrome of occipitoatlantoaxial hypermobility, cranial settling, and Chiari malformation Type I in patients with hereditary disorders of connective tissue|url=https://thejns.org/view/journals/j-neurosurg-spine/7/6/article-p601.xml|journal=Journal of Neurosurgery: Spine|language=en-US|volume=7|issue=6|pages=601–609|doi=10.3171/SPI-07/12/601}}</ref>, [[polydipsia]] (extreme thirst), [[delayed gastric emptying]])

* [[Postural orthostatic tachycardia syndrome|postural orthostatic tachycardia]]<ref name=":0" /><ref name=":1" />
* dizziness<ref name=":4" /><ref name=":5" /><ref name=":6" />
* fatigue<ref name=":0" /><ref>{{Cite journal|last=Henderson|first=Fraser C.|last2=Henderson|first2=Fraser C.|last3=Wilson|first3=William A.|last4=Mark|first4=Alexander S.|last5=Koby|first5=Myles|date=2018-01-01|title=Utility of the clivo-axial angle in assessing brainstem deformity: pilot study and literature review|url=https://doi.org/10.1007/s10143-017-0830-3|journal=Neurosurgical Review|language=en|volume=41|issue=1|pages=149–163|doi=10.1007/s10143-017-0830-3|issn=1437-2320|pmc=PMC5748419|pmid=28258417}}</ref><ref>{{Cite journal|last=Henderson|first=Fraser C.|last2=Wilson|first2=William A.|last3=Mott|first3=Stephen|last4=Mark|first4=Alexander|last5=Schmidt|first5=Kristi|last6=Berry|first6=Joel K.|last7=Vaccaro|first7=Alexander|last8=Benzel|first8=Edward|date=2010-07-16|title=Deformative stress associated with an abnormal clivo-axial angle: A finite element analysis|url=https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2940090/|journal=Surgical Neurology International|volume=1|doi=10.4103/2152-7806.66461|issn=2152-7806|pmc=PMCPMC2940090|pmid=20847911}}</ref>
* cognitive impairment<ref name=":4" />
* [[muscle weakness]]<ref name=":0" />
* GERD<ref name=":0" />

* posterior [[neck pain]]<ref name=":0" /><ref name=":7">{{Cite journal|last=Bergholm|first=Ulla|last2=Johansson|first2=Bengt H.|last3=Johansson|first3=Hakan|date=2004-01-01|title=New Diagnostic Tools Can Contribute to Better Treatment of Patients with Chronic Whiplash Disorders|url=https://doi.org/10.3109/J180v03n02_02|journal=Journal of Whiplash & Related Disorders|volume=3|issue=2|pages=5–19|doi=10.3109/J180v03n02_02|issn=1533-2888}}</ref><ref name=":5" /><ref name=":6" />
* posterior scalp irritation<ref name=":7" />
* neck stiffness, torticollis<ref name=":7" /><ref>{{Cite journal|last=Ghanem|first=Ismat|last2=El Hage|first2=Samer|last3=Rachkidi|first3=Rami|last4=Kharrat|first4=Khalil|last5=Dagher|first5=Fernand|last6=Kreichati|first6=Gabi|date=2008-03-01|title=Pediatric cervical spine instability|url=https://online.boneandjoint.org.uk/doi/full/10.1007/s11832-008-0092-2|journal=Journal of Children's Orthopaedics|volume=2|issue=2|pages=71–84|doi=10.1007/s11832-008-0092-2|issn=1863-2521|pmc=PMC2656787|pmid=19308585}}</ref>
* tinnitus<ref>{{Cite journal|last=Montazem|first=Abbas|author-link=|author-link2=|author-link3=|author-link4=|author-link5=|date=2000|title=Secondary tinnitus as a symptom of instability in the upper cervical spine: Operative management|url=https://pdfs.semanticscholar.org/21c4/85984a6ebe07efed38cf82a2f7a49b2a644e.pdf|journal=International Tinnitus Journal|volume=|issue=|pages=|quote=|via=}}</ref><ref name=":4" />
* Irritable bowel symptoms<ref name=":0" />
* central or mixed [[apnea]]<ref>{{Cite journal|last=Janjua|first=M. Burhan|last2=Hwang|first2=Steven W.|last3=Samdani|first3=Amer F.|last4=Pahys|first4=Joshua M.|last5=Baaj|first5=Ali A.|last6=Härtl|first6=Roger|last7=Greenfield|first7=Jeffrey P.|date=2019-01-01|title=Instrumented arthrodesis for non-traumatic craniocervical instability in very young children|url=https://doi.org/10.1007/s00381-018-3876-9|journal=Child's Nervous System|language=en|volume=35|issue=1|pages=97–106|doi=10.1007/s00381-018-3876-9|issn=1433-0350}}</ref><ref name=":1" /><ref name=":3" />
* dyspnea (shortness of breath)<ref name=":0" /><ref name=":1" />
* dysphagia (difficulty swallowing)<ref name=":1" /><ref name=":2" />
* altered sleep architecture/sleep disturbance<ref name=":0" /><ref name=":4" />
* facial pain or [[numbness]]<ref name=":1" /><ref name=":2" />
* [[dizziness]] and [[vertigo]]<ref name=":0" /><ref name=":2" /><ref name=":7" />
* syncope<ref name=":0" /><ref name=":1" /><ref name=":2" /><ref name=":7" />
* visual disturbances<ref name=":4" />
* reduced gag reflex and [[Dysphagia|difficulty swallowing]]
* impaired [[coordination]]
* downward [[nystagmus]] (irregular eye movements)<ref name=":1" /><ref name=":7" />
* [[paralysis]]
* parasthesias<ref name=":0" /><ref name=":4" /><ref name=":5" /><ref name=":6" />
* gait changes<ref name=":0" /><ref name=":7" />
* poor proprioception<ref name=":7" />
* nausea<ref name=":1" /><ref name=":2" />

== Diagnosis ==
{{Video|id=https://youtu.be/MsYDA3SXTkg|service=youtube|dimensions=550|description=Dr. Paolo Bolognese discusses methods of imaging and measurement for diagnosis.|alignment=right|urlargs=}}
Craniocervical instability is diagnosed via radiological imaging of the craniocervical junction.

=== Imaging ===
CCI is typically diagnosed via a cervical MRI, whether supine or upright. If supine, a 3 Tesla MRI is preferred over a 1.5 Tesla. Most neurosurgeons prefer upright MRI with flexion and extension.<ref>{{Cite web|url=https://www.mechanicalbasis.org/diagnosis.html|title=Craniocervical instability, Atlantoaxial Instability, Myalgic Encephalomyelitis, ME, CFS|website=MEchanical Basis|language=en|access-date=2019-06-02}}</ref>

=== Measurements ===
More than twenty radiological measurements have been proposed or used in the diagnosis of CCI. However, three measurements are most commonly used: the Grabb-Oakes line, which measures focal compression; the Clivo-Axial Angle, which measures brainstem deformity by the odontoid process; and the Basion Dens Interval, which measures vertical instability (cranial settling).

=== Traction ===
Manual traction and invasive cervical traction are often used to aid in the diagnosis of CCI. Symptomatic improvement with traction can help determine whether a patient will benefit from craniocervical fusion surgery.

== Treatment ==
{{Video|id=https://youtu.be/sEi9AlHQTJc|service=youtube|dimensions=550|description=Dr. Fraser Henderson presents the results a five-year follow-up study.|alignment=right|urlargs=}}
=== Conservative treatment ===
Treatment can include “conservative measures” such as rest, bracing with a cervical collar, or physical therapy to strengthen neck muscles. Experimental treatments include stem cell therapy, prolotherapy and upper cervical Chiropractic. Many conservative therapies have little to no supporting evidence of efficacy.

=== Surgery ===
Patients with objective radiological findings, a clinical picture supportive of the diagnosis, a positive response to traction, and who are significantly impaired may be candidates for surgery. Surgery is undertaken as a last resort after conservative measurements have failed. Surgery typically involves using titanium hardware in the occiput, axis and atlas (i.e., C0 to C2) along with rib graft or cadaver bone craft. When cervical instability is present below C2, additional vertebrae may also be fused if the patient is symptomatic.

A five-year follow up study of twenty Ehlers-Danlos Syndrome patients who underwent craniocervical fusion surgery found no deaths or major post-surgical complications.<ref name=":0" /> 25% of patients later required fusion at another level.<ref name=":0" /> A study of 279 patients undergoing craniocervical fusion for CCI with os odontoideum due to trauma and congenital abnormalities found a 2.4% complication rate.<ref>{{Cite journal|last=Wang|first=Chao|last2=Passias|first2=Peter G.|last3=Wang|first3=Shenglin|last4=Zhao|first4=Deng|date=2015-05-01|title=Craniocervical Instability in the Setting of Os OdontoideumAssessment of Cause, Presentation, and Surgical Outcomes in a Series of 279 Cases|url=https://academic.oup.com/neurosurgery/article/76/5/514/2452018|journal=Neurosurgery|language=en|volume=76|issue=5|pages=514–521|doi=10.1227/NEU.0000000000000668|issn=0148-396X}}</ref>

== Risk factors ==
Patients with [[Ehlers-Danlos syndrome]], Down Syndrome, dwarfism and [[Rheumatoid arthritis]] are at greater risk.

== Co-morbid conditions ==
CCI can co-occur in patients with EDS and other connective tissue disorders with [[intracranial hypertension]]<ref name=":0" /> or [[Intracranial hypotension|hypotension]]<ref name=":0" />, abnormalities of intracranial venous drainage due to sinus stenosis or jugular vein stenosis,<ref name=":0" /> [[Migraine]] headaches, [[Temporomandibular joint disorder|temporomandibular joint]] dysfunction<ref name=":0" /><ref name=":1" />, [[tethered cord syndrome]], and scoliosis.<ref name=":1" />

== See also ==

* [[Brainstem compression]]
* [[Ehlers-Danlos syndrome]]
* [[Chiari malformation]]

== Learn more ==

== References ==
<references />
[[Category:Diagnoses]]

Craniocervical instability

2019-06-02T04:11:01Z

Jeff:/* Traction */

'''Craniocervical instability''' (CCI), also known as the Syndrome of Occipitoatlantialaxial Hypermobility, is a structural instability of the craniocervical junction which may lead to deformation or [[Brainstem compression|compression of the brainstem]], upper [[spinal cord]], and [[cerebellum]]. CCI as well as atlanto-axial instability (AAI), instability between the first and second vertebrae, can frequently co-occur. CCI/AAI can be caused by a traumatic injury, such as whiplash or a high-impact trauma. However, there are several known conditions where CCI/AAI can happen, seemingly spontaneously, in absence of obvious trauma: Down Syndrome, dwarfism, [[rheumatoid arthritis]] (RA), [[Ehlers-Danlos syndrome|Ehlers-Danlos Syndrome]] (EDS) (itself a common co-morbidity with [[myalgic encephalomyelitis|Myalgic Encephalomyelitis]] (ME)), and other [[connective tissue]] disorders. The ligaments of the craniocervical junction, which are made up of connective tissue, become too lax to hold the skull in normal relationship with the cervical spine.

There have been anecdotal reports of people with ME who meet the [[International Consensus Criteria]] (ICC) who were ultimately diagnosed with CCI/AAI and have had their symptoms improve or remit follow craniocervical fusion surgery.<ref>{{Cite web|url=https://forums.phoenixrising.me/index.php?threads/have-you-ruled-out-chiari-as-a-cause-of-your-cfs.56908/|title=Have you ruled out Chiari as a cause of your CFS|last=|first=|date=|website=Phoenix Rising|archive-url=|archive-date=|dead-url=|access-date=}}</ref><ref>{{Cite web|url=https://www.healthrising.org/blog/2019/05/21/jennifer-brea-chronic-fatigue-mecfs-recovering-story/|title=Jennifer Brea's Amazing ME/CFS Recovering Story: the Spinal Series - Pt. II|last=Johnson|first=Cort|date=2019-05-21|website=Health Rising|language=en-GB|access-date=2019-06-01}}</ref><ref>{{Cite web|url=https://rsci.app.link/3LpJOxcTaX?_p=f3542c5bfc29c2616780177b27|website=rsci.app.link|access-date=2019-06-01}}</ref><ref>{{Cite web|url=https://www.mechanicalbasis.org/|title=Craniocervical instability, Atlantoaxial Instability, Myalgic Encephalomyelitis, ME, CFS|website=MEchanical Basis|language=en|access-date=2019-06-01}}</ref> However, thus far, any potential connection between CCI and ME remains essentially unexplored: there has been no formal research investigating the relationship between CCI and ME.

== Symptoms ==
There can be a wide range of clinical presentations. Symptoms<ref>{{Cite web|url=http://thezebranetwork.org/craniocervical-instability/|title=Craniocervical Instability|last=|first=|date=|website=The Zebra Network|archive-url=|archive-date=|dead-url=|access-date=}}</ref> of craniocervical instability and atlantoaxial instability, particularly in the context of a connective tissue disorder, can and ''may'' include:
* [[headache]]<ref name=":2">{{Cite journal|last=Henderson|first=Fraser C.|last2=Austin|first2=Claudiu|last3=Benzel|first3=Edward|last4=Bolognese|first4=Paolo|last5=Ellenbogen|first5=Richard|last6=Francomano|first6=Clair A.|last7=Ireton|first7=Candace|last8=Klinge|first8=Petra|last9=Koby|first9=Myles|date=2017|title=Neurological and spinal manifestations of the Ehlers–Danlos syndromes|url=https://onlinelibrary.wiley.com/doi/abs/10.1002/ajmg.c.31549|journal=American Journal of Medical Genetics Part C: Seminars in Medical Genetics|language=en|volume=175|issue=1|pages=195–211|doi=10.1002/ajmg.c.31549|issn=1552-4876}}</ref><ref name=":0">{{Cite journal|last=Henderson|first=Fraser C.|last2=Francomano|first2=C. A.|last3=Koby|first3=M.|last4=Tuchman|first4=K.|last5=Adcock|first5=J.|last6=Patel|first6=S.|date=2019-01-09|title=Cervical medullary syndrome secondary to craniocervical instability and ventral brainstem compression in hereditary hypermobility connective tissue disorders: 5-year follow-up after craniocervical reduction, fusion, and stabilization|url=https://doi.org/10.1007/s10143-018-01070-4|journal=Neurosurgical Review|language=en|doi=10.1007/s10143-018-01070-4|issn=1437-2320}}</ref><ref name=":5">{{Cite journal|last=Rebbeck|first=Trudy|last2=Liebert|first2=Ann|date=2014-12-01|title=Clinical management of cranio-vertebral instability after whiplash, when guidelines should be adapted: A case report|url=http://www.sciencedirect.com/science/article/pii/S1356689X14000101|journal=Manual Therapy|volume=19|issue=6|pages=618–621|doi=10.1016/j.math.2014.01.009|issn=1356-689X}}</ref>: common types include an occipital headache or occipital pain (back lower part of the head)<ref name=":4">{{Cite web|url=https://search.proquest.com/openview/34b3b18a8854c04ffa0fc50273d68313/1?pq-origsite=gscholar&cbl=47886|title=MRI video diagnosis and surgical therapy of soft tissue trauma to the craniocervical junction - ProQuest|website=search.proquest.com|language=en|access-date=2019-06-01}}</ref><ref name=":6">{{Cite journal|last=Mathers|first=K. Sean|last2=Schneider|first2=Michael|last3=Timko|first3=Michael|date=2011-06|title=Occult Hypermobility of the Craniocervical Junction: A Case Report and Review|url=https://www.jospt.org/doi/full/10.2519/jospt.2011.3305|journal=Journal of Orthopaedic & Sports Physical Therapy|language=en|volume=41|issue=6|pages=444–457|doi=10.2519/jospt.2011.3305|issn=0190-6011}}</ref>; a pressure headache: an impairment of [[cerebropinsal fluid]] (CSF) flow causes intracranial pressure which would be aggravated by “valsalva maneuvers” such as yawning, laughing, crying, coughing, sneezing or straining; headache behind the eyes<ref name=":6" />; and a feeling that head is too heavy for the neck to support (feeling like a “bobble-head”).
* [[Dysautonomia]]<ref name=":0" /><ref name=":3">{{Cite journal|last=Henderson|first=Fraser C.|last2=Austin|first2=Claudiu|last3=Benzel|first3=Edward|last4=Bolognese|first4=Paolo|last5=Ellenbogen|first5=Richard|last6=Francomano|first6=Clair A.|last7=Ireton|first7=Candace|last8=Klinge|first8=Petra|last9=Koby|first9=Myles|date=2017|title=Neurological and spinal manifestations of the Ehlers–Danlos syndromes|url=https://onlinelibrary.wiley.com/doi/abs/10.1002/ajmg.c.31549|journal=American Journal of Medical Genetics Part C: Seminars in Medical Genetics|language=en|volume=175|issue=1|pages=195–211|doi=10.1002/ajmg.c.31549|issn=1552-4876}}</ref> (e.g., [[heat intolerance]], [[orthostatic intolerance]]<ref name=":1">{{Cite journal|last=Francomano|first=Clair A.|last2=McDonnell|first2=Nazli B.|last3=Nishikawa|first3=Misao|last4=Bolognese|first4=Paolo A.|last5=Milhorat|first5=Thomas H.|date=2007-12-01|title=Syndrome of occipitoatlantoaxial hypermobility, cranial settling, and Chiari malformation Type I in patients with hereditary disorders of connective tissue|url=https://thejns.org/view/journals/j-neurosurg-spine/7/6/article-p601.xml|journal=Journal of Neurosurgery: Spine|language=en-US|volume=7|issue=6|pages=601–609|doi=10.3171/SPI-07/12/601}}</ref>, [[polydipsia]] (extreme thirst), [[delayed gastric emptying]])

* [[Postural orthostatic tachycardia syndrome|postural orthostatic tachycardia]]<ref name=":0" /><ref name=":1" />
* dizziness<ref name=":4" /><ref name=":5" /><ref name=":6" />
* fatigue<ref name=":0" /><ref>{{Cite journal|last=Henderson|first=Fraser C.|last2=Henderson|first2=Fraser C.|last3=Wilson|first3=William A.|last4=Mark|first4=Alexander S.|last5=Koby|first5=Myles|date=2018-01-01|title=Utility of the clivo-axial angle in assessing brainstem deformity: pilot study and literature review|url=https://doi.org/10.1007/s10143-017-0830-3|journal=Neurosurgical Review|language=en|volume=41|issue=1|pages=149–163|doi=10.1007/s10143-017-0830-3|issn=1437-2320|pmc=PMC5748419|pmid=28258417}}</ref><ref>{{Cite journal|last=Henderson|first=Fraser C.|last2=Wilson|first2=William A.|last3=Mott|first3=Stephen|last4=Mark|first4=Alexander|last5=Schmidt|first5=Kristi|last6=Berry|first6=Joel K.|last7=Vaccaro|first7=Alexander|last8=Benzel|first8=Edward|date=2010-07-16|title=Deformative stress associated with an abnormal clivo-axial angle: A finite element analysis|url=https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2940090/|journal=Surgical Neurology International|volume=1|doi=10.4103/2152-7806.66461|issn=2152-7806|pmc=PMCPMC2940090|pmid=20847911}}</ref>
* cognitive impairment<ref name=":4" />
* [[muscle weakness]]<ref name=":0" />
* GERD<ref name=":0" />

* posterior [[neck pain]]<ref name=":0" /><ref name=":7">{{Cite journal|last=Bergholm|first=Ulla|last2=Johansson|first2=Bengt H.|last3=Johansson|first3=Hakan|date=2004-01-01|title=New Diagnostic Tools Can Contribute to Better Treatment of Patients with Chronic Whiplash Disorders|url=https://doi.org/10.3109/J180v03n02_02|journal=Journal of Whiplash & Related Disorders|volume=3|issue=2|pages=5–19|doi=10.3109/J180v03n02_02|issn=1533-2888}}</ref><ref name=":5" /><ref name=":6" />
* posterior scalp irritation<ref name=":7" />
* neck stiffness, torticollis<ref name=":7" /><ref>{{Cite journal|last=Ghanem|first=Ismat|last2=El Hage|first2=Samer|last3=Rachkidi|first3=Rami|last4=Kharrat|first4=Khalil|last5=Dagher|first5=Fernand|last6=Kreichati|first6=Gabi|date=2008-03-01|title=Pediatric cervical spine instability|url=https://online.boneandjoint.org.uk/doi/full/10.1007/s11832-008-0092-2|journal=Journal of Children's Orthopaedics|volume=2|issue=2|pages=71–84|doi=10.1007/s11832-008-0092-2|issn=1863-2521|pmc=PMC2656787|pmid=19308585}}</ref>
* tinnitus<ref>{{Cite journal|last=Montazem|first=Abbas|author-link=|author-link2=|author-link3=|author-link4=|author-link5=|date=2000|title=Secondary tinnitus as a symptom of instability in the upper cervical spine: Operative management|url=https://pdfs.semanticscholar.org/21c4/85984a6ebe07efed38cf82a2f7a49b2a644e.pdf|journal=International Tinnitus Journal|volume=|issue=|pages=|quote=|via=}}</ref><ref name=":4" />
* Irritable bowel symptoms<ref name=":0" />
* central or mixed [[apnea]]<ref>{{Cite journal|last=Janjua|first=M. Burhan|last2=Hwang|first2=Steven W.|last3=Samdani|first3=Amer F.|last4=Pahys|first4=Joshua M.|last5=Baaj|first5=Ali A.|last6=Härtl|first6=Roger|last7=Greenfield|first7=Jeffrey P.|date=2019-01-01|title=Instrumented arthrodesis for non-traumatic craniocervical instability in very young children|url=https://doi.org/10.1007/s00381-018-3876-9|journal=Child's Nervous System|language=en|volume=35|issue=1|pages=97–106|doi=10.1007/s00381-018-3876-9|issn=1433-0350}}</ref><ref name=":1" /><ref name=":3" />
* dyspnea (shortness of breath)<ref name=":0" /><ref name=":1" />
* dysphagia (difficulty swallowing)<ref name=":1" /><ref name=":2" />
* altered sleep architecture/sleep disturbance<ref name=":0" /><ref name=":4" />
* facial pain or [[numbness]]<ref name=":1" /><ref name=":2" />
* [[dizziness]] and [[vertigo]]<ref name=":0" /><ref name=":2" /><ref name=":7" />
* syncope<ref name=":0" /><ref name=":1" /><ref name=":2" /><ref name=":7" />
* visual disturbances<ref name=":4" />
* reduced gag reflex and [[Dysphagia|difficulty swallowing]]
* impaired [[coordination]]
* downward [[nystagmus]] (irregular eye movements)<ref name=":1" /><ref name=":7" />
* [[paralysis]]
* parasthesias<ref name=":0" /><ref name=":4" /><ref name=":5" /><ref name=":6" />
* gait changes<ref name=":0" /><ref name=":7" />
* poor proprioception<ref name=":7" />
* nausea<ref name=":1" /><ref name=":2" />

== Diagnosis ==
{{Video|id=https://youtu.be/MsYDA3SXTkg|service=youtube|dimensions=550|description=Dr. Paolo Bolognese discusses methods of imaging and measurement for diagnosis.|alignment=right|urlargs=}}
Craniocervical instability is diagnosed via radiological imaging of the craniocervical junction.

=== Imaging ===
CCI is typically diagnosed via a cervical MRI, whether supine or upright. If supine, a 3 Tesla MRI is preferred over a 1.5 Tesla. Most neurosurgeons prefer upright MRI with flexion and extension.<ref>{{Cite web|url=https://www.mechanicalbasis.org/diagnosis.html|title=Craniocervical instability, Atlantoaxial Instability, Myalgic Encephalomyelitis, ME, CFS|website=MEchanical Basis|language=en|access-date=2019-06-02}}</ref>

=== Measurements ===
More than twenty radiological measurements have been proposed or used in the diagnosis of CCI. However, three measurements are most commonly used: the Grabb-Oakes line, which measures focal compression; the Clivo-Axial Angle, which measures brainstem deformity by the odontoid process; and the Basion Dens Interval, which measures vertical instability (cranial settling).

=== Traction ===
Manual traction and invasive cervical traction are often used to aid in the diagnosis of CCI. Symptomatic improvement with traction can help determine whether a patient will benefit from craniocervical fusion surgery.

== Treatment ==
{{Video|id=https://youtu.be/sEi9AlHQTJc|service=youtube|dimensions=550|description=Dr. Fraser Henderson presents the results a five-year follow-up study.|alignment=right|urlargs=}}
=== Conservative treatment ===
Treatment can include “conservative measures” such as rest, bracing with a cervical collar, or physical therapy to strengthen neck muscles. Experimental treatments include stem cell therapy, prolotherapy and upper cervical Chiropractic. Many conservative therapies have little to no supporting evidence of efficacy.

=== Surgery ===
Patients with objective radiological findings, a clinical picture supportive of the diagnosis, positive response to traction, and who are significantly impaired may be candidates for surgery. Surgery is considered a last resort where conservative measurements have failed. Surgery typically involves the implantation of metal hardware in the occiput, axis and atlas (i.e., C0 to C2) along with rib graft or cadaver bone craft, although additional vertebrae can also be fused.

A five-year follow up study of twenty Ehlers-Danlos Syndrome patients who underwent craniocervical fusion surgery found no deaths or major post-surgical complications.<ref name=":0" /> 25% of patients later required fusion at another level.<ref name=":0" /> A study of 279 patients undergoing craniocervical fusion for CCI with os odontoideum due to trauma and congenital abnormalities found a 2.4% complication rate.<ref>{{Cite journal|last=Wang|first=Chao|last2=Passias|first2=Peter G.|last3=Wang|first3=Shenglin|last4=Zhao|first4=Deng|date=2015-05-01|title=Craniocervical Instability in the Setting of Os OdontoideumAssessment of Cause, Presentation, and Surgical Outcomes in a Series of 279 Cases|url=https://academic.oup.com/neurosurgery/article/76/5/514/2452018|journal=Neurosurgery|language=en|volume=76|issue=5|pages=514–521|doi=10.1227/NEU.0000000000000668|issn=0148-396X}}</ref>

== Risk factors ==
Patients with [[Ehlers-Danlos syndrome]], Down Syndrome, dwarfism and [[Rheumatoid arthritis]] are at greater risk.

== Co-morbid conditions ==
CCI can co-occur in patients with EDS and other connective tissue disorders with [[intracranial hypertension]]<ref name=":0" /> or [[Intracranial hypotension|hypotension]]<ref name=":0" />, abnormalities of intracranial venous drainage due to sinus stenosis or jugular vein stenosis,<ref name=":0" /> [[Migraine]] headaches, [[Temporomandibular joint disorder|temporomandibular joint]] dysfunction<ref name=":0" /><ref name=":1" />, [[tethered cord syndrome]], and scoliosis.<ref name=":1" />

== See also ==

* [[Brainstem compression]]
* [[Ehlers-Danlos syndrome]]
* [[Chiari malformation]]

== Learn more ==

== References ==
<references />
[[Category:Diagnoses]]

Craniocervical instability

2019-06-02T04:10:36Z

Jeff:/* Conservative treatment */

'''Craniocervical instability''' (CCI), also known as the Syndrome of Occipitoatlantialaxial Hypermobility, is a structural instability of the craniocervical junction which may lead to deformation or [[Brainstem compression|compression of the brainstem]], upper [[spinal cord]], and [[cerebellum]]. CCI as well as atlanto-axial instability (AAI), instability between the first and second vertebrae, can frequently co-occur. CCI/AAI can be caused by a traumatic injury, such as whiplash or a high-impact trauma. However, there are several known conditions where CCI/AAI can happen, seemingly spontaneously, in absence of obvious trauma: Down Syndrome, dwarfism, [[rheumatoid arthritis]] (RA), [[Ehlers-Danlos syndrome|Ehlers-Danlos Syndrome]] (EDS) (itself a common co-morbidity with [[myalgic encephalomyelitis|Myalgic Encephalomyelitis]] (ME)), and other [[connective tissue]] disorders. The ligaments of the craniocervical junction, which are made up of connective tissue, become too lax to hold the skull in normal relationship with the cervical spine.

There have been anecdotal reports of people with ME who meet the [[International Consensus Criteria]] (ICC) who were ultimately diagnosed with CCI/AAI and have had their symptoms improve or remit follow craniocervical fusion surgery.<ref>{{Cite web|url=https://forums.phoenixrising.me/index.php?threads/have-you-ruled-out-chiari-as-a-cause-of-your-cfs.56908/|title=Have you ruled out Chiari as a cause of your CFS|last=|first=|date=|website=Phoenix Rising|archive-url=|archive-date=|dead-url=|access-date=}}</ref><ref>{{Cite web|url=https://www.healthrising.org/blog/2019/05/21/jennifer-brea-chronic-fatigue-mecfs-recovering-story/|title=Jennifer Brea's Amazing ME/CFS Recovering Story: the Spinal Series - Pt. II|last=Johnson|first=Cort|date=2019-05-21|website=Health Rising|language=en-GB|access-date=2019-06-01}}</ref><ref>{{Cite web|url=https://rsci.app.link/3LpJOxcTaX?_p=f3542c5bfc29c2616780177b27|website=rsci.app.link|access-date=2019-06-01}}</ref><ref>{{Cite web|url=https://www.mechanicalbasis.org/|title=Craniocervical instability, Atlantoaxial Instability, Myalgic Encephalomyelitis, ME, CFS|website=MEchanical Basis|language=en|access-date=2019-06-01}}</ref> However, thus far, any potential connection between CCI and ME remains essentially unexplored: there has been no formal research investigating the relationship between CCI and ME.

== Symptoms ==
There can be a wide range of clinical presentations. Symptoms<ref>{{Cite web|url=http://thezebranetwork.org/craniocervical-instability/|title=Craniocervical Instability|last=|first=|date=|website=The Zebra Network|archive-url=|archive-date=|dead-url=|access-date=}}</ref> of craniocervical instability and atlantoaxial instability, particularly in the context of a connective tissue disorder, can and ''may'' include:
* [[headache]]<ref name=":2">{{Cite journal|last=Henderson|first=Fraser C.|last2=Austin|first2=Claudiu|last3=Benzel|first3=Edward|last4=Bolognese|first4=Paolo|last5=Ellenbogen|first5=Richard|last6=Francomano|first6=Clair A.|last7=Ireton|first7=Candace|last8=Klinge|first8=Petra|last9=Koby|first9=Myles|date=2017|title=Neurological and spinal manifestations of the Ehlers–Danlos syndromes|url=https://onlinelibrary.wiley.com/doi/abs/10.1002/ajmg.c.31549|journal=American Journal of Medical Genetics Part C: Seminars in Medical Genetics|language=en|volume=175|issue=1|pages=195–211|doi=10.1002/ajmg.c.31549|issn=1552-4876}}</ref><ref name=":0">{{Cite journal|last=Henderson|first=Fraser C.|last2=Francomano|first2=C. A.|last3=Koby|first3=M.|last4=Tuchman|first4=K.|last5=Adcock|first5=J.|last6=Patel|first6=S.|date=2019-01-09|title=Cervical medullary syndrome secondary to craniocervical instability and ventral brainstem compression in hereditary hypermobility connective tissue disorders: 5-year follow-up after craniocervical reduction, fusion, and stabilization|url=https://doi.org/10.1007/s10143-018-01070-4|journal=Neurosurgical Review|language=en|doi=10.1007/s10143-018-01070-4|issn=1437-2320}}</ref><ref name=":5">{{Cite journal|last=Rebbeck|first=Trudy|last2=Liebert|first2=Ann|date=2014-12-01|title=Clinical management of cranio-vertebral instability after whiplash, when guidelines should be adapted: A case report|url=http://www.sciencedirect.com/science/article/pii/S1356689X14000101|journal=Manual Therapy|volume=19|issue=6|pages=618–621|doi=10.1016/j.math.2014.01.009|issn=1356-689X}}</ref>: common types include an occipital headache or occipital pain (back lower part of the head)<ref name=":4">{{Cite web|url=https://search.proquest.com/openview/34b3b18a8854c04ffa0fc50273d68313/1?pq-origsite=gscholar&cbl=47886|title=MRI video diagnosis and surgical therapy of soft tissue trauma to the craniocervical junction - ProQuest|website=search.proquest.com|language=en|access-date=2019-06-01}}</ref><ref name=":6">{{Cite journal|last=Mathers|first=K. Sean|last2=Schneider|first2=Michael|last3=Timko|first3=Michael|date=2011-06|title=Occult Hypermobility of the Craniocervical Junction: A Case Report and Review|url=https://www.jospt.org/doi/full/10.2519/jospt.2011.3305|journal=Journal of Orthopaedic & Sports Physical Therapy|language=en|volume=41|issue=6|pages=444–457|doi=10.2519/jospt.2011.3305|issn=0190-6011}}</ref>; a pressure headache: an impairment of [[cerebropinsal fluid]] (CSF) flow causes intracranial pressure which would be aggravated by “valsalva maneuvers” such as yawning, laughing, crying, coughing, sneezing or straining; headache behind the eyes<ref name=":6" />; and a feeling that head is too heavy for the neck to support (feeling like a “bobble-head”).
* [[Dysautonomia]]<ref name=":0" /><ref name=":3">{{Cite journal|last=Henderson|first=Fraser C.|last2=Austin|first2=Claudiu|last3=Benzel|first3=Edward|last4=Bolognese|first4=Paolo|last5=Ellenbogen|first5=Richard|last6=Francomano|first6=Clair A.|last7=Ireton|first7=Candace|last8=Klinge|first8=Petra|last9=Koby|first9=Myles|date=2017|title=Neurological and spinal manifestations of the Ehlers–Danlos syndromes|url=https://onlinelibrary.wiley.com/doi/abs/10.1002/ajmg.c.31549|journal=American Journal of Medical Genetics Part C: Seminars in Medical Genetics|language=en|volume=175|issue=1|pages=195–211|doi=10.1002/ajmg.c.31549|issn=1552-4876}}</ref> (e.g., [[heat intolerance]], [[orthostatic intolerance]]<ref name=":1">{{Cite journal|last=Francomano|first=Clair A.|last2=McDonnell|first2=Nazli B.|last3=Nishikawa|first3=Misao|last4=Bolognese|first4=Paolo A.|last5=Milhorat|first5=Thomas H.|date=2007-12-01|title=Syndrome of occipitoatlantoaxial hypermobility, cranial settling, and Chiari malformation Type I in patients with hereditary disorders of connective tissue|url=https://thejns.org/view/journals/j-neurosurg-spine/7/6/article-p601.xml|journal=Journal of Neurosurgery: Spine|language=en-US|volume=7|issue=6|pages=601–609|doi=10.3171/SPI-07/12/601}}</ref>, [[polydipsia]] (extreme thirst), [[delayed gastric emptying]])

* [[Postural orthostatic tachycardia syndrome|postural orthostatic tachycardia]]<ref name=":0" /><ref name=":1" />
* dizziness<ref name=":4" /><ref name=":5" /><ref name=":6" />
* fatigue<ref name=":0" /><ref>{{Cite journal|last=Henderson|first=Fraser C.|last2=Henderson|first2=Fraser C.|last3=Wilson|first3=William A.|last4=Mark|first4=Alexander S.|last5=Koby|first5=Myles|date=2018-01-01|title=Utility of the clivo-axial angle in assessing brainstem deformity: pilot study and literature review|url=https://doi.org/10.1007/s10143-017-0830-3|journal=Neurosurgical Review|language=en|volume=41|issue=1|pages=149–163|doi=10.1007/s10143-017-0830-3|issn=1437-2320|pmc=PMC5748419|pmid=28258417}}</ref><ref>{{Cite journal|last=Henderson|first=Fraser C.|last2=Wilson|first2=William A.|last3=Mott|first3=Stephen|last4=Mark|first4=Alexander|last5=Schmidt|first5=Kristi|last6=Berry|first6=Joel K.|last7=Vaccaro|first7=Alexander|last8=Benzel|first8=Edward|date=2010-07-16|title=Deformative stress associated with an abnormal clivo-axial angle: A finite element analysis|url=https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2940090/|journal=Surgical Neurology International|volume=1|doi=10.4103/2152-7806.66461|issn=2152-7806|pmc=PMCPMC2940090|pmid=20847911}}</ref>
* cognitive impairment<ref name=":4" />
* [[muscle weakness]]<ref name=":0" />
* GERD<ref name=":0" />

* posterior [[neck pain]]<ref name=":0" /><ref name=":7">{{Cite journal|last=Bergholm|first=Ulla|last2=Johansson|first2=Bengt H.|last3=Johansson|first3=Hakan|date=2004-01-01|title=New Diagnostic Tools Can Contribute to Better Treatment of Patients with Chronic Whiplash Disorders|url=https://doi.org/10.3109/J180v03n02_02|journal=Journal of Whiplash & Related Disorders|volume=3|issue=2|pages=5–19|doi=10.3109/J180v03n02_02|issn=1533-2888}}</ref><ref name=":5" /><ref name=":6" />
* posterior scalp irritation<ref name=":7" />
* neck stiffness, torticollis<ref name=":7" /><ref>{{Cite journal|last=Ghanem|first=Ismat|last2=El Hage|first2=Samer|last3=Rachkidi|first3=Rami|last4=Kharrat|first4=Khalil|last5=Dagher|first5=Fernand|last6=Kreichati|first6=Gabi|date=2008-03-01|title=Pediatric cervical spine instability|url=https://online.boneandjoint.org.uk/doi/full/10.1007/s11832-008-0092-2|journal=Journal of Children's Orthopaedics|volume=2|issue=2|pages=71–84|doi=10.1007/s11832-008-0092-2|issn=1863-2521|pmc=PMC2656787|pmid=19308585}}</ref>
* tinnitus<ref>{{Cite journal|last=Montazem|first=Abbas|author-link=|author-link2=|author-link3=|author-link4=|author-link5=|date=2000|title=Secondary tinnitus as a symptom of instability in the upper cervical spine: Operative management|url=https://pdfs.semanticscholar.org/21c4/85984a6ebe07efed38cf82a2f7a49b2a644e.pdf|journal=International Tinnitus Journal|volume=|issue=|pages=|quote=|via=}}</ref><ref name=":4" />
* Irritable bowel symptoms<ref name=":0" />
* central or mixed [[apnea]]<ref>{{Cite journal|last=Janjua|first=M. Burhan|last2=Hwang|first2=Steven W.|last3=Samdani|first3=Amer F.|last4=Pahys|first4=Joshua M.|last5=Baaj|first5=Ali A.|last6=Härtl|first6=Roger|last7=Greenfield|first7=Jeffrey P.|date=2019-01-01|title=Instrumented arthrodesis for non-traumatic craniocervical instability in very young children|url=https://doi.org/10.1007/s00381-018-3876-9|journal=Child's Nervous System|language=en|volume=35|issue=1|pages=97–106|doi=10.1007/s00381-018-3876-9|issn=1433-0350}}</ref><ref name=":1" /><ref name=":3" />
* dyspnea (shortness of breath)<ref name=":0" /><ref name=":1" />
* dysphagia (difficulty swallowing)<ref name=":1" /><ref name=":2" />
* altered sleep architecture/sleep disturbance<ref name=":0" /><ref name=":4" />
* facial pain or [[numbness]]<ref name=":1" /><ref name=":2" />
* [[dizziness]] and [[vertigo]]<ref name=":0" /><ref name=":2" /><ref name=":7" />
* syncope<ref name=":0" /><ref name=":1" /><ref name=":2" /><ref name=":7" />
* visual disturbances<ref name=":4" />
* reduced gag reflex and [[Dysphagia|difficulty swallowing]]
* impaired [[coordination]]
* downward [[nystagmus]] (irregular eye movements)<ref name=":1" /><ref name=":7" />
* [[paralysis]]
* parasthesias<ref name=":0" /><ref name=":4" /><ref name=":5" /><ref name=":6" />
* gait changes<ref name=":0" /><ref name=":7" />
* poor proprioception<ref name=":7" />
* nausea<ref name=":1" /><ref name=":2" />

== Diagnosis ==
{{Video|id=https://youtu.be/MsYDA3SXTkg|service=youtube|dimensions=550|description=Dr. Paolo Bolognese discusses methods of imaging and measurement for diagnosis.|alignment=right|urlargs=}}
Craniocervical instability is diagnosed via radiological imaging of the craniocervical junction.

=== Imaging ===
CCI is typically diagnosed via a cervical MRI, whether supine or upright. If supine, a 3 Tesla MRI is preferred over a 1.5 Tesla. Most neurosurgeons prefer upright MRI with flexion and extension.<ref>{{Cite web|url=https://www.mechanicalbasis.org/diagnosis.html|title=Craniocervical instability, Atlantoaxial Instability, Myalgic Encephalomyelitis, ME, CFS|website=MEchanical Basis|language=en|access-date=2019-06-02}}</ref>

=== Measurements ===
More than twenty radiological measurements have been proposed or used in the diagnosis of CCI. However, three measurements are most commonly used: the Grabb-Oakes line, which measures focal compression; the Clivo-Axial Angle, which measures brainstem deformity by the odontoid process; and the Basion Dens Interval, which measures vertical instability (cranial settling).

=== Traction ===
Manual traction and invasive cervical traction are often used to aid in the diagnosis of CCI. Symptomatic improvement with traction can help determine whether a patient with positive radiological findings will benefit from craniocervical fusion surgery.

== Treatment ==
{{Video|id=https://youtu.be/sEi9AlHQTJc|service=youtube|dimensions=550|description=Dr. Fraser Henderson presents the results a five-year follow-up study.|alignment=right|urlargs=}}
=== Conservative treatment ===
Treatment can include “conservative measures” such as rest, bracing with a cervical collar, or physical therapy to strengthen neck muscles. Experimental treatments include stem cell therapy, prolotherapy and upper cervical Chiropractic. Many conservative therapies have little to no supporting evidence of efficacy.

=== Surgery ===
Patients with objective radiological findings, a clinical picture supportive of the diagnosis, positive response to traction, and who are significantly impaired may be candidates for surgery. Surgery is considered a last resort where conservative measurements have failed. Surgery typically involves the implantation of metal hardware in the occiput, axis and atlas (i.e., C0 to C2) along with rib graft or cadaver bone craft, although additional vertebrae can also be fused.

A five-year follow up study of twenty Ehlers-Danlos Syndrome patients who underwent craniocervical fusion surgery found no deaths or major post-surgical complications.<ref name=":0" /> 25% of patients later required fusion at another level.<ref name=":0" /> A study of 279 patients undergoing craniocervical fusion for CCI with os odontoideum due to trauma and congenital abnormalities found a 2.4% complication rate.<ref>{{Cite journal|last=Wang|first=Chao|last2=Passias|first2=Peter G.|last3=Wang|first3=Shenglin|last4=Zhao|first4=Deng|date=2015-05-01|title=Craniocervical Instability in the Setting of Os OdontoideumAssessment of Cause, Presentation, and Surgical Outcomes in a Series of 279 Cases|url=https://academic.oup.com/neurosurgery/article/76/5/514/2452018|journal=Neurosurgery|language=en|volume=76|issue=5|pages=514–521|doi=10.1227/NEU.0000000000000668|issn=0148-396X}}</ref>

== Risk factors ==
Patients with [[Ehlers-Danlos syndrome]], Down Syndrome, dwarfism and [[Rheumatoid arthritis]] are at greater risk.

== Co-morbid conditions ==
CCI can co-occur in patients with EDS and other connective tissue disorders with [[intracranial hypertension]]<ref name=":0" /> or [[Intracranial hypotension|hypotension]]<ref name=":0" />, abnormalities of intracranial venous drainage due to sinus stenosis or jugular vein stenosis,<ref name=":0" /> [[Migraine]] headaches, [[Temporomandibular joint disorder|temporomandibular joint]] dysfunction<ref name=":0" /><ref name=":1" />, [[tethered cord syndrome]], and scoliosis.<ref name=":1" />

== See also ==

* [[Brainstem compression]]
* [[Ehlers-Danlos syndrome]]
* [[Chiari malformation]]

== Learn more ==

== References ==
<references />
[[Category:Diagnoses]]

Craniocervical instability

2019-06-02T04:06:26Z

Jeff:

'''Craniocervical instability''' (CCI), also known as the Syndrome of Occipitoatlantialaxial Hypermobility, is a structural instability of the craniocervical junction which may lead to deformation or [[Brainstem compression|compression of the brainstem]], upper [[spinal cord]], and [[cerebellum]]. CCI as well as atlanto-axial instability (AAI), instability between the first and second vertebrae, can frequently co-occur. CCI/AAI can be caused by a traumatic injury, such as whiplash or a high-impact trauma. However, there are several known conditions where CCI/AAI can happen, seemingly spontaneously, in absence of obvious trauma: Down Syndrome, dwarfism, [[rheumatoid arthritis]] (RA), [[Ehlers-Danlos syndrome|Ehlers-Danlos Syndrome]] (EDS) (itself a common co-morbidity with [[myalgic encephalomyelitis|Myalgic Encephalomyelitis]] (ME)), and other [[connective tissue]] disorders. The ligaments of the craniocervical junction, which are made up of connective tissue, become too lax to hold the skull in normal relationship with the cervical spine.

There have been anecdotal reports of people with ME who meet the [[International Consensus Criteria]] (ICC) who were ultimately diagnosed with CCI/AAI and have had their symptoms improve or remit follow craniocervical fusion surgery.<ref>{{Cite web|url=https://forums.phoenixrising.me/index.php?threads/have-you-ruled-out-chiari-as-a-cause-of-your-cfs.56908/|title=Have you ruled out Chiari as a cause of your CFS|last=|first=|date=|website=Phoenix Rising|archive-url=|archive-date=|dead-url=|access-date=}}</ref><ref>{{Cite web|url=https://www.healthrising.org/blog/2019/05/21/jennifer-brea-chronic-fatigue-mecfs-recovering-story/|title=Jennifer Brea's Amazing ME/CFS Recovering Story: the Spinal Series - Pt. II|last=Johnson|first=Cort|date=2019-05-21|website=Health Rising|language=en-GB|access-date=2019-06-01}}</ref><ref>{{Cite web|url=https://rsci.app.link/3LpJOxcTaX?_p=f3542c5bfc29c2616780177b27|website=rsci.app.link|access-date=2019-06-01}}</ref><ref>{{Cite web|url=https://www.mechanicalbasis.org/|title=Craniocervical instability, Atlantoaxial Instability, Myalgic Encephalomyelitis, ME, CFS|website=MEchanical Basis|language=en|access-date=2019-06-01}}</ref> However, thus far, any potential connection between CCI and ME remains essentially unexplored: there has been no formal research investigating the relationship between CCI and ME.

== Symptoms ==
There can be a wide range of clinical presentations. Symptoms<ref>{{Cite web|url=http://thezebranetwork.org/craniocervical-instability/|title=Craniocervical Instability|last=|first=|date=|website=The Zebra Network|archive-url=|archive-date=|dead-url=|access-date=}}</ref> of craniocervical instability and atlantoaxial instability, particularly in the context of a connective tissue disorder, can and ''may'' include:
* [[headache]]<ref name=":2">{{Cite journal|last=Henderson|first=Fraser C.|last2=Austin|first2=Claudiu|last3=Benzel|first3=Edward|last4=Bolognese|first4=Paolo|last5=Ellenbogen|first5=Richard|last6=Francomano|first6=Clair A.|last7=Ireton|first7=Candace|last8=Klinge|first8=Petra|last9=Koby|first9=Myles|date=2017|title=Neurological and spinal manifestations of the Ehlers–Danlos syndromes|url=https://onlinelibrary.wiley.com/doi/abs/10.1002/ajmg.c.31549|journal=American Journal of Medical Genetics Part C: Seminars in Medical Genetics|language=en|volume=175|issue=1|pages=195–211|doi=10.1002/ajmg.c.31549|issn=1552-4876}}</ref><ref name=":0">{{Cite journal|last=Henderson|first=Fraser C.|last2=Francomano|first2=C. A.|last3=Koby|first3=M.|last4=Tuchman|first4=K.|last5=Adcock|first5=J.|last6=Patel|first6=S.|date=2019-01-09|title=Cervical medullary syndrome secondary to craniocervical instability and ventral brainstem compression in hereditary hypermobility connective tissue disorders: 5-year follow-up after craniocervical reduction, fusion, and stabilization|url=https://doi.org/10.1007/s10143-018-01070-4|journal=Neurosurgical Review|language=en|doi=10.1007/s10143-018-01070-4|issn=1437-2320}}</ref><ref name=":5">{{Cite journal|last=Rebbeck|first=Trudy|last2=Liebert|first2=Ann|date=2014-12-01|title=Clinical management of cranio-vertebral instability after whiplash, when guidelines should be adapted: A case report|url=http://www.sciencedirect.com/science/article/pii/S1356689X14000101|journal=Manual Therapy|volume=19|issue=6|pages=618–621|doi=10.1016/j.math.2014.01.009|issn=1356-689X}}</ref>: common types include an occipital headache or occipital pain (back lower part of the head)<ref name=":4">{{Cite web|url=https://search.proquest.com/openview/34b3b18a8854c04ffa0fc50273d68313/1?pq-origsite=gscholar&cbl=47886|title=MRI video diagnosis and surgical therapy of soft tissue trauma to the craniocervical junction - ProQuest|website=search.proquest.com|language=en|access-date=2019-06-01}}</ref><ref name=":6">{{Cite journal|last=Mathers|first=K. Sean|last2=Schneider|first2=Michael|last3=Timko|first3=Michael|date=2011-06|title=Occult Hypermobility of the Craniocervical Junction: A Case Report and Review|url=https://www.jospt.org/doi/full/10.2519/jospt.2011.3305|journal=Journal of Orthopaedic & Sports Physical Therapy|language=en|volume=41|issue=6|pages=444–457|doi=10.2519/jospt.2011.3305|issn=0190-6011}}</ref>; a pressure headache: an impairment of [[cerebropinsal fluid]] (CSF) flow causes intracranial pressure which would be aggravated by “valsalva maneuvers” such as yawning, laughing, crying, coughing, sneezing or straining; headache behind the eyes<ref name=":6" />; and a feeling that head is too heavy for the neck to support (feeling like a “bobble-head”).
* [[Dysautonomia]]<ref name=":0" /><ref name=":3">{{Cite journal|last=Henderson|first=Fraser C.|last2=Austin|first2=Claudiu|last3=Benzel|first3=Edward|last4=Bolognese|first4=Paolo|last5=Ellenbogen|first5=Richard|last6=Francomano|first6=Clair A.|last7=Ireton|first7=Candace|last8=Klinge|first8=Petra|last9=Koby|first9=Myles|date=2017|title=Neurological and spinal manifestations of the Ehlers–Danlos syndromes|url=https://onlinelibrary.wiley.com/doi/abs/10.1002/ajmg.c.31549|journal=American Journal of Medical Genetics Part C: Seminars in Medical Genetics|language=en|volume=175|issue=1|pages=195–211|doi=10.1002/ajmg.c.31549|issn=1552-4876}}</ref> (e.g., [[heat intolerance]], [[orthostatic intolerance]]<ref name=":1">{{Cite journal|last=Francomano|first=Clair A.|last2=McDonnell|first2=Nazli B.|last3=Nishikawa|first3=Misao|last4=Bolognese|first4=Paolo A.|last5=Milhorat|first5=Thomas H.|date=2007-12-01|title=Syndrome of occipitoatlantoaxial hypermobility, cranial settling, and Chiari malformation Type I in patients with hereditary disorders of connective tissue|url=https://thejns.org/view/journals/j-neurosurg-spine/7/6/article-p601.xml|journal=Journal of Neurosurgery: Spine|language=en-US|volume=7|issue=6|pages=601–609|doi=10.3171/SPI-07/12/601}}</ref>, [[polydipsia]] (extreme thirst), [[delayed gastric emptying]])

* [[Postural orthostatic tachycardia syndrome|postural orthostatic tachycardia]]<ref name=":0" /><ref name=":1" />
* dizziness<ref name=":4" /><ref name=":5" /><ref name=":6" />
* fatigue<ref name=":0" /><ref>{{Cite journal|last=Henderson|first=Fraser C.|last2=Henderson|first2=Fraser C.|last3=Wilson|first3=William A.|last4=Mark|first4=Alexander S.|last5=Koby|first5=Myles|date=2018-01-01|title=Utility of the clivo-axial angle in assessing brainstem deformity: pilot study and literature review|url=https://doi.org/10.1007/s10143-017-0830-3|journal=Neurosurgical Review|language=en|volume=41|issue=1|pages=149–163|doi=10.1007/s10143-017-0830-3|issn=1437-2320|pmc=PMC5748419|pmid=28258417}}</ref><ref>{{Cite journal|last=Henderson|first=Fraser C.|last2=Wilson|first2=William A.|last3=Mott|first3=Stephen|last4=Mark|first4=Alexander|last5=Schmidt|first5=Kristi|last6=Berry|first6=Joel K.|last7=Vaccaro|first7=Alexander|last8=Benzel|first8=Edward|date=2010-07-16|title=Deformative stress associated with an abnormal clivo-axial angle: A finite element analysis|url=https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2940090/|journal=Surgical Neurology International|volume=1|doi=10.4103/2152-7806.66461|issn=2152-7806|pmc=PMCPMC2940090|pmid=20847911}}</ref>
* cognitive impairment<ref name=":4" />
* [[muscle weakness]]<ref name=":0" />
* GERD<ref name=":0" />

* posterior [[neck pain]]<ref name=":0" /><ref name=":7">{{Cite journal|last=Bergholm|first=Ulla|last2=Johansson|first2=Bengt H.|last3=Johansson|first3=Hakan|date=2004-01-01|title=New Diagnostic Tools Can Contribute to Better Treatment of Patients with Chronic Whiplash Disorders|url=https://doi.org/10.3109/J180v03n02_02|journal=Journal of Whiplash & Related Disorders|volume=3|issue=2|pages=5–19|doi=10.3109/J180v03n02_02|issn=1533-2888}}</ref><ref name=":5" /><ref name=":6" />
* posterior scalp irritation<ref name=":7" />
* neck stiffness, torticollis<ref name=":7" /><ref>{{Cite journal|last=Ghanem|first=Ismat|last2=El Hage|first2=Samer|last3=Rachkidi|first3=Rami|last4=Kharrat|first4=Khalil|last5=Dagher|first5=Fernand|last6=Kreichati|first6=Gabi|date=2008-03-01|title=Pediatric cervical spine instability|url=https://online.boneandjoint.org.uk/doi/full/10.1007/s11832-008-0092-2|journal=Journal of Children's Orthopaedics|volume=2|issue=2|pages=71–84|doi=10.1007/s11832-008-0092-2|issn=1863-2521|pmc=PMC2656787|pmid=19308585}}</ref>
* tinnitus<ref>{{Cite journal|last=Montazem|first=Abbas|author-link=|author-link2=|author-link3=|author-link4=|author-link5=|date=2000|title=Secondary tinnitus as a symptom of instability in the upper cervical spine: Operative management|url=https://pdfs.semanticscholar.org/21c4/85984a6ebe07efed38cf82a2f7a49b2a644e.pdf|journal=International Tinnitus Journal|volume=|issue=|pages=|quote=|via=}}</ref><ref name=":4" />
* Irritable bowel symptoms<ref name=":0" />
* central or mixed [[apnea]]<ref>{{Cite journal|last=Janjua|first=M. Burhan|last2=Hwang|first2=Steven W.|last3=Samdani|first3=Amer F.|last4=Pahys|first4=Joshua M.|last5=Baaj|first5=Ali A.|last6=Härtl|first6=Roger|last7=Greenfield|first7=Jeffrey P.|date=2019-01-01|title=Instrumented arthrodesis for non-traumatic craniocervical instability in very young children|url=https://doi.org/10.1007/s00381-018-3876-9|journal=Child's Nervous System|language=en|volume=35|issue=1|pages=97–106|doi=10.1007/s00381-018-3876-9|issn=1433-0350}}</ref><ref name=":1" /><ref name=":3" />
* dyspnea (shortness of breath)<ref name=":0" /><ref name=":1" />
* dysphagia (difficulty swallowing)<ref name=":1" /><ref name=":2" />
* altered sleep architecture/sleep disturbance<ref name=":0" /><ref name=":4" />
* facial pain or [[numbness]]<ref name=":1" /><ref name=":2" />
* [[dizziness]] and [[vertigo]]<ref name=":0" /><ref name=":2" /><ref name=":7" />
* syncope<ref name=":0" /><ref name=":1" /><ref name=":2" /><ref name=":7" />
* visual disturbances<ref name=":4" />
* reduced gag reflex and [[Dysphagia|difficulty swallowing]]
* impaired [[coordination]]
* downward [[nystagmus]] (irregular eye movements)<ref name=":1" /><ref name=":7" />
* [[paralysis]]
* parasthesias<ref name=":0" /><ref name=":4" /><ref name=":5" /><ref name=":6" />
* gait changes<ref name=":0" /><ref name=":7" />
* poor proprioception<ref name=":7" />
* nausea<ref name=":1" /><ref name=":2" />

== Diagnosis ==
{{Video|id=https://youtu.be/MsYDA3SXTkg|service=youtube|dimensions=550|description=Dr. Paolo Bolognese discusses methods of imaging and measurement for diagnosis.|alignment=right|urlargs=}}
Craniocervical instability is diagnosed via radiological imaging of the craniocervical junction.

=== Imaging ===
CCI is typically diagnosed via a cervical MRI, whether supine or upright. If supine, a 3 Tesla MRI is preferred over a 1.5 Tesla. Most neurosurgeons prefer upright MRI with flexion and extension.<ref>{{Cite web|url=https://www.mechanicalbasis.org/diagnosis.html|title=Craniocervical instability, Atlantoaxial Instability, Myalgic Encephalomyelitis, ME, CFS|website=MEchanical Basis|language=en|access-date=2019-06-02}}</ref>

=== Measurements ===
More than twenty radiological measurements have been proposed or used in the diagnosis of CCI. However, three measurements are most commonly used: the Grabb-Oakes line, which measures focal compression; the Clivo-Axial Angle, which measures brainstem deformity by the odontoid process; and the Basion Dens Interval, which measures vertical instability (cranial settling).

=== Traction ===
Manual traction and invasive cervical traction are often used to aid in the diagnosis of CCI. Symptomatic improvement with traction can help determine whether a patient with positive radiological findings will benefit from craniocervical fusion surgery.

== Treatment ==
{{Video|id=https://youtu.be/sEi9AlHQTJc|service=youtube|dimensions=550|description=Dr. Fraser Henderson presents the results a five-year follow-up study.|alignment=right|urlargs=}}
=== Conservative treatment ===
Treatment can include “conservative measures” such as rest, bracing with a cervical collar, or physical therapy to strengthen neck muscles. Experimental treatments include stem cell therapy, prolotherapy and upper cervical Chiropractic. Many conservative therapies have little to no evidence.

=== Surgery ===
Patients with objective radiological findings, a clinical picture supportive of the diagnosis, positive response to traction, and who are significantly impaired may be candidates for surgery. Surgery is considered a last resort where conservative measurements have failed. Surgery typically involves the implantation of metal hardware in the occiput, axis and atlas (i.e., C0 to C2) along with rib graft or cadaver bone craft, although additional vertebrae can also be fused.

A five-year follow up study of twenty Ehlers-Danlos Syndrome patients who underwent craniocervical fusion surgery found no deaths or major post-surgical complications.<ref name=":0" /> 25% of patients later required fusion at another level.<ref name=":0" /> A study of 279 patients undergoing craniocervical fusion for CCI with os odontoideum due to trauma and congenital abnormalities found a 2.4% complication rate.<ref>{{Cite journal|last=Wang|first=Chao|last2=Passias|first2=Peter G.|last3=Wang|first3=Shenglin|last4=Zhao|first4=Deng|date=2015-05-01|title=Craniocervical Instability in the Setting of Os OdontoideumAssessment of Cause, Presentation, and Surgical Outcomes in a Series of 279 Cases|url=https://academic.oup.com/neurosurgery/article/76/5/514/2452018|journal=Neurosurgery|language=en|volume=76|issue=5|pages=514–521|doi=10.1227/NEU.0000000000000668|issn=0148-396X}}</ref>

== Risk factors ==
Patients with [[Ehlers-Danlos syndrome]], Down Syndrome, dwarfism and [[Rheumatoid arthritis]] are at greater risk.

== Co-morbid conditions ==
CCI can co-occur in patients with EDS and other connective tissue disorders with [[intracranial hypertension]]<ref name=":0" /> or [[Intracranial hypotension|hypotension]]<ref name=":0" />, abnormalities of intracranial venous drainage due to sinus stenosis or jugular vein stenosis,<ref name=":0" /> [[Migraine]] headaches, [[Temporomandibular joint disorder|temporomandibular joint]] dysfunction<ref name=":0" /><ref name=":1" />, [[tethered cord syndrome]], and scoliosis.<ref name=":1" />

== See also ==

* [[Brainstem compression]]
* [[Ehlers-Danlos syndrome]]
* [[Chiari malformation]]

== Learn more ==

== References ==
<references />
[[Category:Diagnoses]]

Craniocervical instability

2019-06-02T00:08:53Z

Jeff:

'''Craniocervical instability''' (CCI), also known as the Syndrome of Occipitoatlantialaxial Hypermobility, is a structural instability of the craniocervical junction which may lead to deformation or [[Brainstem compression|compression of the brainstem]], upper [[spinal cord]], and [[cerebellum]]. CCI as well as atlanto-axial instability (AAI), instability between the first and second vertebrae, can frequently co-occur. CCI/AAI can be caused by a traumatic injury, such as whiplash or a high-impact trauma. However, there are several known conditions where CCI/AAI can happen, seemingly spontaneously, in absence of obvious trauma: Down Syndrome, dwarfism, [[rheumatoid arthritis]] (RA), [[Ehlers-Danlos syndrome|Ehlers-Danlos Syndrome]] (EDS) (itself a common co-morbidity with [[myalgic encephalomyelitis|Myalgic Encephalomyelitis]] (ME)), and other [[connective tissue]] disorders. The ligaments of the craniocervical junction, which are made up of connective tissue, become too lax to hold the skull in normal relationship with the cervical spine.

There have been anecdotal reports of people with ME who meet the [[International Consensus Criteria]] (ICC) who were ultimately diagnosed with CCI/AAI and have had their symptoms improve or remit follow craniocervical fusion surgery.<ref>{{Cite web|url=https://forums.phoenixrising.me/index.php?threads/have-you-ruled-out-chiari-as-a-cause-of-your-cfs.56908/|title=Have you ruled out Chiari as a cause of your CFS|last=|first=|date=|website=Phoenix Rising|archive-url=|archive-date=|dead-url=|access-date=}}</ref><ref>{{Cite web|url=https://www.healthrising.org/blog/2019/05/21/jennifer-brea-chronic-fatigue-mecfs-recovering-story/|title=Jennifer Brea's Amazing ME/CFS Recovering Story: the Spinal Series - Pt. II|last=Johnson|first=Cort|date=2019-05-21|website=Health Rising|language=en-GB|access-date=2019-06-01}}</ref><ref>{{Cite web|url=https://rsci.app.link/3LpJOxcTaX?_p=f3542c5bfc29c2616780177b27|website=rsci.app.link|access-date=2019-06-01}}</ref><ref>{{Cite web|url=https://www.mechanicalbasis.org/|title=Craniocervical instability, Atlantoaxial Instability, Myalgic Encephalomyelitis, ME, CFS|website=MEchanical Basis|language=en|access-date=2019-06-01}}</ref> However, thus far, any potential connection between CCI and ME remains essentially unexplored: there has been no formal research investigating the relationship between CCI and ME.

== Symptoms ==
There can be a wide range of clinical presentations. Symptoms<ref>{{Cite web|url=http://thezebranetwork.org/craniocervical-instability/|title=Craniocervical Instability|last=|first=|date=|website=The Zebra Network|archive-url=|archive-date=|dead-url=|access-date=}}</ref> of craniocervical instability and atlantoaxial instability, particularly in the context of a connective tissue disorder, can and ''may'' include:
* [[headache]]<ref name=":2">{{Cite journal|last=Henderson|first=Fraser C.|last2=Austin|first2=Claudiu|last3=Benzel|first3=Edward|last4=Bolognese|first4=Paolo|last5=Ellenbogen|first5=Richard|last6=Francomano|first6=Clair A.|last7=Ireton|first7=Candace|last8=Klinge|first8=Petra|last9=Koby|first9=Myles|date=2017|title=Neurological and spinal manifestations of the Ehlers–Danlos syndromes|url=https://onlinelibrary.wiley.com/doi/abs/10.1002/ajmg.c.31549|journal=American Journal of Medical Genetics Part C: Seminars in Medical Genetics|language=en|volume=175|issue=1|pages=195–211|doi=10.1002/ajmg.c.31549|issn=1552-4876}}</ref><ref name=":0">{{Cite journal|last=Henderson|first=Fraser C.|last2=Francomano|first2=C. A.|last3=Koby|first3=M.|last4=Tuchman|first4=K.|last5=Adcock|first5=J.|last6=Patel|first6=S.|date=2019-01-09|title=Cervical medullary syndrome secondary to craniocervical instability and ventral brainstem compression in hereditary hypermobility connective tissue disorders: 5-year follow-up after craniocervical reduction, fusion, and stabilization|url=https://doi.org/10.1007/s10143-018-01070-4|journal=Neurosurgical Review|language=en|doi=10.1007/s10143-018-01070-4|issn=1437-2320}}</ref><ref name=":5">{{Cite journal|last=Rebbeck|first=Trudy|last2=Liebert|first2=Ann|date=2014-12-01|title=Clinical management of cranio-vertebral instability after whiplash, when guidelines should be adapted: A case report|url=http://www.sciencedirect.com/science/article/pii/S1356689X14000101|journal=Manual Therapy|volume=19|issue=6|pages=618–621|doi=10.1016/j.math.2014.01.009|issn=1356-689X}}</ref>: common types include an occipital headache or occipital pain (back lower part of the head)<ref name=":4">{{Cite web|url=https://search.proquest.com/openview/34b3b18a8854c04ffa0fc50273d68313/1?pq-origsite=gscholar&cbl=47886|title=MRI video diagnosis and surgical therapy of soft tissue trauma to the craniocervical junction - ProQuest|website=search.proquest.com|language=en|access-date=2019-06-01}}</ref><ref name=":6">{{Cite journal|last=Mathers|first=K. Sean|last2=Schneider|first2=Michael|last3=Timko|first3=Michael|date=2011-06|title=Occult Hypermobility of the Craniocervical Junction: A Case Report and Review|url=https://www.jospt.org/doi/full/10.2519/jospt.2011.3305|journal=Journal of Orthopaedic & Sports Physical Therapy|language=en|volume=41|issue=6|pages=444–457|doi=10.2519/jospt.2011.3305|issn=0190-6011}}</ref>; a pressure headache: an impairment of [[cerebropinsal fluid]] (CSF) flow causes intracranial pressure which would be aggravated by “valsalva maneuvers” such as yawning, laughing, crying, coughing, sneezing or straining; headache behind the eyes<ref name=":6" />; and a feeling that head is too heavy for the neck to support (feeling like a “bobble-head”).
* [[Dysautonomia]]<ref name=":0" /><ref name=":3">{{Cite journal|last=Henderson|first=Fraser C.|last2=Austin|first2=Claudiu|last3=Benzel|first3=Edward|last4=Bolognese|first4=Paolo|last5=Ellenbogen|first5=Richard|last6=Francomano|first6=Clair A.|last7=Ireton|first7=Candace|last8=Klinge|first8=Petra|last9=Koby|first9=Myles|date=2017|title=Neurological and spinal manifestations of the Ehlers–Danlos syndromes|url=https://onlinelibrary.wiley.com/doi/abs/10.1002/ajmg.c.31549|journal=American Journal of Medical Genetics Part C: Seminars in Medical Genetics|language=en|volume=175|issue=1|pages=195–211|doi=10.1002/ajmg.c.31549|issn=1552-4876}}</ref> (e.g., [[heat intolerance]], [[orthostatic intolerance]]<ref name=":1">{{Cite journal|last=Francomano|first=Clair A.|last2=McDonnell|first2=Nazli B.|last3=Nishikawa|first3=Misao|last4=Bolognese|first4=Paolo A.|last5=Milhorat|first5=Thomas H.|date=2007-12-01|title=Syndrome of occipitoatlantoaxial hypermobility, cranial settling, and Chiari malformation Type I in patients with hereditary disorders of connective tissue|url=https://thejns.org/view/journals/j-neurosurg-spine/7/6/article-p601.xml|journal=Journal of Neurosurgery: Spine|language=en-US|volume=7|issue=6|pages=601–609|doi=10.3171/SPI-07/12/601}}</ref>, [[polydipsia]] (extreme thirst), [[delayed gastric emptying]])

* [[Postural orthostatic tachycardia syndrome|postural orthostatic tachycardia]]<ref name=":0" /><ref name=":1" />
* dizziness<ref name=":4" /><ref name=":5" /><ref name=":6" />
* fatigue<ref name=":0" /><ref>{{Cite journal|last=Henderson|first=Fraser C.|last2=Henderson|first2=Fraser C.|last3=Wilson|first3=William A.|last4=Mark|first4=Alexander S.|last5=Koby|first5=Myles|date=2018-01-01|title=Utility of the clivo-axial angle in assessing brainstem deformity: pilot study and literature review|url=https://doi.org/10.1007/s10143-017-0830-3|journal=Neurosurgical Review|language=en|volume=41|issue=1|pages=149–163|doi=10.1007/s10143-017-0830-3|issn=1437-2320|pmc=PMC5748419|pmid=28258417}}</ref><ref>{{Cite journal|last=Henderson|first=Fraser C.|last2=Wilson|first2=William A.|last3=Mott|first3=Stephen|last4=Mark|first4=Alexander|last5=Schmidt|first5=Kristi|last6=Berry|first6=Joel K.|last7=Vaccaro|first7=Alexander|last8=Benzel|first8=Edward|date=2010-07-16|title=Deformative stress associated with an abnormal clivo-axial angle: A finite element analysis|url=https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2940090/|journal=Surgical Neurology International|volume=1|doi=10.4103/2152-7806.66461|issn=2152-7806|pmc=PMCPMC2940090|pmid=20847911}}</ref>
* cognitive impairment<ref name=":4" />
* [[muscle weakness]]<ref name=":0" />
* GERD<ref name=":0" />

* posterior [[neck pain]]<ref name=":0" /><ref name=":7">{{Cite journal|last=Bergholm|first=Ulla|last2=Johansson|first2=Bengt H.|last3=Johansson|first3=Hakan|date=2004-01-01|title=New Diagnostic Tools Can Contribute to Better Treatment of Patients with Chronic Whiplash Disorders|url=https://doi.org/10.3109/J180v03n02_02|journal=Journal of Whiplash & Related Disorders|volume=3|issue=2|pages=5–19|doi=10.3109/J180v03n02_02|issn=1533-2888}}</ref><ref name=":5" /><ref name=":6" />
* posterior scalp irritation<ref name=":7" />
* neck stiffness, torticollis<ref name=":7" /><ref>{{Cite journal|last=Ghanem|first=Ismat|last2=El Hage|first2=Samer|last3=Rachkidi|first3=Rami|last4=Kharrat|first4=Khalil|last5=Dagher|first5=Fernand|last6=Kreichati|first6=Gabi|date=2008-03-01|title=Pediatric cervical spine instability|url=https://online.boneandjoint.org.uk/doi/full/10.1007/s11832-008-0092-2|journal=Journal of Children's Orthopaedics|volume=2|issue=2|pages=71–84|doi=10.1007/s11832-008-0092-2|issn=1863-2521|pmc=PMC2656787|pmid=19308585}}</ref>
* tinnitus<ref>{{Cite journal|last=Montazem|first=Abbas|author-link=|author-link2=|author-link3=|author-link4=|author-link5=|date=2000|title=Secondary tinnitus as a symptom of instability in the upper cervical spine: Operative management|url=https://pdfs.semanticscholar.org/21c4/85984a6ebe07efed38cf82a2f7a49b2a644e.pdf|journal=International Tinnitus Journal|volume=|issue=|pages=|quote=|via=}}</ref><ref name=":4" />
* Irritable bowel symptoms<ref name=":0" />
* central or mixed [[apnea]]<ref>{{Cite journal|last=Janjua|first=M. Burhan|last2=Hwang|first2=Steven W.|last3=Samdani|first3=Amer F.|last4=Pahys|first4=Joshua M.|last5=Baaj|first5=Ali A.|last6=Härtl|first6=Roger|last7=Greenfield|first7=Jeffrey P.|date=2019-01-01|title=Instrumented arthrodesis for non-traumatic craniocervical instability in very young children|url=https://doi.org/10.1007/s00381-018-3876-9|journal=Child's Nervous System|language=en|volume=35|issue=1|pages=97–106|doi=10.1007/s00381-018-3876-9|issn=1433-0350}}</ref><ref name=":1" /><ref name=":3" />
* dyspnea (shortness of breath)<ref name=":0" /><ref name=":1" />
* dysphagia (difficulty swallowing)<ref name=":1" /><ref name=":2" />
* altered sleep architecture/sleep disturbance<ref name=":0" /><ref name=":4" />
* facial pain or [[numbness]]<ref name=":1" /><ref name=":2" />
* [[dizziness]] and [[vertigo]]<ref name=":0" /><ref name=":2" /><ref name=":7" />
* syncope<ref name=":0" /><ref name=":1" /><ref name=":2" /><ref name=":7" />
* visual disturbances<ref name=":4" />
* reduced gag reflex and [[Dysphagia|difficulty swallowing]]
* impaired [[coordination]]
* downward [[nystagmus]] (irregular eye movements)<ref name=":1" /><ref name=":7" />
* [[paralysis]]
* parasthesias<ref name=":0" /><ref name=":4" /><ref name=":5" /><ref name=":6" />
* gait changes<ref name=":0" /><ref name=":7" />
* poor proprioception<ref name=":7" />
* nausea<ref name=":1" /><ref name=":2" />

== Diagnosis ==
Craniocervical instability is diagnosed via radiographic imaging of the craniocervical junction.
== Treatment ==
{{Video|id=https://youtu.be/sEi9AlHQTJc|service=youtube|dimensions=550|description=|alignment=right|urlargs=}}

=== Conservative treatment ===
Treatment can include “conservative measures” such as rest, bracing with a cervical collar, or physical therapy to strengthen neck muscles. Experimental treatments include stem cell therapy, prolotherapy and upper cervical Chiropractic. Many conservative therapies have little to no evidence.

=== Surgery ===
Surgery is considered a last resort for patients with objective radiological findings together with a clinical picture supportive of the diagnosis and major disability. Surgery typically involves the implantation of metal hardware in the occiput, axis and atlas (i.e., C0 to C2) alone with rib graft or cadaver bone craft, although additional vertebrae can also be fused.

A five-year follow up study of twenty Ehlers-Danlos Syndrome patients who underwent craniocervical fusion surgery found no deaths or major post-surgical complications.<ref name=":0" /> 25% of patients later required fusion at another level.<ref name=":0" /> A study of 279 patients undergoing craniocervical fusion for CCI with os odontoideum due to trauma and congenital abnormalities found a 2.4% complication rate.<ref>{{Cite journal|last=Wang|first=Chao|last2=Passias|first2=Peter G.|last3=Wang|first3=Shenglin|last4=Zhao|first4=Deng|date=2015-05-01|title=Craniocervical Instability in the Setting of Os OdontoideumAssessment of Cause, Presentation, and Surgical Outcomes in a Series of 279 Cases|url=https://academic.oup.com/neurosurgery/article/76/5/514/2452018|journal=Neurosurgery|language=en|volume=76|issue=5|pages=514–521|doi=10.1227/NEU.0000000000000668|issn=0148-396X}}</ref>

== Risk factors ==
Patients with [[Ehlers-Danlos syndrome]], Down Syndrome, dwarfism and [[Rheumatoid arthritis]] are at greater risk.

== Co-morbid conditions ==
CCI can co-occur in patients with EDS and other connective tissue disorders with [[intracranial hypertension]]<ref name=":0" /> or [[Intracranial hypotension|hypotension]]<ref name=":0" />, abnormalities of intracranial venous drainage due to sinus stenosis or jugular vein stenosis,<ref name=":0" /> [[Migraine]] headaches, [[Temporomandibular joint disorder|temporomandibular joint]] dysfunction<ref name=":0" /><ref name=":1" />, [[tethered cord syndrome]], and scoliosis.<ref name=":1" />

== See also ==

* [[Brainstem compression]]
* [[Ehlers-Danlos syndrome]]
* [[Chiari malformation]]

== Learn more ==

== References ==
<references />
[[Category:Diagnoses]]

Craniocervical instability

2019-06-02T00:02:57Z

Jeff:Added citation for bulletpoint "muscle weakness," and moved this bulletpoint (now cited!) higher on the symptom list.

'''Craniocervical instability''' (CCI), also known as the Syndrome of Occipitoatlantialaxial Hypermobility, is a structural instability of the craniocervical junction which may lead to deformation or [[Brainstem compression|compression of the brainstem]], upper [[spinal cord]], and [[cerebellum]]. CCI as well as atlanto-axial instability (AAI), instability between the first and second vertebrae, can frequently co-occur. CCI/AAI can be caused by a traumatic injury, such as whiplash or a high-impact trauma. However, there are several known conditions where CCI/AAI can happen, seemingly spontaneously, in absence of obvious trauma: Down Syndrome, dwarfism, [[rheumatoid arthritis]] (RA), [[Ehlers-Danlos syndrome|Ehlers-Danlos Syndrome]] (EDS) (itself a common co-morbidity with [[myalgic encephalomyelitis|Myalgic Encephalomyelitis]] (ME)), and other [[connective tissue]] disorders. The ligaments of the craniocervical junction, which are made up of connective tissue, become too lax to hold the skull in normal relationship with the cervical spine.

There have been anecdotal reports of people with ME who meet the [[International Consensus Criteria]] (ICC) who were ultimately diagnosed with CCI/AAI and/or [[Chiari malformation]], a related condition.<ref>{{Cite web|url=https://forums.phoenixrising.me/index.php?threads/have-you-ruled-out-chiari-as-a-cause-of-your-cfs.56908/|title=Have you ruled out Chiari as a cause of your CFS|last=|first=|date=|website=Phoenix Rising|archive-url=|archive-date=|dead-url=|access-date=}}</ref><ref>{{Cite web|url=https://www.healthrising.org/blog/2019/05/21/jennifer-brea-chronic-fatigue-mecfs-recovering-story/|title=Jennifer Brea's Amazing ME/CFS Recovering Story: the Spinal Series - Pt. II|last=Johnson|first=Cort|date=2019-05-21|website=Health Rising|language=en-GB|access-date=2019-06-01}}</ref><ref>{{Cite web|url=https://rsci.app.link/3LpJOxcTaX?_p=f3542c5bfc29c2616780177b27|website=rsci.app.link|access-date=2019-06-01}}</ref><ref>{{Cite web|url=https://www.mechanicalbasis.org/|title=Craniocervical instability, Atlantoaxial Instability, Myalgic Encephalomyelitis, ME, CFS|website=MEchanical Basis|language=en|access-date=2019-06-01}}</ref> However, thus far, any potential connection between CCI and ME remains essentially unexplored, as there has been no formal research investigating the relationship between CCI and ME.

== Symptoms ==
There can be a wide range of clinical presentations. Symptoms<ref>{{Cite web|url=http://thezebranetwork.org/craniocervical-instability/|title=Craniocervical Instability|last=|first=|date=|website=The Zebra Network|archive-url=|archive-date=|dead-url=|access-date=}}</ref> of craniocervical instability and atlantoaxial instability, particularly in the context of a connective tissue disorder, can and ''may'' include:
* [[headache]]<ref name=":2">{{Cite journal|last=Henderson|first=Fraser C.|last2=Austin|first2=Claudiu|last3=Benzel|first3=Edward|last4=Bolognese|first4=Paolo|last5=Ellenbogen|first5=Richard|last6=Francomano|first6=Clair A.|last7=Ireton|first7=Candace|last8=Klinge|first8=Petra|last9=Koby|first9=Myles|date=2017|title=Neurological and spinal manifestations of the Ehlers–Danlos syndromes|url=https://onlinelibrary.wiley.com/doi/abs/10.1002/ajmg.c.31549|journal=American Journal of Medical Genetics Part C: Seminars in Medical Genetics|language=en|volume=175|issue=1|pages=195–211|doi=10.1002/ajmg.c.31549|issn=1552-4876}}</ref><ref name=":0">{{Cite journal|last=Henderson|first=Fraser C.|last2=Francomano|first2=C. A.|last3=Koby|first3=M.|last4=Tuchman|first4=K.|last5=Adcock|first5=J.|last6=Patel|first6=S.|date=2019-01-09|title=Cervical medullary syndrome secondary to craniocervical instability and ventral brainstem compression in hereditary hypermobility connective tissue disorders: 5-year follow-up after craniocervical reduction, fusion, and stabilization|url=https://doi.org/10.1007/s10143-018-01070-4|journal=Neurosurgical Review|language=en|doi=10.1007/s10143-018-01070-4|issn=1437-2320}}</ref><ref name=":5">{{Cite journal|last=Rebbeck|first=Trudy|last2=Liebert|first2=Ann|date=2014-12-01|title=Clinical management of cranio-vertebral instability after whiplash, when guidelines should be adapted: A case report|url=http://www.sciencedirect.com/science/article/pii/S1356689X14000101|journal=Manual Therapy|volume=19|issue=6|pages=618–621|doi=10.1016/j.math.2014.01.009|issn=1356-689X}}</ref>: common types include an occipital headache or occipital pain (back lower part of the head)<ref name=":4">{{Cite web|url=https://search.proquest.com/openview/34b3b18a8854c04ffa0fc50273d68313/1?pq-origsite=gscholar&cbl=47886|title=MRI video diagnosis and surgical therapy of soft tissue trauma to the craniocervical junction - ProQuest|website=search.proquest.com|language=en|access-date=2019-06-01}}</ref><ref name=":6">{{Cite journal|last=Mathers|first=K. Sean|last2=Schneider|first2=Michael|last3=Timko|first3=Michael|date=2011-06|title=Occult Hypermobility of the Craniocervical Junction: A Case Report and Review|url=https://www.jospt.org/doi/full/10.2519/jospt.2011.3305|journal=Journal of Orthopaedic & Sports Physical Therapy|language=en|volume=41|issue=6|pages=444–457|doi=10.2519/jospt.2011.3305|issn=0190-6011}}</ref>; a pressure headache: an impairment of [[cerebropinsal fluid]] (CSF) flow causes intracranial pressure which would be aggravated by “valsalva maneuvers” such as yawning, laughing, crying, coughing, sneezing or straining; headache behind the eyes<ref name=":6" />; and a feeling that head is too heavy for the neck to support (feeling like a “bobble-head”).
* [[Dysautonomia]]<ref name=":0" /><ref name=":3">{{Cite journal|last=Henderson|first=Fraser C.|last2=Austin|first2=Claudiu|last3=Benzel|first3=Edward|last4=Bolognese|first4=Paolo|last5=Ellenbogen|first5=Richard|last6=Francomano|first6=Clair A.|last7=Ireton|first7=Candace|last8=Klinge|first8=Petra|last9=Koby|first9=Myles|date=2017|title=Neurological and spinal manifestations of the Ehlers–Danlos syndromes|url=https://onlinelibrary.wiley.com/doi/abs/10.1002/ajmg.c.31549|journal=American Journal of Medical Genetics Part C: Seminars in Medical Genetics|language=en|volume=175|issue=1|pages=195–211|doi=10.1002/ajmg.c.31549|issn=1552-4876}}</ref> (e.g., [[heat intolerance]], [[orthostatic intolerance]]<ref name=":1">{{Cite journal|last=Francomano|first=Clair A.|last2=McDonnell|first2=Nazli B.|last3=Nishikawa|first3=Misao|last4=Bolognese|first4=Paolo A.|last5=Milhorat|first5=Thomas H.|date=2007-12-01|title=Syndrome of occipitoatlantoaxial hypermobility, cranial settling, and Chiari malformation Type I in patients with hereditary disorders of connective tissue|url=https://thejns.org/view/journals/j-neurosurg-spine/7/6/article-p601.xml|journal=Journal of Neurosurgery: Spine|language=en-US|volume=7|issue=6|pages=601–609|doi=10.3171/SPI-07/12/601}}</ref>, [[polydipsia]] (extreme thirst), [[delayed gastric emptying]])

* [[Postural orthostatic tachycardia syndrome|postural orthostatic tachycardia]]<ref name=":0" /><ref name=":1" />
* dizziness<ref name=":4" /><ref name=":5" /><ref name=":6" />
* fatigue<ref name=":0" /><ref>{{Cite journal|last=Henderson|first=Fraser C.|last2=Henderson|first2=Fraser C.|last3=Wilson|first3=William A.|last4=Mark|first4=Alexander S.|last5=Koby|first5=Myles|date=2018-01-01|title=Utility of the clivo-axial angle in assessing brainstem deformity: pilot study and literature review|url=https://doi.org/10.1007/s10143-017-0830-3|journal=Neurosurgical Review|language=en|volume=41|issue=1|pages=149–163|doi=10.1007/s10143-017-0830-3|issn=1437-2320|pmc=PMC5748419|pmid=28258417}}</ref><ref>{{Cite journal|last=Henderson|first=Fraser C.|last2=Wilson|first2=William A.|last3=Mott|first3=Stephen|last4=Mark|first4=Alexander|last5=Schmidt|first5=Kristi|last6=Berry|first6=Joel K.|last7=Vaccaro|first7=Alexander|last8=Benzel|first8=Edward|date=2010-07-16|title=Deformative stress associated with an abnormal clivo-axial angle: A finite element analysis|url=https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2940090/|journal=Surgical Neurology International|volume=1|doi=10.4103/2152-7806.66461|issn=2152-7806|pmc=PMCPMC2940090|pmid=20847911}}</ref>
* cognitive impairment<ref name=":4" />
* [[muscle weakness]]<ref name=":0" />

* posterior [[neck pain]]<ref name=":0" /><ref name=":7">{{Cite journal|last=Bergholm|first=Ulla|last2=Johansson|first2=Bengt H.|last3=Johansson|first3=Hakan|date=2004-01-01|title=New Diagnostic Tools Can Contribute to Better Treatment of Patients with Chronic Whiplash Disorders|url=https://doi.org/10.3109/J180v03n02_02|journal=Journal of Whiplash & Related Disorders|volume=3|issue=2|pages=5–19|doi=10.3109/J180v03n02_02|issn=1533-2888}}</ref><ref name=":5" /><ref name=":6" />
* posterior scalp irritation<ref name=":7" />
* neck stiffness, torticollis<ref name=":7" /><ref>{{Cite journal|last=Ghanem|first=Ismat|last2=El Hage|first2=Samer|last3=Rachkidi|first3=Rami|last4=Kharrat|first4=Khalil|last5=Dagher|first5=Fernand|last6=Kreichati|first6=Gabi|date=2008-03-01|title=Pediatric cervical spine instability|url=https://online.boneandjoint.org.uk/doi/full/10.1007/s11832-008-0092-2|journal=Journal of Children's Orthopaedics|volume=2|issue=2|pages=71–84|doi=10.1007/s11832-008-0092-2|issn=1863-2521|pmc=PMC2656787|pmid=19308585}}</ref>
* tinnitus<ref>{{Cite journal|last=Montazem|first=Abbas|author-link=|author-link2=|author-link3=|author-link4=|author-link5=|date=2000|title=Secondary tinnitus as a symptom of instability in the upper cervical spine: Operative management|url=https://pdfs.semanticscholar.org/21c4/85984a6ebe07efed38cf82a2f7a49b2a644e.pdf|journal=International Tinnitus Journal|volume=|issue=|pages=|quote=|via=}}</ref><ref name=":4" />
* central or mixed [[apnea]]<ref>{{Cite journal|last=Janjua|first=M. Burhan|last2=Hwang|first2=Steven W.|last3=Samdani|first3=Amer F.|last4=Pahys|first4=Joshua M.|last5=Baaj|first5=Ali A.|last6=Härtl|first6=Roger|last7=Greenfield|first7=Jeffrey P.|date=2019-01-01|title=Instrumented arthrodesis for non-traumatic craniocervical instability in very young children|url=https://doi.org/10.1007/s00381-018-3876-9|journal=Child's Nervous System|language=en|volume=35|issue=1|pages=97–106|doi=10.1007/s00381-018-3876-9|issn=1433-0350}}</ref><ref name=":1" /><ref name=":3" />
* dyspnea (shortness of breath)<ref name=":0" /><ref name=":1" />
* dysphagia (difficulty swallowing)<ref name=":1" /><ref name=":2" />
* altered sleep architecture/sleep disturbance<ref name=":0" /><ref name=":4" />
* facial pain or [[numbness]]<ref name=":1" /><ref name=":2" />
* [[dizziness]] and [[vertigo]]<ref name=":0" /><ref name=":2" /><ref name=":7" />
* syncope<ref name=":0" /><ref name=":1" /><ref name=":2" /><ref name=":7" />
* visual disturbances<ref name=":4" />
* reduced gag reflex and [[Dysphagia|difficulty swallowing]]
* impaired [[coordination]]
* downward [[nystagmus]] (irregular eye movements)<ref name=":1" /><ref name=":7" />
* [[paralysis]]
* parasthesias<ref name=":0" /><ref name=":4" /><ref name=":5" /><ref name=":6" />
* gait changes<ref name=":0" /><ref name=":7" />
* poor proprioception<ref name=":7" />
* nausea<ref name=":1" /><ref name=":2" />

== Diagnosis ==
Craniocervical instability is diagnosed via radiographic imaging of the craniocervical junction.
== Treatment ==
{{Video|id=https://youtu.be/sEi9AlHQTJc|service=youtube|dimensions=550|description=|alignment=right|urlargs=}}

=== Conservative treatment ===
Treatment can include “conservative measures” such as rest, bracing with a cervical collar, or physical therapy to strengthen neck muscles. Experimental treatments include stem cell therapy, prolotherapy and upper cervical Chiropractic. Many conservative therapies have little to no evidence.

=== Surgery ===
Surgery is considered a last resort for patients with objective radiological findings together with a clinical picture supportive of the diagnosis and major disability. Surgery typically involves the implantation of metal hardware in the occiput, axis and atlas (i.e., C0 to C2) alone with rib graft or cadaver bone craft, although additional vertebrae can also be fused.

A five-year follow up study of twenty Ehlers-Danlos Syndrome patients who underwent craniocervical fusion surgery found no deaths or major post-surgical complications.<ref name=":0" /> 25% of patients later required fusion at another level.<ref name=":0" /> A study of 279 patients undergoing craniocervical fusion for CCI with os odontoideum due to trauma and congenital abnormalities found a 2.4% complication rate.<ref>{{Cite journal|last=Wang|first=Chao|last2=Passias|first2=Peter G.|last3=Wang|first3=Shenglin|last4=Zhao|first4=Deng|date=2015-05-01|title=Craniocervical Instability in the Setting of Os OdontoideumAssessment of Cause, Presentation, and Surgical Outcomes in a Series of 279 Cases|url=https://academic.oup.com/neurosurgery/article/76/5/514/2452018|journal=Neurosurgery|language=en|volume=76|issue=5|pages=514–521|doi=10.1227/NEU.0000000000000668|issn=0148-396X}}</ref>

== Risk factors ==
Patients with [[Ehlers-Danlos syndrome]], Down Syndrome, dwarfism and [[Rheumatoid arthritis]] are at greater risk.

== Co-morbid conditions ==
CCI can co-occur in patients with EDS and other connective tissue disorders with [[intracranial hypertension]]<ref name=":0" /> or [[Intracranial hypotension|hypotension]]<ref name=":0" />, abnormalities of intracranial venous drainage due to sinus stenosis or jugular vein stenosis,<ref name=":0" /> [[Migraine]] headaches, [[Temporomandibular joint disorder|temporomandibular joint]] dysfunction<ref name=":0" /><ref name=":1" />, and scoliosis.<ref name=":1" />

== See also ==

* [[Brainstem compression]]
* [[Ehlers-Danlos syndrome]]
* [[Chiari malformation]]

== Learn more ==

== References ==
<references />
[[Category:Diagnoses]]

Craniocervical instability

2019-06-01T23:49:35Z

Jeff:Added article to the references and cited it under the "fatigue" bulletpoint.

'''Craniocervical instability''' (CCI), also known as the Syndrome of Occipitoatlantialaxial Hypermobility, is a structural instability of the craniocervical junction which may lead to deformation or [[Brainstem compression|compression of the brainstem]], upper [[spinal cord]], and [[cerebellum]]. CCI as well as atlanto-axial instability (AAI), instability between the first and second vertebrae, can frequently co-occur. CCI/AAI can be caused by a traumatic injury, such as whiplash or a high-impact trauma. However, there are several known conditions where CCI/AAI can happen, seemingly spontaneously, in absence of obvious trauma: Down Syndrome, dwarfism, [[rheumatoid arthritis]] (RA), [[Ehlers-Danlos syndrome|Ehlers-Danlos Syndrome]] (EDS) (itself a common co-morbidity with [[myalgic encephalomyelitis|Myalgic Encephalomyelitis]] (ME)), and other [[connective tissue]] disorders. The ligaments of the craniocervical junction, which are made up of connective tissue, become too lax to hold the skull in normal relationship with the cervical spine.

There have been anecdotal reports of people with ME who meet the [[International Consensus Criteria]] (ICC) who were ultimately diagnosed with CCI/AAI and/or [[Chiari malformation]], a related condition.<ref>{{Cite web|url=https://forums.phoenixrising.me/index.php?threads/have-you-ruled-out-chiari-as-a-cause-of-your-cfs.56908/|title=Have you ruled out Chiari as a cause of your CFS|last=|first=|date=|website=Phoenix Rising|archive-url=|archive-date=|dead-url=|access-date=}}</ref><ref>{{Cite web|url=https://www.healthrising.org/blog/2019/05/21/jennifer-brea-chronic-fatigue-mecfs-recovering-story/|title=Jennifer Brea's Amazing ME/CFS Recovering Story: the Spinal Series - Pt. II|last=Johnson|first=Cort|date=2019-05-21|website=Health Rising|language=en-GB|access-date=2019-06-01}}</ref><ref>{{Cite web|url=https://rsci.app.link/3LpJOxcTaX?_p=f3542c5bfc29c2616780177b27|website=rsci.app.link|access-date=2019-06-01}}</ref><ref>{{Cite web|url=https://www.mechanicalbasis.org/|title=Craniocervical instability, Atlantoaxial Instability, Myalgic Encephalomyelitis, ME, CFS|website=MEchanical Basis|language=en|access-date=2019-06-01}}</ref> However, thus far, any potential connection between CCI and ME remains essentially unexplored, as there has been no formal research investigating the relationship between CCI and ME.

== Diagnosis ==
Craniocervical instability is diagnosed via radiographic imaging of the craniocervical junction.

== Symptoms ==
There can be a wide range of clinical presentations. Symptoms<ref>{{Cite web|url=http://thezebranetwork.org/craniocervical-instability/|title=Craniocervical Instability|last=|first=|date=|website=The Zebra Network|archive-url=|archive-date=|dead-url=|access-date=}}</ref> of craniocervical instability and atlantoaxial instability, particularly in the context of a connective tissue disorder, can and ''may'' include:
* [[headache]]<ref name=":2">{{Cite journal|last=Henderson|first=Fraser C.|last2=Austin|first2=Claudiu|last3=Benzel|first3=Edward|last4=Bolognese|first4=Paolo|last5=Ellenbogen|first5=Richard|last6=Francomano|first6=Clair A.|last7=Ireton|first7=Candace|last8=Klinge|first8=Petra|last9=Koby|first9=Myles|date=2017|title=Neurological and spinal manifestations of the Ehlers–Danlos syndromes|url=https://onlinelibrary.wiley.com/doi/abs/10.1002/ajmg.c.31549|journal=American Journal of Medical Genetics Part C: Seminars in Medical Genetics|language=en|volume=175|issue=1|pages=195–211|doi=10.1002/ajmg.c.31549|issn=1552-4876}}</ref><ref name=":0">{{Cite journal|last=Henderson|first=Fraser C.|last2=Francomano|first2=C. A.|last3=Koby|first3=M.|last4=Tuchman|first4=K.|last5=Adcock|first5=J.|last6=Patel|first6=S.|date=2019-01-09|title=Cervical medullary syndrome secondary to craniocervical instability and ventral brainstem compression in hereditary hypermobility connective tissue disorders: 5-year follow-up after craniocervical reduction, fusion, and stabilization|url=https://doi.org/10.1007/s10143-018-01070-4|journal=Neurosurgical Review|language=en|doi=10.1007/s10143-018-01070-4|issn=1437-2320}}</ref><ref name=":5">{{Cite journal|last=Rebbeck|first=Trudy|last2=Liebert|first2=Ann|date=2014-12-01|title=Clinical management of cranio-vertebral instability after whiplash, when guidelines should be adapted: A case report|url=http://www.sciencedirect.com/science/article/pii/S1356689X14000101|journal=Manual Therapy|volume=19|issue=6|pages=618–621|doi=10.1016/j.math.2014.01.009|issn=1356-689X}}</ref>: common types include an occipital headache or occipital pain (back lower part of the head)<ref name=":4">{{Cite web|url=https://search.proquest.com/openview/34b3b18a8854c04ffa0fc50273d68313/1?pq-origsite=gscholar&cbl=47886|title=MRI video diagnosis and surgical therapy of soft tissue trauma to the craniocervical junction - ProQuest|website=search.proquest.com|language=en|access-date=2019-06-01}}</ref><ref name=":6">{{Cite journal|last=Mathers|first=K. Sean|last2=Schneider|first2=Michael|last3=Timko|first3=Michael|date=2011-06|title=Occult Hypermobility of the Craniocervical Junction: A Case Report and Review|url=https://www.jospt.org/doi/full/10.2519/jospt.2011.3305|journal=Journal of Orthopaedic & Sports Physical Therapy|language=en|volume=41|issue=6|pages=444–457|doi=10.2519/jospt.2011.3305|issn=0190-6011}}</ref>; a pressure headache: an impairment of [[cerebropinsal fluid]] (CSF) flow causes intracranial pressure which would be aggravated by “valsalva maneuvers” such as yawning, laughing, crying, coughing, sneezing or straining; headache behind the eyes<ref name=":6" />; and a feeling that head is too heavy for the neck to support (feeling like a “bobble-head”).
* [[Dysautonomia]]<ref name=":0" /><ref name=":3">{{Cite journal|last=Henderson|first=Fraser C.|last2=Austin|first2=Claudiu|last3=Benzel|first3=Edward|last4=Bolognese|first4=Paolo|last5=Ellenbogen|first5=Richard|last6=Francomano|first6=Clair A.|last7=Ireton|first7=Candace|last8=Klinge|first8=Petra|last9=Koby|first9=Myles|date=2017|title=Neurological and spinal manifestations of the Ehlers–Danlos syndromes|url=https://onlinelibrary.wiley.com/doi/abs/10.1002/ajmg.c.31549|journal=American Journal of Medical Genetics Part C: Seminars in Medical Genetics|language=en|volume=175|issue=1|pages=195–211|doi=10.1002/ajmg.c.31549|issn=1552-4876}}</ref> (e.g., [[heat intolerance]], [[orthostatic intolerance]]<ref name=":1">{{Cite journal|last=Francomano|first=Clair A.|last2=McDonnell|first2=Nazli B.|last3=Nishikawa|first3=Misao|last4=Bolognese|first4=Paolo A.|last5=Milhorat|first5=Thomas H.|date=2007-12-01|title=Syndrome of occipitoatlantoaxial hypermobility, cranial settling, and Chiari malformation Type I in patients with hereditary disorders of connective tissue|url=https://thejns.org/view/journals/j-neurosurg-spine/7/6/article-p601.xml|journal=Journal of Neurosurgery: Spine|language=en-US|volume=7|issue=6|pages=601–609|doi=10.3171/SPI-07/12/601}}</ref>, [[polydipsia]] (extreme thirst), [[delayed gastric emptying]], [[chronic fatigue]])

* [[Postural orthostatic tachycardia syndrome|postural orthostatic tachycardia]]<ref name=":0" /><ref name=":1" />
* dizziness<ref name=":4" /><ref name=":5" /><ref name=":6" />
* fatigue<ref name=":0" /><ref>{{Cite journal|last=Henderson|first=Fraser C.|last2=Henderson|first2=Fraser C.|last3=Wilson|first3=William A.|last4=Mark|first4=Alexander S.|last5=Koby|first5=Myles|date=2018-01-01|title=Utility of the clivo-axial angle in assessing brainstem deformity: pilot study and literature review|url=https://doi.org/10.1007/s10143-017-0830-3|journal=Neurosurgical Review|language=en|volume=41|issue=1|pages=149–163|doi=10.1007/s10143-017-0830-3|issn=1437-2320|pmc=PMC5748419|pmid=28258417}}</ref><ref>{{Cite journal|last=Henderson|first=Fraser C.|last2=Wilson|first2=William A.|last3=Mott|first3=Stephen|last4=Mark|first4=Alexander|last5=Schmidt|first5=Kristi|last6=Berry|first6=Joel K.|last7=Vaccaro|first7=Alexander|last8=Benzel|first8=Edward|date=2010-07-16|title=Deformative stress associated with an abnormal clivo-axial angle: A finite element analysis|url=https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2940090/|journal=Surgical Neurology International|volume=1|doi=10.4103/2152-7806.66461|issn=2152-7806|pmc=PMCPMC2940090|pmid=20847911}}</ref>
* cognitive impairment<ref name=":4" />

* posterior [[neck pain]]<ref name=":0" /><ref name=":7">{{Cite journal|last=Bergholm|first=Ulla|last2=Johansson|first2=Bengt H.|last3=Johansson|first3=Hakan|date=2004-01-01|title=New Diagnostic Tools Can Contribute to Better Treatment of Patients with Chronic Whiplash Disorders|url=https://doi.org/10.3109/J180v03n02_02|journal=Journal of Whiplash & Related Disorders|volume=3|issue=2|pages=5–19|doi=10.3109/J180v03n02_02|issn=1533-2888}}</ref><ref name=":5" /><ref name=":6" />
* posterior scalp irritation<ref name=":7" />
* neck stiffness, torticollis<ref name=":7" /><ref>{{Cite journal|last=Ghanem|first=Ismat|last2=El Hage|first2=Samer|last3=Rachkidi|first3=Rami|last4=Kharrat|first4=Khalil|last5=Dagher|first5=Fernand|last6=Kreichati|first6=Gabi|date=2008-03-01|title=Pediatric cervical spine instability|url=https://online.boneandjoint.org.uk/doi/full/10.1007/s11832-008-0092-2|journal=Journal of Children's Orthopaedics|volume=2|issue=2|pages=71–84|doi=10.1007/s11832-008-0092-2|issn=1863-2521|pmc=PMC2656787|pmid=19308585}}</ref>
* tinnitus<ref>{{Cite journal|last=Montazem|first=Abbas|author-link=|author-link2=|author-link3=|author-link4=|author-link5=|date=2000|title=Secondary tinnitus as a symptom of instability in the upper cervical spine: Operative management|url=https://pdfs.semanticscholar.org/21c4/85984a6ebe07efed38cf82a2f7a49b2a644e.pdf|journal=International Tinnitus Journal|volume=|issue=|pages=|quote=|via=}}</ref><ref name=":4" />
* central or mixed [[apnea]]<ref>{{Cite journal|last=Janjua|first=M. Burhan|last2=Hwang|first2=Steven W.|last3=Samdani|first3=Amer F.|last4=Pahys|first4=Joshua M.|last5=Baaj|first5=Ali A.|last6=Härtl|first6=Roger|last7=Greenfield|first7=Jeffrey P.|date=2019-01-01|title=Instrumented arthrodesis for non-traumatic craniocervical instability in very young children|url=https://doi.org/10.1007/s00381-018-3876-9|journal=Child's Nervous System|language=en|volume=35|issue=1|pages=97–106|doi=10.1007/s00381-018-3876-9|issn=1433-0350}}</ref><ref name=":1" /><ref name=":3" />
* dyspnea (shortness of breath)<ref name=":0" /><ref name=":1" />
* dysphagia (difficulty swallowing)<ref name=":1" /><ref name=":2" />
* altered sleep architecture/sleep disturbance<ref name=":0" /><ref name=":4" />
* facial pain or [[numbness]]<ref name=":1" /><ref name=":2" />
* [[muscle weakness]]
* [[dizziness]] and [[vertigo]]<ref name=":0" /><ref name=":2" /><ref name=":7" />
* syncope<ref name=":0" /><ref name=":1" /><ref name=":2" /><ref name=":7" />
* visual disturbances<ref name=":4" />
* reduced gag reflex and [[Dysphagia|difficulty swallowing]]
* impaired [[coordination]]
* downward [[nystagmus]] (irregular eye movements)<ref name=":1" /><ref name=":7" />
* [[paralysis]]
* parasthesias<ref name=":0" /><ref name=":4" /><ref name=":5" /><ref name=":6" />
* gait changes<ref name=":0" /><ref name=":7" />
* poor proprioception<ref name=":7" />
* nausea<ref name=":1" /><ref name=":2" />

== Treatment ==
Treatment can include “conservative measures” such as rest, bracing with a cervical collar, or physical therapy to strengthen neck muscles. Experimental treatments include stem cell therapy, prolotherapy and upper cervical Chiropractic. Many conservative therapies have little to no evidence.

Surgery is considered a last resort for patients with objective radiological findings together with a clinical picture supportive of the diagnosis and major disability. Surgery typically involves the implantation of metal hardware in the occiput, axis and atlas (i.e., C0 to C2) alone with rib graft or cadaver bone craft, although additional vertebrae can also be fused.

A five-year follow up study of twenty Ehlers-Danlos Syndrome patients who underwent craniocervical fusion surgery found no deaths or major post-surgical complications.<ref name=":0" /> 25% of patients later required fusion at another level.<ref name=":0" /> A study of 279 patients undergoing craniocervical fusion for CCI with os odontoideum due to trauma and congenital abnormalities found a 2.4% complication rate.<ref>{{Cite journal|last=Wang|first=Chao|last2=Passias|first2=Peter G.|last3=Wang|first3=Shenglin|last4=Zhao|first4=Deng|date=2015-05-01|title=Craniocervical Instability in the Setting of Os OdontoideumAssessment of Cause, Presentation, and Surgical Outcomes in a Series of 279 Cases|url=https://academic.oup.com/neurosurgery/article/76/5/514/2452018|journal=Neurosurgery|language=en|volume=76|issue=5|pages=514–521|doi=10.1227/NEU.0000000000000668|issn=0148-396X}}</ref>
== Risk factors ==
Patients with [[Ehlers-Danlos syndrome]], Down Syndrome, dwarfism and [[Rheumatoid arthritis]] are at greater risk.

== Co-morbid conditions ==
CCI can co-occur in patients with EDS and other connective tissue disorders with [[intracranial hypertension]]<ref name=":0" /> or [[Intracranial hypotension|hypotension]]<ref name=":0" />, abnormalities of intracranial venous drainage due to sinus stenosis or jugular vein stenosis,<ref name=":0" /> [[Migraine]] headaches, [[Temporomandibular joint disorder|temporomandibular joint]] dysfunction<ref name=":0" /><ref name=":1" />, and scoliosis.<ref name=":1" />

== See also ==

* [[Brainstem compression]]
* [[Ehlers-Danlos syndrome]]
* [[Chiari malformation]]

== Learn more ==

== References ==
<references />
[[Category:Diagnoses]]

Craniocervical instability

2019-06-01T23:45:22Z

Jeff:

'''Craniocervical instability''' (CCI), also known as the Syndrome of Occipitoatlantialaxial Hypermobility, is a structural instability of the craniocervical junction which may lead to deformation or [[Brainstem compression|compression of the brainstem]], upper [[spinal cord]], and [[cerebellum]]. CCI as well as atlanto-axial instability (AAI), instability between the first and second vertebrae, can frequently co-occur. CCI/AAI can be caused by a traumatic injury, such as whiplash or a high-impact trauma. However, there are several known conditions where CCI/AAI can happen, seemingly spontaneously, in absence of obvious trauma: Down Syndrome, dwarfism, [[rheumatoid arthritis]] (RA), [[Ehlers-Danlos syndrome|Ehlers-Danlos Syndrome]] (EDS) (itself a common co-morbidity with [[myalgic encephalomyelitis|Myalgic Encephalomyelitis]] (ME)), and other [[connective tissue]] disorders. The ligaments of the craniocervical junction, which are made up of connective tissue, become too lax to hold the skull in normal relationship with the cervical spine.

There have been anecdotal reports of people with ME who meet the [[International Consensus Criteria]] (ICC) who were ultimately diagnosed with CCI/AAI and/or [[Chiari malformation]], a related condition.<ref>{{Cite web|url=https://forums.phoenixrising.me/index.php?threads/have-you-ruled-out-chiari-as-a-cause-of-your-cfs.56908/|title=Have you ruled out Chiari as a cause of your CFS|last=|first=|date=|website=Phoenix Rising|archive-url=|archive-date=|dead-url=|access-date=}}</ref><ref>{{Cite web|url=https://www.healthrising.org/blog/2019/05/21/jennifer-brea-chronic-fatigue-mecfs-recovering-story/|title=Jennifer Brea's Amazing ME/CFS Recovering Story: the Spinal Series - Pt. II|last=Johnson|first=Cort|date=2019-05-21|website=Health Rising|language=en-GB|access-date=2019-06-01}}</ref><ref>{{Cite web|url=https://rsci.app.link/3LpJOxcTaX?_p=f3542c5bfc29c2616780177b27|website=rsci.app.link|access-date=2019-06-01}}</ref><ref>{{Cite web|url=https://www.mechanicalbasis.org/|title=Craniocervical instability, Atlantoaxial Instability, Myalgic Encephalomyelitis, ME, CFS|website=MEchanical Basis|language=en|access-date=2019-06-01}}</ref> However, thus far, any potential connection between CCI and ME remains essentially unexplored, as there has been no formal research investigating the relationship between CCI and ME.

== Diagnosis ==
Craniocervical instability is diagnosed via radiographic imaging of the craniocervical junction.

== Symptoms ==
There can be a wide range of clinical presentations. Symptoms<ref>{{Cite web|url=http://thezebranetwork.org/craniocervical-instability/|title=Craniocervical Instability|last=|first=|date=|website=The Zebra Network|archive-url=|archive-date=|dead-url=|access-date=}}</ref> of craniocervical instability and atlantoaxial instability, particularly in the context of a connective tissue disorder, can and ''may'' include:
* [[headache]]<ref name=":2">{{Cite journal|last=Henderson|first=Fraser C.|last2=Austin|first2=Claudiu|last3=Benzel|first3=Edward|last4=Bolognese|first4=Paolo|last5=Ellenbogen|first5=Richard|last6=Francomano|first6=Clair A.|last7=Ireton|first7=Candace|last8=Klinge|first8=Petra|last9=Koby|first9=Myles|date=2017|title=Neurological and spinal manifestations of the Ehlers–Danlos syndromes|url=https://onlinelibrary.wiley.com/doi/abs/10.1002/ajmg.c.31549|journal=American Journal of Medical Genetics Part C: Seminars in Medical Genetics|language=en|volume=175|issue=1|pages=195–211|doi=10.1002/ajmg.c.31549|issn=1552-4876}}</ref><ref name=":0">{{Cite journal|last=Henderson|first=Fraser C.|last2=Francomano|first2=C. A.|last3=Koby|first3=M.|last4=Tuchman|first4=K.|last5=Adcock|first5=J.|last6=Patel|first6=S.|date=2019-01-09|title=Cervical medullary syndrome secondary to craniocervical instability and ventral brainstem compression in hereditary hypermobility connective tissue disorders: 5-year follow-up after craniocervical reduction, fusion, and stabilization|url=https://doi.org/10.1007/s10143-018-01070-4|journal=Neurosurgical Review|language=en|doi=10.1007/s10143-018-01070-4|issn=1437-2320}}</ref><ref name=":5">{{Cite journal|last=Rebbeck|first=Trudy|last2=Liebert|first2=Ann|date=2014-12-01|title=Clinical management of cranio-vertebral instability after whiplash, when guidelines should be adapted: A case report|url=http://www.sciencedirect.com/science/article/pii/S1356689X14000101|journal=Manual Therapy|volume=19|issue=6|pages=618–621|doi=10.1016/j.math.2014.01.009|issn=1356-689X}}</ref>: common types include an occipital headache or occipital pain (back lower part of the head)<ref name=":4">{{Cite web|url=https://search.proquest.com/openview/34b3b18a8854c04ffa0fc50273d68313/1?pq-origsite=gscholar&cbl=47886|title=MRI video diagnosis and surgical therapy of soft tissue trauma to the craniocervical junction - ProQuest|website=search.proquest.com|language=en|access-date=2019-06-01}}</ref><ref name=":6">{{Cite journal|last=Mathers|first=K. Sean|last2=Schneider|first2=Michael|last3=Timko|first3=Michael|date=2011-06|title=Occult Hypermobility of the Craniocervical Junction: A Case Report and Review|url=https://www.jospt.org/doi/full/10.2519/jospt.2011.3305|journal=Journal of Orthopaedic & Sports Physical Therapy|language=en|volume=41|issue=6|pages=444–457|doi=10.2519/jospt.2011.3305|issn=0190-6011}}</ref>; a pressure headache: an impairment of [[cerebropinsal fluid]] (CSF) flow causes intracranial pressure which would be aggravated by “valsalva maneuvers” such as yawning, laughing, crying, coughing, sneezing or straining; headache behind the eyes<ref name=":6" />; and a feeling that head is too heavy for the neck to support (feeling like a “bobble-head”).
* [[Dysautonomia]]<ref name=":0" /><ref name=":3">{{Cite journal|last=Henderson|first=Fraser C.|last2=Austin|first2=Claudiu|last3=Benzel|first3=Edward|last4=Bolognese|first4=Paolo|last5=Ellenbogen|first5=Richard|last6=Francomano|first6=Clair A.|last7=Ireton|first7=Candace|last8=Klinge|first8=Petra|last9=Koby|first9=Myles|date=2017|title=Neurological and spinal manifestations of the Ehlers–Danlos syndromes|url=https://onlinelibrary.wiley.com/doi/abs/10.1002/ajmg.c.31549|journal=American Journal of Medical Genetics Part C: Seminars in Medical Genetics|language=en|volume=175|issue=1|pages=195–211|doi=10.1002/ajmg.c.31549|issn=1552-4876}}</ref> (e.g., [[heat intolerance]], [[orthostatic intolerance]]<ref name=":1">{{Cite journal|last=Francomano|first=Clair A.|last2=McDonnell|first2=Nazli B.|last3=Nishikawa|first3=Misao|last4=Bolognese|first4=Paolo A.|last5=Milhorat|first5=Thomas H.|date=2007-12-01|title=Syndrome of occipitoatlantoaxial hypermobility, cranial settling, and Chiari malformation Type I in patients with hereditary disorders of connective tissue|url=https://thejns.org/view/journals/j-neurosurg-spine/7/6/article-p601.xml|journal=Journal of Neurosurgery: Spine|language=en-US|volume=7|issue=6|pages=601–609|doi=10.3171/SPI-07/12/601}}</ref>, [[polydipsia]] (extreme thirst), [[delayed gastric emptying]], [[chronic fatigue]])

* [[Postural orthostatic tachycardia syndrome|postural orthostatic tachycardia]]<ref name=":0" /><ref name=":1" />
* dizziness<ref name=":4" /><ref name=":5" /><ref name=":6" />
* fatigue<ref name=":0" />
* cognitive impairment<ref name=":4" />

* posterior [[neck pain]]<ref name=":0" /><ref name=":7">{{Cite journal|last=Bergholm|first=Ulla|last2=Johansson|first2=Bengt H.|last3=Johansson|first3=Hakan|date=2004-01-01|title=New Diagnostic Tools Can Contribute to Better Treatment of Patients with Chronic Whiplash Disorders|url=https://doi.org/10.3109/J180v03n02_02|journal=Journal of Whiplash & Related Disorders|volume=3|issue=2|pages=5–19|doi=10.3109/J180v03n02_02|issn=1533-2888}}</ref><ref name=":5" /><ref name=":6" />
* posterior scalp irritation<ref name=":7" />
* neck stiffness, torticollis<ref name=":7" /><ref>{{Cite journal|last=Ghanem|first=Ismat|last2=El Hage|first2=Samer|last3=Rachkidi|first3=Rami|last4=Kharrat|first4=Khalil|last5=Dagher|first5=Fernand|last6=Kreichati|first6=Gabi|date=2008-03-01|title=Pediatric cervical spine instability|url=https://online.boneandjoint.org.uk/doi/full/10.1007/s11832-008-0092-2|journal=Journal of Children's Orthopaedics|volume=2|issue=2|pages=71–84|doi=10.1007/s11832-008-0092-2|issn=1863-2521|pmc=PMC2656787|pmid=19308585}}</ref>
* tinnitus<ref>{{Cite journal|last=Montazem|first=Abbas|author-link=|author-link2=|author-link3=|author-link4=|author-link5=|date=2000|title=Secondary tinnitus as a symptom of instability in the upper cervical spine: Operative management|url=https://pdfs.semanticscholar.org/21c4/85984a6ebe07efed38cf82a2f7a49b2a644e.pdf|journal=International Tinnitus Journal|volume=|issue=|pages=|quote=|via=}}</ref><ref name=":4" />
* central or mixed [[apnea]]<ref>{{Cite journal|last=Janjua|first=M. Burhan|last2=Hwang|first2=Steven W.|last3=Samdani|first3=Amer F.|last4=Pahys|first4=Joshua M.|last5=Baaj|first5=Ali A.|last6=Härtl|first6=Roger|last7=Greenfield|first7=Jeffrey P.|date=2019-01-01|title=Instrumented arthrodesis for non-traumatic craniocervical instability in very young children|url=https://doi.org/10.1007/s00381-018-3876-9|journal=Child's Nervous System|language=en|volume=35|issue=1|pages=97–106|doi=10.1007/s00381-018-3876-9|issn=1433-0350}}</ref><ref name=":1" /><ref name=":3" />
* dyspnea (shortness of breath)<ref name=":0" /><ref name=":1" />
* dysphagia (difficulty swallowing)<ref name=":1" /><ref name=":2" />
* altered sleep architecture/sleep disturbance<ref name=":0" /><ref name=":4" />
* facial pain or [[numbness]]<ref name=":1" /><ref name=":2" />
* [[muscle weakness]]
* [[dizziness]] and [[vertigo]]<ref name=":0" /><ref name=":2" /><ref name=":7" />
* syncope<ref name=":0" /><ref name=":1" /><ref name=":2" /><ref name=":7" />
* visual disturbances<ref name=":4" />
* reduced gag reflex and [[Dysphagia|difficulty swallowing]]
* impaired [[coordination]]
* downward [[nystagmus]] (irregular eye movements)<ref name=":1" /><ref name=":7" />
* [[paralysis]]
* parasthesias<ref name=":0" /><ref name=":4" /><ref name=":5" /><ref name=":6" />
* gait changes<ref name=":0" /><ref name=":7" />
* poor proprioception<ref name=":7" />
* nausea<ref name=":1" /><ref name=":2" />

== Treatment ==
Treatment can include “conservative measures” such as rest, bracing with a cervical collar, or physical therapy to strengthen neck muscles. Experimental treatments include stem cell therapy, prolotherapy and upper cervical Chiropractic. Many conservative therapies have little to no evidence.

Surgery is considered a last resort for patients with objective radiological findings together with a clinical picture supportive of the diagnosis and major disability. Surgery typically involves the implantation of metal hardware in the occiput, axis and atlas (i.e., C0 to C2) alone with rib graft or cadaver bone craft, although additional vertebrae can also be fused.

A five-year follow up study of twenty Ehlers-Danlos Syndrome patients who underwent craniocervical fusion surgery found no deaths or major post-surgical complications.<ref name=":0" /> 25% of patients later required fusion at another level.<ref name=":0" /> A study of 279 patients undergoing craniocervical fusion for CCI with os odontoideum due to trauma and congenital abnormalities found a 2.4% complication rate.<ref>{{Cite journal|last=Wang|first=Chao|last2=Passias|first2=Peter G.|last3=Wang|first3=Shenglin|last4=Zhao|first4=Deng|date=2015-05-01|title=Craniocervical Instability in the Setting of Os OdontoideumAssessment of Cause, Presentation, and Surgical Outcomes in a Series of 279 Cases|url=https://academic.oup.com/neurosurgery/article/76/5/514/2452018|journal=Neurosurgery|language=en|volume=76|issue=5|pages=514–521|doi=10.1227/NEU.0000000000000668|issn=0148-396X}}</ref>
== Risk factors ==
Patients with [[Ehlers-Danlos syndrome]], Down Syndrome, dwarfism and [[Rheumatoid arthritis]] are at greater risk.

== Co-morbid conditions ==
CCI can co-occur in patients with EDS and other connective tissue disorders with [[intracranial hypertension]]<ref name=":0" /> or [[Intracranial hypotension|hypotension]]<ref name=":0" />, abnormalities of intracranial venous drainage due to sinus stenosis or jugular vein stenosis,<ref name=":0" /> [[Migraine]] headaches, [[Temporomandibular joint disorder|temporomandibular joint]] dysfunction<ref name=":0" /><ref name=":1" />, and scoliosis.<ref name=":1" />

== See also ==

* [[Brainstem compression]]
* [[Ehlers-Danlos syndrome]]
* [[Chiari malformation]]

== Learn more ==

== References ==
<references />
[[Category:Diagnoses]]

Craniocervical instability

2019-06-01T23:44:50Z

Jeff:/* References */

'''Craniocervical instability''' (CCI), also known as the Syndrome of Occipitoatlantialaxial Hypermobility, is a structural instability of the craniocervical junction which may lead to deformation or [[Brainstem compression|compression of the brainstem]], upper [[spinal cord]], and [[cerebellum]]. CCI as well as atlanto-axial instability (AAI), instability between the first and second vertebrae, can frequently co-occur. CCI/AAI can be caused by a traumatic injury, such as whiplash or a high-impact trauma. However, there are several known conditions where CCI/AAI can happen, seemingly spontaneously, in absence of obvious trauma: Down Syndrome, dwarfism, [[rheumatoid arthritis]] (RA), [[Ehlers-Danlos syndrome|Ehlers-Danlos Syndrome]] (EDS) (itself a common co-morbidity with [[myalgic encephalomyelitis|Myalgic Encephalomyelitis]] (ME)), and other [[connective tissue]] disorders. The ligaments of the craniocervical junction, which are made up of connective tissue, become too lax to hold the skull in normal relationship with the cervical spine.

There have been anecdotal reports of people with ME who meet the [[International Consensus Criteria]] (ICC) who were ultimately diagnosed with CCI/AAI and/or [[Chiari malformation]], a related condition.<ref>{{Cite web|url=https://forums.phoenixrising.me/index.php?threads/have-you-ruled-out-chiari-as-a-cause-of-your-cfs.56908/|title=Have you ruled out Chiari as a cause of your CFS|last=|first=|date=|website=Phoenix Rising|archive-url=|archive-date=|dead-url=|access-date=}}</ref><ref>{{Cite web|url=https://www.healthrising.org/blog/2019/05/21/jennifer-brea-chronic-fatigue-mecfs-recovering-story/|title=Jennifer Brea's Amazing ME/CFS Recovering Story: the Spinal Series - Pt. II|last=Johnson|first=Cort|date=2019-05-21|website=Health Rising|language=en-GB|access-date=2019-06-01}}</ref><ref>{{Cite web|url=https://rsci.app.link/3LpJOxcTaX?_p=f3542c5bfc29c2616780177b27|website=rsci.app.link|access-date=2019-06-01}}</ref><ref>{{Cite web|url=https://www.mechanicalbasis.org/|title=Craniocervical instability, Atlantoaxial Instability, Myalgic Encephalomyelitis, ME, CFS|website=MEchanical Basis|language=en|access-date=2019-06-01}}</ref> However, thus far, any potential connection between CCI and ME remains formally unexplored, as there has been no formal research investigating the relationship between CCI and ME.

== Diagnosis ==
Craniocervical instability is diagnosed via radiographic imaging of the craniocervical junction.

== Symptoms ==
There can be a wide range of clinical presentations. Symptoms<ref>{{Cite web|url=http://thezebranetwork.org/craniocervical-instability/|title=Craniocervical Instability|last=|first=|date=|website=The Zebra Network|archive-url=|archive-date=|dead-url=|access-date=}}</ref> of craniocervical instability and atlantoaxial instability, particularly in the context of a connective tissue disorder, can and ''may'' include:
* [[headache]]<ref name=":2">{{Cite journal|last=Henderson|first=Fraser C.|last2=Austin|first2=Claudiu|last3=Benzel|first3=Edward|last4=Bolognese|first4=Paolo|last5=Ellenbogen|first5=Richard|last6=Francomano|first6=Clair A.|last7=Ireton|first7=Candace|last8=Klinge|first8=Petra|last9=Koby|first9=Myles|date=2017|title=Neurological and spinal manifestations of the Ehlers–Danlos syndromes|url=https://onlinelibrary.wiley.com/doi/abs/10.1002/ajmg.c.31549|journal=American Journal of Medical Genetics Part C: Seminars in Medical Genetics|language=en|volume=175|issue=1|pages=195–211|doi=10.1002/ajmg.c.31549|issn=1552-4876}}</ref><ref name=":0">{{Cite journal|last=Henderson|first=Fraser C.|last2=Francomano|first2=C. A.|last3=Koby|first3=M.|last4=Tuchman|first4=K.|last5=Adcock|first5=J.|last6=Patel|first6=S.|date=2019-01-09|title=Cervical medullary syndrome secondary to craniocervical instability and ventral brainstem compression in hereditary hypermobility connective tissue disorders: 5-year follow-up after craniocervical reduction, fusion, and stabilization|url=https://doi.org/10.1007/s10143-018-01070-4|journal=Neurosurgical Review|language=en|doi=10.1007/s10143-018-01070-4|issn=1437-2320}}</ref><ref name=":5">{{Cite journal|last=Rebbeck|first=Trudy|last2=Liebert|first2=Ann|date=2014-12-01|title=Clinical management of cranio-vertebral instability after whiplash, when guidelines should be adapted: A case report|url=http://www.sciencedirect.com/science/article/pii/S1356689X14000101|journal=Manual Therapy|volume=19|issue=6|pages=618–621|doi=10.1016/j.math.2014.01.009|issn=1356-689X}}</ref>: common types include an occipital headache or occipital pain (back lower part of the head)<ref name=":4">{{Cite web|url=https://search.proquest.com/openview/34b3b18a8854c04ffa0fc50273d68313/1?pq-origsite=gscholar&cbl=47886|title=MRI video diagnosis and surgical therapy of soft tissue trauma to the craniocervical junction - ProQuest|website=search.proquest.com|language=en|access-date=2019-06-01}}</ref><ref name=":6">{{Cite journal|last=Mathers|first=K. Sean|last2=Schneider|first2=Michael|last3=Timko|first3=Michael|date=2011-06|title=Occult Hypermobility of the Craniocervical Junction: A Case Report and Review|url=https://www.jospt.org/doi/full/10.2519/jospt.2011.3305|journal=Journal of Orthopaedic & Sports Physical Therapy|language=en|volume=41|issue=6|pages=444–457|doi=10.2519/jospt.2011.3305|issn=0190-6011}}</ref>; a pressure headache: an impairment of [[cerebropinsal fluid]] (CSF) flow causes intracranial pressure which would be aggravated by “valsalva maneuvers” such as yawning, laughing, crying, coughing, sneezing or straining; headache behind the eyes<ref name=":6" />; and a feeling that head is too heavy for the neck to support (feeling like a “bobble-head”).
* [[Dysautonomia]]<ref name=":0" /><ref name=":3">{{Cite journal|last=Henderson|first=Fraser C.|last2=Austin|first2=Claudiu|last3=Benzel|first3=Edward|last4=Bolognese|first4=Paolo|last5=Ellenbogen|first5=Richard|last6=Francomano|first6=Clair A.|last7=Ireton|first7=Candace|last8=Klinge|first8=Petra|last9=Koby|first9=Myles|date=2017|title=Neurological and spinal manifestations of the Ehlers–Danlos syndromes|url=https://onlinelibrary.wiley.com/doi/abs/10.1002/ajmg.c.31549|journal=American Journal of Medical Genetics Part C: Seminars in Medical Genetics|language=en|volume=175|issue=1|pages=195–211|doi=10.1002/ajmg.c.31549|issn=1552-4876}}</ref> (e.g., [[heat intolerance]], [[orthostatic intolerance]]<ref name=":1">{{Cite journal|last=Francomano|first=Clair A.|last2=McDonnell|first2=Nazli B.|last3=Nishikawa|first3=Misao|last4=Bolognese|first4=Paolo A.|last5=Milhorat|first5=Thomas H.|date=2007-12-01|title=Syndrome of occipitoatlantoaxial hypermobility, cranial settling, and Chiari malformation Type I in patients with hereditary disorders of connective tissue|url=https://thejns.org/view/journals/j-neurosurg-spine/7/6/article-p601.xml|journal=Journal of Neurosurgery: Spine|language=en-US|volume=7|issue=6|pages=601–609|doi=10.3171/SPI-07/12/601}}</ref>, [[polydipsia]] (extreme thirst), [[delayed gastric emptying]], [[chronic fatigue]])

* [[Postural orthostatic tachycardia syndrome|postural orthostatic tachycardia]]<ref name=":0" /><ref name=":1" />
* dizziness<ref name=":4" /><ref name=":5" /><ref name=":6" />
* fatigue<ref name=":0" />
* cognitive impairment<ref name=":4" />

* posterior [[neck pain]]<ref name=":0" /><ref name=":7">{{Cite journal|last=Bergholm|first=Ulla|last2=Johansson|first2=Bengt H.|last3=Johansson|first3=Hakan|date=2004-01-01|title=New Diagnostic Tools Can Contribute to Better Treatment of Patients with Chronic Whiplash Disorders|url=https://doi.org/10.3109/J180v03n02_02|journal=Journal of Whiplash & Related Disorders|volume=3|issue=2|pages=5–19|doi=10.3109/J180v03n02_02|issn=1533-2888}}</ref><ref name=":5" /><ref name=":6" />
* posterior scalp irritation<ref name=":7" />
* neck stiffness, torticollis<ref name=":7" /><ref>{{Cite journal|last=Ghanem|first=Ismat|last2=El Hage|first2=Samer|last3=Rachkidi|first3=Rami|last4=Kharrat|first4=Khalil|last5=Dagher|first5=Fernand|last6=Kreichati|first6=Gabi|date=2008-03-01|title=Pediatric cervical spine instability|url=https://online.boneandjoint.org.uk/doi/full/10.1007/s11832-008-0092-2|journal=Journal of Children's Orthopaedics|volume=2|issue=2|pages=71–84|doi=10.1007/s11832-008-0092-2|issn=1863-2521|pmc=PMC2656787|pmid=19308585}}</ref>
* tinnitus<ref>{{Cite journal|last=Montazem|first=Abbas|author-link=|author-link2=|author-link3=|author-link4=|author-link5=|date=2000|title=Secondary tinnitus as a symptom of instability in the upper cervical spine: Operative management|url=https://pdfs.semanticscholar.org/21c4/85984a6ebe07efed38cf82a2f7a49b2a644e.pdf|journal=International Tinnitus Journal|volume=|issue=|pages=|quote=|via=}}</ref><ref name=":4" />
* central or mixed [[apnea]]<ref>{{Cite journal|last=Janjua|first=M. Burhan|last2=Hwang|first2=Steven W.|last3=Samdani|first3=Amer F.|last4=Pahys|first4=Joshua M.|last5=Baaj|first5=Ali A.|last6=Härtl|first6=Roger|last7=Greenfield|first7=Jeffrey P.|date=2019-01-01|title=Instrumented arthrodesis for non-traumatic craniocervical instability in very young children|url=https://doi.org/10.1007/s00381-018-3876-9|journal=Child's Nervous System|language=en|volume=35|issue=1|pages=97–106|doi=10.1007/s00381-018-3876-9|issn=1433-0350}}</ref><ref name=":1" /><ref name=":3" />
* dyspnea (shortness of breath)<ref name=":0" /><ref name=":1" />
* dysphagia (difficulty swallowing)<ref name=":1" /><ref name=":2" />
* altered sleep architecture/sleep disturbance<ref name=":0" /><ref name=":4" />
* facial pain or [[numbness]]<ref name=":1" /><ref name=":2" />
* [[muscle weakness]]
* [[dizziness]] and [[vertigo]]<ref name=":0" /><ref name=":2" /><ref name=":7" />
* syncope<ref name=":0" /><ref name=":1" /><ref name=":2" /><ref name=":7" />
* visual disturbances<ref name=":4" />
* reduced gag reflex and [[Dysphagia|difficulty swallowing]]
* impaired [[coordination]]
* downward [[nystagmus]] (irregular eye movements)<ref name=":1" /><ref name=":7" />
* [[paralysis]]
* parasthesias<ref name=":0" /><ref name=":4" /><ref name=":5" /><ref name=":6" />
* gait changes<ref name=":0" /><ref name=":7" />
* poor proprioception<ref name=":7" />
* nausea<ref name=":1" /><ref name=":2" />

== Treatment ==
Treatment can include “conservative measures” such as rest, bracing with a cervical collar, or physical therapy to strengthen neck muscles. Experimental treatments include stem cell therapy, prolotherapy and upper cervical Chiropractic. Many conservative therapies have little to no evidence.

Surgery is considered a last resort for patients with objective radiological findings together with a clinical picture supportive of the diagnosis and major disability. Surgery typically involves the implantation of metal hardware in the occiput, axis and atlas (i.e., C0 to C2) alone with rib graft or cadaver bone craft, although additional vertebrae can also be fused.

A five-year follow up study of twenty Ehlers-Danlos Syndrome patients who underwent craniocervical fusion surgery found no deaths or major post-surgical complications.<ref name=":0" /> 25% of patients later required fusion at another level.<ref name=":0" /> A study of 279 patients undergoing craniocervical fusion for CCI with os odontoideum due to trauma and congenital abnormalities found a 2.4% complication rate.<ref>{{Cite journal|last=Wang|first=Chao|last2=Passias|first2=Peter G.|last3=Wang|first3=Shenglin|last4=Zhao|first4=Deng|date=2015-05-01|title=Craniocervical Instability in the Setting of Os OdontoideumAssessment of Cause, Presentation, and Surgical Outcomes in a Series of 279 Cases|url=https://academic.oup.com/neurosurgery/article/76/5/514/2452018|journal=Neurosurgery|language=en|volume=76|issue=5|pages=514–521|doi=10.1227/NEU.0000000000000668|issn=0148-396X}}</ref>
== Risk factors ==
Patients with [[Ehlers-Danlos syndrome]], Down Syndrome, dwarfism and [[Rheumatoid arthritis]] are at greater risk.

== Co-morbid conditions ==
CCI can co-occur in patients with EDS and other connective tissue disorders with [[intracranial hypertension]]<ref name=":0" /> or [[Intracranial hypotension|hypotension]]<ref name=":0" />, abnormalities of intracranial venous drainage due to sinus stenosis or jugular vein stenosis,<ref name=":0" /> [[Migraine]] headaches, [[Temporomandibular joint disorder|temporomandibular joint]] dysfunction<ref name=":0" /><ref name=":1" />, and scoliosis.<ref name=":1" />

== See also ==

* [[Brainstem compression]]
* [[Ehlers-Danlos syndrome]]
* [[Chiari malformation]]

== Learn more ==

== References ==
<references />
[[Category:Diagnoses]]

Craniocervical instability

2019-06-01T23:36:10Z

Jeff:/* Symptoms */

'''Craniocervical instability''' (CCI), also known as the Syndrome of Occipitoatlantialaxial Hypermobility, is a structural instability of the craniocervical junction which may lead to deformation or [[Brainstem compression|compression of the brainstem]], upper [[spinal cord]], and [[cerebellum]]. CCI as well as atlanto-axial instability (AAI), instability between the first and second vertebrae, can frequently co-occur. CCI/AAI can be caused by a traumatic injury, such as whiplash or a high-impact trauma. However, there are several known conditions where CCI/AAI can happen, seemingly spontaneously, in absence of obvious trauma: Down Syndrome, dwarfism, [[rheumatoid arthritis]] (RA), [[Ehlers-Danlos syndrome|Ehlers-Danlos Syndrome]] (EDS) (itself a common co-morbidity with [[myalgic encephalomyelitis|Myalgic Encephalomyelitis]] (ME)), and other [[connective tissue]] disorders. The ligaments of the craniocervical junction, which are made up of connective tissue, become too lax to hold the skull in normal relationship with the cervical spine.

There have been anecdotal reports of people with ME who meet the [[International Consensus Criteria]] (ICC) who were ultimately diagnosed with CCI/AAI and/or [[Chiari malformation]], a related condition.<ref>{{Cite web|url=https://forums.phoenixrising.me/index.php?threads/have-you-ruled-out-chiari-as-a-cause-of-your-cfs.56908/|title=Have you ruled out Chiari as a cause of your CFS|last=|first=|date=|website=Phoenix Rising|archive-url=|archive-date=|dead-url=|access-date=}}</ref><ref>{{Cite web|url=https://www.healthrising.org/blog/2019/05/21/jennifer-brea-chronic-fatigue-mecfs-recovering-story/|title=Jennifer Brea's Amazing ME/CFS Recovering Story: the Spinal Series - Pt. II|last=Johnson|first=Cort|date=2019-05-21|website=Health Rising|language=en-GB|access-date=2019-06-01}}</ref><ref>{{Cite web|url=https://rsci.app.link/3LpJOxcTaX?_p=f3542c5bfc29c2616780177b27|website=rsci.app.link|access-date=2019-06-01}}</ref><ref>{{Cite web|url=https://www.mechanicalbasis.org/|title=Craniocervical instability, Atlantoaxial Instability, Myalgic Encephalomyelitis, ME, CFS|website=MEchanical Basis|language=en|access-date=2019-06-01}}</ref> However, there is no systematic evidence supporting a relationship between CCI and ME.

== Diagnosis ==
Craniocervical instability is diagnosed via radiographic imaging of the craniocervical junction.

== Symptoms ==
There can be a wide range of clinical presentations. Symptoms<ref>{{Cite web|url=http://thezebranetwork.org/craniocervical-instability/|title=Craniocervical Instability|last=|first=|date=|website=The Zebra Network|archive-url=|archive-date=|dead-url=|access-date=}}</ref> of craniocervical instability and atlantoaxial instability, particularly in the context of a connective tissue disorder, can and ''may'' include:
* [[headache]]<ref name=":2">{{Cite journal|last=Henderson|first=Fraser C.|last2=Austin|first2=Claudiu|last3=Benzel|first3=Edward|last4=Bolognese|first4=Paolo|last5=Ellenbogen|first5=Richard|last6=Francomano|first6=Clair A.|last7=Ireton|first7=Candace|last8=Klinge|first8=Petra|last9=Koby|first9=Myles|date=2017|title=Neurological and spinal manifestations of the Ehlers–Danlos syndromes|url=https://onlinelibrary.wiley.com/doi/abs/10.1002/ajmg.c.31549|journal=American Journal of Medical Genetics Part C: Seminars in Medical Genetics|language=en|volume=175|issue=1|pages=195–211|doi=10.1002/ajmg.c.31549|issn=1552-4876}}</ref><ref name=":0">{{Cite journal|last=Henderson|first=Fraser C.|last2=Francomano|first2=C. A.|last3=Koby|first3=M.|last4=Tuchman|first4=K.|last5=Adcock|first5=J.|last6=Patel|first6=S.|date=2019-01-09|title=Cervical medullary syndrome secondary to craniocervical instability and ventral brainstem compression in hereditary hypermobility connective tissue disorders: 5-year follow-up after craniocervical reduction, fusion, and stabilization|url=https://doi.org/10.1007/s10143-018-01070-4|journal=Neurosurgical Review|language=en|doi=10.1007/s10143-018-01070-4|issn=1437-2320}}</ref><ref name=":5">{{Cite journal|last=Rebbeck|first=Trudy|last2=Liebert|first2=Ann|date=2014-12-01|title=Clinical management of cranio-vertebral instability after whiplash, when guidelines should be adapted: A case report|url=http://www.sciencedirect.com/science/article/pii/S1356689X14000101|journal=Manual Therapy|volume=19|issue=6|pages=618–621|doi=10.1016/j.math.2014.01.009|issn=1356-689X}}</ref>: common types include an occipital headache or occipital pain (back lower part of the head)<ref name=":4">{{Cite web|url=https://search.proquest.com/openview/34b3b18a8854c04ffa0fc50273d68313/1?pq-origsite=gscholar&cbl=47886|title=MRI video diagnosis and surgical therapy of soft tissue trauma to the craniocervical junction - ProQuest|website=search.proquest.com|language=en|access-date=2019-06-01}}</ref><ref name=":6">{{Cite journal|last=Mathers|first=K. Sean|last2=Schneider|first2=Michael|last3=Timko|first3=Michael|date=2011-06|title=Occult Hypermobility of the Craniocervical Junction: A Case Report and Review|url=https://www.jospt.org/doi/full/10.2519/jospt.2011.3305|journal=Journal of Orthopaedic & Sports Physical Therapy|language=en|volume=41|issue=6|pages=444–457|doi=10.2519/jospt.2011.3305|issn=0190-6011}}</ref>; a pressure headache: an impairment of [[cerebropinsal fluid]] (CSF) flow causes intracranial pressure which would be aggravated by “valsalva maneuvers” such as yawning, laughing, crying, coughing, sneezing or straining; headache behind the eyes<ref name=":6" />; and a feeling that head is too heavy for the neck to support (feeling like a “bobble-head”).
* [[Dysautonomia]]<ref name=":0" /><ref name=":3">{{Cite journal|last=Henderson|first=Fraser C.|last2=Austin|first2=Claudiu|last3=Benzel|first3=Edward|last4=Bolognese|first4=Paolo|last5=Ellenbogen|first5=Richard|last6=Francomano|first6=Clair A.|last7=Ireton|first7=Candace|last8=Klinge|first8=Petra|last9=Koby|first9=Myles|date=2017|title=Neurological and spinal manifestations of the Ehlers–Danlos syndromes|url=https://onlinelibrary.wiley.com/doi/abs/10.1002/ajmg.c.31549|journal=American Journal of Medical Genetics Part C: Seminars in Medical Genetics|language=en|volume=175|issue=1|pages=195–211|doi=10.1002/ajmg.c.31549|issn=1552-4876}}</ref> (e.g., [[heat intolerance]], [[orthostatic intolerance]]<ref name=":1">{{Cite journal|last=Francomano|first=Clair A.|last2=McDonnell|first2=Nazli B.|last3=Nishikawa|first3=Misao|last4=Bolognese|first4=Paolo A.|last5=Milhorat|first5=Thomas H.|date=2007-12-01|title=Syndrome of occipitoatlantoaxial hypermobility, cranial settling, and Chiari malformation Type I in patients with hereditary disorders of connective tissue|url=https://thejns.org/view/journals/j-neurosurg-spine/7/6/article-p601.xml|journal=Journal of Neurosurgery: Spine|language=en-US|volume=7|issue=6|pages=601–609|doi=10.3171/SPI-07/12/601}}</ref>, [[polydipsia]] (extreme thirst), [[delayed gastric emptying]], [[chronic fatigue]])

* [[Postural orthostatic tachycardia syndrome|postural orthostatic tachycardia]]<ref name=":0" /><ref name=":1" />
* dizziness<ref name=":4" /><ref name=":5" /><ref name=":6" />
* fatigue<ref name=":0" />
* cognitive impairment<ref name=":4" />

* posterior [[neck pain]]<ref name=":0" /><ref name=":7">{{Cite journal|last=Bergholm|first=Ulla|last2=Johansson|first2=Bengt H.|last3=Johansson|first3=Hakan|date=2004-01-01|title=New Diagnostic Tools Can Contribute to Better Treatment of Patients with Chronic Whiplash Disorders|url=https://doi.org/10.3109/J180v03n02_02|journal=Journal of Whiplash & Related Disorders|volume=3|issue=2|pages=5–19|doi=10.3109/J180v03n02_02|issn=1533-2888}}</ref><ref name=":5" /><ref name=":6" />
* posterior scalp irritation<ref name=":7" />
* neck stiffness, torticollis<ref name=":7" /><ref>{{Cite journal|last=Ghanem|first=Ismat|last2=El Hage|first2=Samer|last3=Rachkidi|first3=Rami|last4=Kharrat|first4=Khalil|last5=Dagher|first5=Fernand|last6=Kreichati|first6=Gabi|date=2008-03-01|title=Pediatric cervical spine instability|url=https://online.boneandjoint.org.uk/doi/full/10.1007/s11832-008-0092-2|journal=Journal of Children's Orthopaedics|volume=2|issue=2|pages=71–84|doi=10.1007/s11832-008-0092-2|issn=1863-2521|pmc=PMC2656787|pmid=19308585}}</ref>
* tinnitus<ref>{{Cite journal|last=Montazem|first=Abbas|author-link=|author-link2=|author-link3=|author-link4=|author-link5=|date=2000|title=Secondary tinnitus as a symptom of instability in the upper cervical spine: Operative management|url=https://pdfs.semanticscholar.org/21c4/85984a6ebe07efed38cf82a2f7a49b2a644e.pdf|journal=International Tinnitus Journal|volume=|issue=|pages=|quote=|via=}}</ref><ref name=":4" />
* central or mixed [[apnea]]<ref>{{Cite journal|last=Janjua|first=M. Burhan|last2=Hwang|first2=Steven W.|last3=Samdani|first3=Amer F.|last4=Pahys|first4=Joshua M.|last5=Baaj|first5=Ali A.|last6=Härtl|first6=Roger|last7=Greenfield|first7=Jeffrey P.|date=2019-01-01|title=Instrumented arthrodesis for non-traumatic craniocervical instability in very young children|url=https://doi.org/10.1007/s00381-018-3876-9|journal=Child's Nervous System|language=en|volume=35|issue=1|pages=97–106|doi=10.1007/s00381-018-3876-9|issn=1433-0350}}</ref><ref name=":1" /><ref name=":3" />
* dyspnea (shortness of breath)<ref name=":0" /><ref name=":1" />
* dysphagia (difficulty swallowing)<ref name=":1" /><ref name=":2" />
* altered sleep architecture/sleep disturbance<ref name=":0" /><ref name=":4" />
* facial pain or [[numbness]]<ref name=":1" /><ref name=":2" />
* [[muscle weakness]]
* [[dizziness]] and [[vertigo]]<ref name=":0" /><ref name=":2" /><ref name=":7" />
* syncope<ref name=":0" /><ref name=":1" /><ref name=":2" /><ref name=":7" />
* visual disturbances<ref name=":4" />
* reduced gag reflex and [[Dysphagia|difficulty swallowing]]
* impaired [[coordination]]
* downward [[nystagmus]] (irregular eye movements)<ref name=":1" /><ref name=":7" />
* [[paralysis]]
* parasthesias<ref name=":0" /><ref name=":4" /><ref name=":5" /><ref name=":6" />
* gait changes<ref name=":0" /><ref name=":7" />
* poor proprioception<ref name=":7" />
* nausea<ref name=":1" /><ref name=":2" />

== Treatment ==
Treatment can include “conservative measures” such as rest, bracing with a cervical collar, or physical therapy to strengthen neck muscles. Experimental treatments include stem cell therapy, prolotherapy and upper cervical Chiropractic. Many conservative therapies have little to no evidence.

Surgery is considered a last resort for patients with objective radiological findings together with a clinical picture supportive of the diagnosis and major disability. Surgery typically involves the implantation of metal hardware in the occiput, axis and atlas (i.e., C0 to C2) alone with rib graft or cadaver bone craft, although additional vertebrae can also be fused.

A five-year follow up study of twenty Ehlers-Danlos Syndrome patients who underwent craniocervical fusion surgery found no deaths or major post-surgical complications.<ref name=":0" /> 25% of patients later required fusion at another level.<ref name=":0" /> A study of 279 patients undergoing craniocervical fusion for CCI with os odontoideum due to trauma and congenital abnormalities found a 2.4% complication rate.<ref>{{Cite journal|last=Wang|first=Chao|last2=Passias|first2=Peter G.|last3=Wang|first3=Shenglin|last4=Zhao|first4=Deng|date=2015-05-01|title=Craniocervical Instability in the Setting of Os OdontoideumAssessment of Cause, Presentation, and Surgical Outcomes in a Series of 279 Cases|url=https://academic.oup.com/neurosurgery/article/76/5/514/2452018|journal=Neurosurgery|language=en|volume=76|issue=5|pages=514–521|doi=10.1227/NEU.0000000000000668|issn=0148-396X}}</ref>
== Risk factors ==
Patients with [[Ehlers-Danlos syndrome]], Down Syndrome, dwarfism and [[Rheumatoid arthritis]] are at greater risk.

== Co-morbid conditions ==
CCI can co-occur in patients with EDS and other connective tissue disorders with [[intracranial hypertension]]<ref name=":0" /> or [[Intracranial hypotension|hypotension]]<ref name=":0" />, abnormalities of intracranial venous drainage due to sinus stenosis or jugular vein stenosis,<ref name=":0" /> [[Migraine]] headaches, [[Temporomandibular joint disorder|temporomandibular joint]] dysfunction<ref name=":0" /><ref name=":1" />, and scoliosis.<ref name=":1" />

== See also ==

* [[Brainstem compression]]
* [[Ehlers-Danlos syndrome]]
* [[Chiari malformation]]

== Learn more ==

== References ==
<references />
[[Category:Diagnoses]]

Brainstem

2018-10-27T03:40:06Z

Jeff:added a few words for clarity

Brainstem compression often occurs as a result of [[Craniocervical instability|craniocervical instability (CCI)]].<ref name=":0">{{Cite journal|last=Henderson|first=Fraser C.|last2=Henderson|first2=Fraser C.|last3=Wilson|first3=William A.|last4=Mark|first4=Alexander S.|last5=Koby|first5=Myles|date=2017-03-03|title=Utility of the clivo-axial angle in assessing brainstem deformity: pilot study and literature review|url=https://link.springer.com/article/10.1007/s10143-017-0830-3|journal=Neurosurgical Review|language=en|volume=41|issue=1|pages=149–163|doi=10.1007/s10143-017-0830-3|issn=0344-5607|pmc=PMC5748419|pmid=28258417}}</ref><ref name=":1">{{Cite journal|last=Henderson|first=Fraser C.|last2=Wilson|first2=William A.|last3=Mott|first3=Stephen|last4=Mark|first4=Alexander|last5=Schmidt|first5=Kristi|last6=Berry|first6=Joel K.|last7=Vaccaro|first7=Alexander|last8=Benzel|first8=Edward|date=2010-07-16|title=Deformative stress associated with an abnormal clivo-axial angle: A finite element analysis|url=https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2940090/|journal=Surgical Neurology International|volume=1|doi=10.4103/2152-7806.66461|issn=2152-7806|pmc=PMC2940090|pmid=20847911}}</ref>
==Evidence==
Recent peer-reviewed research indicates that mechanical brainstem compression can be directly responsible for [[dysautonomia]] and fatigue.<ref name=":0" /><ref name=":1" /> Several research studies have shown that when patients had a fusion surgery to correct their brainstem compression, their symptoms of brainstem compression significantly improved or resolved completely.<ref name=":1" />

There are anecdotal accounts of all ME/CFS symptoms, including [[Postural orthostatic tachycardia syndrome|POTS]] and fatigue, going away after a craniocervical fusion to correct the brainstem compression.<ref>{{Cite news|url=https://forums.phoenixrising.me/index.php?threads/have-you-ruled-out-chiari-as-a-cause-of-your-cfs.56908/#post-945253|title=Have you ruled out Chiari as a cause of your CFS|work=Phoenix Rising ME / CFS Forums|access-date=2018-10-26|language=en-US}}</ref>

As the brainstem is central to regulating the [[autonomic nervous system]],<ref>{{Cite journal|last=Martín-Gallego|first=A.|last2=González-García|first2=L.|last3=Carrasco-Brenes|first3=A.|last4=Segura-Fernández-Nogueras|first4=M.|last5=Delgado-Babiano|first5=A.|last6=Ros-Sanjuán|first6=A.|last7=Romero-Moreno|first7=L.|last8=Domínguez-Páez|first8=M.|last9=Dawid-Milner|first9=M. S.|date=2017|title=Brainstem and Autonomic Nervous System Dysfunction: A Neurosurgical Point of View|url=https://www.ncbi.nlm.nih.gov/pubmed/28120078|journal=Acta Neurochirurgica. Supplement|volume=124|pages=221–229|doi=10.1007/978-3-319-39546-3_34|issn=0065-1419|pmid=28120078}}</ref> it is perhaps unsurprising that dysautonomia (such as POTS) can be alleviated by correcting brainstem compression.
==Diagnosis==
Brainstem compression is diagnosed via dynamic imaging, such as flexion-extension MRIs and rotational CT scans. The most common measurements used to diagnose and quantify brainstem compression are the Clivo-Axial Angle, the Grabb-Oakes Measurement, and the Basion-Dens Interval.<ref name=":1" />

==Treatment==
A cervical collar can be tried to see if it helps alleviate the neurological symptoms (even if only partially). However, a cervical collar is not an actual treatment for CCI. The standard treatment to correct brainstem compression is a craniocervical fusion surgery,<ref name=":1" /> performed by a CCI-literate neurosurgeon.
==See also==
* [[Craniocervical instability]]
* Cervical stenosis
* Autonomic control of energy metabolism

==References==
<references />

Brainstem

2018-10-26T23:52:08Z

Jeff:corrected a capitalization error

Brainstem compression often occurs as a result of [[Craniocervical instability|craniocervical instability (CCI)]].<ref name=":0">{{Cite journal|last=Henderson|first=Fraser C.|last2=Henderson|first2=Fraser C.|last3=Wilson|first3=William A.|last4=Mark|first4=Alexander S.|last5=Koby|first5=Myles|date=2017-03-03|title=Utility of the clivo-axial angle in assessing brainstem deformity: pilot study and literature review|url=https://link.springer.com/article/10.1007/s10143-017-0830-3|journal=Neurosurgical Review|language=en|volume=41|issue=1|pages=149–163|doi=10.1007/s10143-017-0830-3|issn=0344-5607|pmc=PMC5748419|pmid=28258417}}</ref><ref name=":1">{{Cite journal|last=Henderson|first=Fraser C.|last2=Wilson|first2=William A.|last3=Mott|first3=Stephen|last4=Mark|first4=Alexander|last5=Schmidt|first5=Kristi|last6=Berry|first6=Joel K.|last7=Vaccaro|first7=Alexander|last8=Benzel|first8=Edward|date=2010-07-16|title=Deformative stress associated with an abnormal clivo-axial angle: A finite element analysis|url=https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2940090/|journal=Surgical Neurology International|volume=1|doi=10.4103/2152-7806.66461|issn=2152-7806|pmc=PMC2940090|pmid=20847911}}</ref>
==Evidence==
Recent peer-reviewed research indicates that mechanical brainstem compression can be directly responsible for [[dysautonomia]] and fatigue.<ref name=":0" /><ref name=":1" /> When patients had a fusion surgery to correct their brainstem compression, their symptoms of brainstem compression significantly improved or resolved completely.<ref name=":1" />

There are anecdotal accounts of all ME/CFS symptoms, including [[Postural orthostatic tachycardia syndrome|POTS]] and fatigue, going away after a craniocervical fusion to correct the brainstem compression.<ref>{{Cite news|url=https://forums.phoenixrising.me/index.php?threads/have-you-ruled-out-chiari-as-a-cause-of-your-cfs.56908/#post-945253|title=Have you ruled out Chiari as a cause of your CFS|work=Phoenix Rising ME / CFS Forums|access-date=2018-10-26|language=en-US}}</ref>

As the brainstem is central to regulating the [[autonomic nervous system]],<ref>{{Cite journal|last=Martín-Gallego|first=A.|last2=González-García|first2=L.|last3=Carrasco-Brenes|first3=A.|last4=Segura-Fernández-Nogueras|first4=M.|last5=Delgado-Babiano|first5=A.|last6=Ros-Sanjuán|first6=A.|last7=Romero-Moreno|first7=L.|last8=Domínguez-Páez|first8=M.|last9=Dawid-Milner|first9=M. S.|date=2017|title=Brainstem and Autonomic Nervous System Dysfunction: A Neurosurgical Point of View|url=https://www.ncbi.nlm.nih.gov/pubmed/28120078|journal=Acta Neurochirurgica. Supplement|volume=124|pages=221–229|doi=10.1007/978-3-319-39546-3_34|issn=0065-1419|pmid=28120078}}</ref> it is perhaps unsurprising that dysautonomia (such as POTS) can be alleviated by correcting brainstem compression.
==Diagnosis==
Brainstem compression is diagnosed via dynamic imaging, such as flexion-extension MRIs and rotational CT scans. The most common measurements used to diagnose and quantify brainstem compression are the Clivo-Axial Angle, the Grabb-Oakes Measurement, and the Basion-Dens Interval.<ref name=":1" />

==Treatment==
A cervical collar can be tried to see if it helps alleviate the neurological symptoms (even if only partially). However, a cervical collar is not an actual treatment for CCI. The standard treatment to correct brainstem compression is a craniocervical fusion surgery,<ref name=":1" /> performed by a CCI-literate neurosurgeon.
==See also==
* [[Craniocervical instability]]
* Cervical stenosis
* Autonomic control of energy metabolism

==References==
<references />

Craniocervical instability

2018-10-26T23:50:49Z

Jeff:/* See also */ I added "Brainstem compression" and linked to that new page.

Craniocervical instability

2018-10-26T23:49:49Z

Jeff:/* See also */

Craniocervical instability

2018-10-26T23:48:52Z

Jeff:I linked this article to the internal MEpedia page "Brainstem compression"

User:Jeff

2018-10-26T23:46:57Z

Jeff:Created page with "Hi, I'm Jeff."

Hi, I'm Jeff.

Brainstem

2018-10-26T23:37:40Z

Jeff:/* Evidence */ I cited a primary source article describing the brainstem's control of the ANS. I also internally linked to the ANS MEpedia page.

Brainstem Compression often occurs as a result of [[Craniocervical instability|craniocervical instability (CCI)]].<ref name=":0">{{Cite journal|last=Henderson|first=Fraser C.|last2=Henderson|first2=Fraser C.|last3=Wilson|first3=William A.|last4=Mark|first4=Alexander S.|last5=Koby|first5=Myles|date=2017-03-03|title=Utility of the clivo-axial angle in assessing brainstem deformity: pilot study and literature review|url=https://link.springer.com/article/10.1007/s10143-017-0830-3|journal=Neurosurgical Review|language=en|volume=41|issue=1|pages=149–163|doi=10.1007/s10143-017-0830-3|issn=0344-5607|pmc=PMC5748419|pmid=28258417}}</ref><ref name=":1">{{Cite journal|last=Henderson|first=Fraser C.|last2=Wilson|first2=William A.|last3=Mott|first3=Stephen|last4=Mark|first4=Alexander|last5=Schmidt|first5=Kristi|last6=Berry|first6=Joel K.|last7=Vaccaro|first7=Alexander|last8=Benzel|first8=Edward|date=2010-07-16|title=Deformative stress associated with an abnormal clivo-axial angle: A finite element analysis|url=https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2940090/|journal=Surgical Neurology International|volume=1|doi=10.4103/2152-7806.66461|issn=2152-7806|pmc=PMC2940090|pmid=20847911}}</ref>
==Evidence==
Recent peer-reviewed research indicates that mechanical brainstem compression can be directly responsible for [[dysautonomia]] and fatigue.<ref name=":0" /><ref name=":1" /> When patients had a fusion surgery to correct their brainstem compression, their symptoms of brainstem compression significantly improved or resolved completely.<ref name=":1" />

There are anecdotal accounts of all ME/CFS symptoms, including [[Postural orthostatic tachycardia syndrome|POTS]] and fatigue, going away after a craniocervical fusion to correct the brainstem compression.<ref>{{Cite news|url=https://forums.phoenixrising.me/index.php?threads/have-you-ruled-out-chiari-as-a-cause-of-your-cfs.56908/#post-945253|title=Have you ruled out Chiari as a cause of your CFS|work=Phoenix Rising ME / CFS Forums|access-date=2018-10-26|language=en-US}}</ref>

As the brainstem is central to regulating the [[autonomic nervous system]],<ref>{{Cite journal|last=Martín-Gallego|first=A.|last2=González-García|first2=L.|last3=Carrasco-Brenes|first3=A.|last4=Segura-Fernández-Nogueras|first4=M.|last5=Delgado-Babiano|first5=A.|last6=Ros-Sanjuán|first6=A.|last7=Romero-Moreno|first7=L.|last8=Domínguez-Páez|first8=M.|last9=Dawid-Milner|first9=M. S.|date=2017|title=Brainstem and Autonomic Nervous System Dysfunction: A Neurosurgical Point of View|url=https://www.ncbi.nlm.nih.gov/pubmed/28120078|journal=Acta Neurochirurgica. Supplement|volume=124|pages=221–229|doi=10.1007/978-3-319-39546-3_34|issn=0065-1419|pmid=28120078}}</ref> it is perhaps unsurprising that dysautonomia (such as POTS) can be alleviated by correcting brainstem compression.
==Diagnosis==
Brainstem compression is diagnosed via dynamic imaging, such as flexion-extension MRIs and rotational CT scans. The most common measurements used to diagnose and quantify brainstem compression are the Clivo-Axial Angle, the Grabb-Oakes Measurement, and the Basion-Dens Interval.<ref name=":1" />

==Treatment==
A cervical collar can be tried to see if it helps alleviate the neurological symptoms (even if only partially). However, a cervical collar is not an actual treatment for CCI. The standard treatment to correct brainstem compression is a craniocervical fusion surgery,<ref name=":1" /> performed by a CCI-literate neurosurgeon.
==See also==
* [[Craniocervical instability]]
* Cervical stenosis
* Autonomic control of energy metabolism

==References==
<references />

Brainstem

2018-10-26T23:33:22Z

Jeff:/* Evidence */

Brainstem Compression often occurs as a result of [[Craniocervical instability|craniocervical instability (CCI)]].<ref name=":0">{{Cite journal|last=Henderson|first=Fraser C.|last2=Henderson|first2=Fraser C.|last3=Wilson|first3=William A.|last4=Mark|first4=Alexander S.|last5=Koby|first5=Myles|date=2017-03-03|title=Utility of the clivo-axial angle in assessing brainstem deformity: pilot study and literature review|url=https://link.springer.com/article/10.1007/s10143-017-0830-3|journal=Neurosurgical Review|language=en|volume=41|issue=1|pages=149–163|doi=10.1007/s10143-017-0830-3|issn=0344-5607|pmc=PMC5748419|pmid=28258417}}</ref><ref name=":1">{{Cite journal|last=Henderson|first=Fraser C.|last2=Wilson|first2=William A.|last3=Mott|first3=Stephen|last4=Mark|first4=Alexander|last5=Schmidt|first5=Kristi|last6=Berry|first6=Joel K.|last7=Vaccaro|first7=Alexander|last8=Benzel|first8=Edward|date=2010-07-16|title=Deformative stress associated with an abnormal clivo-axial angle: A finite element analysis|url=https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2940090/|journal=Surgical Neurology International|volume=1|doi=10.4103/2152-7806.66461|issn=2152-7806|pmc=PMC2940090|pmid=20847911}}</ref>
==Evidence==
Recent peer-reviewed research indicates that mechanical brainstem compression can be directly responsible for [[dysautonomia]] and fatigue.<ref name=":0" /><ref name=":1" /> When patients had a fusion surgery to correct their brainstem compression, their symptoms of brainstem compression significantly improved or resolved completely.<ref name=":1" />

There are anecdotal accounts of all ME/CFS symptoms, including [[Postural orthostatic tachycardia syndrome|POTS]] and fatigue, going away after a craniocervical fusion to correct the brainstem compression.<ref>{{Cite news|url=https://forums.phoenixrising.me/index.php?threads/have-you-ruled-out-chiari-as-a-cause-of-your-cfs.56908/#post-945253|title=Have you ruled out Chiari as a cause of your CFS|work=Phoenix Rising ME / CFS Forums|access-date=2018-10-26|language=en-US}}</ref>

As the brainstem is central to regulating the autonomic nervous system, it is perhaps unsurprising that dysautonomia (such as POTS) can be corrected by correcting brainstem compression.
==Diagnosis==
Brainstem compression is diagnosed via dynamic imaging, such as flexion-extension MRIs and rotational CT scans. The most common measurements used to diagnose and quantify brainstem compression are the Clivo-Axial Angle, the Grabb-Oakes Measurement, and the Basion-Dens Interval.<ref name=":1" />

==Treatment==
A cervical collar can be tried to see if it helps alleviate the neurological symptoms (even if only partially). However, a cervical collar is not an actual treatment for CCI. The standard treatment to correct brainstem compression is a craniocervical fusion surgery,<ref name=":1" /> performed by a CCI-literate neurosurgeon.
==See also==
* [[Craniocervical instability]]
* Cervical stenosis
* Autonomic control of energy metabolism

==References==
<references />

Brainstem

2018-10-26T23:32:07Z

Jeff:linked in-article text to another MEpedia page

Brainstem Compression often occurs as a result of [[Craniocervical instability|craniocervical instability (CCI)]].<ref name=":0">{{Cite journal|last=Henderson|first=Fraser C.|last2=Henderson|first2=Fraser C.|last3=Wilson|first3=William A.|last4=Mark|first4=Alexander S.|last5=Koby|first5=Myles|date=2017-03-03|title=Utility of the clivo-axial angle in assessing brainstem deformity: pilot study and literature review|url=https://link.springer.com/article/10.1007/s10143-017-0830-3|journal=Neurosurgical Review|language=en|volume=41|issue=1|pages=149–163|doi=10.1007/s10143-017-0830-3|issn=0344-5607|pmc=PMC5748419|pmid=28258417}}</ref><ref name=":1">{{Cite journal|last=Henderson|first=Fraser C.|last2=Wilson|first2=William A.|last3=Mott|first3=Stephen|last4=Mark|first4=Alexander|last5=Schmidt|first5=Kristi|last6=Berry|first6=Joel K.|last7=Vaccaro|first7=Alexander|last8=Benzel|first8=Edward|date=2010-07-16|title=Deformative stress associated with an abnormal clivo-axial angle: A finite element analysis|url=https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2940090/|journal=Surgical Neurology International|volume=1|doi=10.4103/2152-7806.66461|issn=2152-7806|pmc=PMC2940090|pmid=20847911}}</ref>
==Evidence==
Recent peer-reviewed research indicates that mechanical brainstem compression can be directly responsible for dysautonomia and fatigue.<ref name=":0" /><ref name=":1" /> When patients had a fusion surgery to correct their brainstem compression, their symptoms of brainstem compression resolved.<ref name=":1" />

There are anecdotal accounts of all ME/CFS symptoms, including POTS and fatigue, going away after a craniocervical fusion to correct the brainstem compression.<ref>{{Cite news|url=https://forums.phoenixrising.me/index.php?threads/have-you-ruled-out-chiari-as-a-cause-of-your-cfs.56908/#post-945253|title=Have you ruled out Chiari as a cause of your CFS|work=Phoenix Rising ME / CFS Forums|access-date=2018-10-26|language=en-US}}</ref>

As the brainstem is central to regulating the autonomic nervous system, it is perhaps unsurprising that dysautonomia (such as POTS) can be corrected by correcting brainstem compression.
==Diagnosis==
Brainstem compression is diagnosed via dynamic imaging, such as flexion-extension MRIs and rotational CT scans. The most common measurements used to diagnose and quantify brainstem compression are the Clivo-Axial Angle, the Grabb-Oakes Measurement, and the Basion-Dens Interval.<ref name=":1" />

==Treatment==
A cervical collar can be tried to see if it helps alleviate the neurological symptoms (even if only partially). However, a cervical collar is not an actual treatment for CCI. The standard treatment to correct brainstem compression is a craniocervical fusion surgery,<ref name=":1" /> performed by a CCI-literate neurosurgeon.
==See also==
* [[Craniocervical instability]]
* Cervical stenosis
* Autonomic control of energy metabolism

==References==
<references />

Brainstem

2018-10-26T23:30:37Z

Jeff:/* See also */

Brainstem Compression often occurs as a result of craniocervical instability (CCI).<ref name=":0">{{Cite journal|last=Henderson|first=Fraser C.|last2=Henderson|first2=Fraser C.|last3=Wilson|first3=William A.|last4=Mark|first4=Alexander S.|last5=Koby|first5=Myles|date=2017-03-03|title=Utility of the clivo-axial angle in assessing brainstem deformity: pilot study and literature review|url=https://link.springer.com/article/10.1007/s10143-017-0830-3|journal=Neurosurgical Review|language=en|volume=41|issue=1|pages=149–163|doi=10.1007/s10143-017-0830-3|issn=0344-5607|pmc=PMC5748419|pmid=28258417}}</ref><ref name=":1">{{Cite journal|last=Henderson|first=Fraser C.|last2=Wilson|first2=William A.|last3=Mott|first3=Stephen|last4=Mark|first4=Alexander|last5=Schmidt|first5=Kristi|last6=Berry|first6=Joel K.|last7=Vaccaro|first7=Alexander|last8=Benzel|first8=Edward|date=2010-07-16|title=Deformative stress associated with an abnormal clivo-axial angle: A finite element analysis|url=https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2940090/|journal=Surgical Neurology International|volume=1|doi=10.4103/2152-7806.66461|issn=2152-7806|pmc=PMC2940090|pmid=20847911}}</ref>
==Evidence==
Recent peer-reviewed research indicates that mechanical brainstem compression can be directly responsible for dysautonomia and fatigue.<ref name=":0" /><ref name=":1" /> When patients had a fusion surgery to correct their brainstem compression, their symptoms of brainstem compression resolved.<ref name=":1" />

There are anecdotal accounts of all ME/CFS symptoms, including POTS and fatigue, going away after a craniocervical fusion to correct the brainstem compression.<ref>{{Cite news|url=https://forums.phoenixrising.me/index.php?threads/have-you-ruled-out-chiari-as-a-cause-of-your-cfs.56908/#post-945253|title=Have you ruled out Chiari as a cause of your CFS|work=Phoenix Rising ME / CFS Forums|access-date=2018-10-26|language=en-US}}</ref>

As the brainstem is central to regulating the autonomic nervous system, it is perhaps unsurprising that dysautonomia (such as POTS) can be corrected by correcting brainstem compression.
==Diagnosis==
Brainstem compression is diagnosed via dynamic imaging, such as flexion-extension MRIs and rotational CT scans. The most common measurements used to diagnose and quantify brainstem compression are the Clivo-Axial Angle, the Grabb-Oakes Measurement, and the Basion-Dens Interval.<ref name=":1" />

==Treatment==
A cervical collar can be tried to see if it helps alleviate the neurological symptoms (even if only partially). However, a cervical collar is not an actual treatment for CCI. The standard treatment to correct brainstem compression is a craniocervical fusion surgery,<ref name=":1" /> performed by a CCI-literate neurosurgeon.
==See also==
* [[Craniocervical instability]]
* Cervical stenosis
* Autonomic control of energy metabolism

==References==
<references />

Brainstem

2018-10-26T23:21:50Z

Jeff:I made the wording more concise

Brainstem Compression often occurs as a result of craniocervical instability (CCI).<ref name=":0">{{Cite journal|last=Henderson|first=Fraser C.|last2=Henderson|first2=Fraser C.|last3=Wilson|first3=William A.|last4=Mark|first4=Alexander S.|last5=Koby|first5=Myles|date=2017-03-03|title=Utility of the clivo-axial angle in assessing brainstem deformity: pilot study and literature review|url=https://link.springer.com/article/10.1007/s10143-017-0830-3|journal=Neurosurgical Review|language=en|volume=41|issue=1|pages=149–163|doi=10.1007/s10143-017-0830-3|issn=0344-5607|pmc=PMC5748419|pmid=28258417}}</ref><ref name=":1">{{Cite journal|last=Henderson|first=Fraser C.|last2=Wilson|first2=William A.|last3=Mott|first3=Stephen|last4=Mark|first4=Alexander|last5=Schmidt|first5=Kristi|last6=Berry|first6=Joel K.|last7=Vaccaro|first7=Alexander|last8=Benzel|first8=Edward|date=2010-07-16|title=Deformative stress associated with an abnormal clivo-axial angle: A finite element analysis|url=https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2940090/|journal=Surgical Neurology International|volume=1|doi=10.4103/2152-7806.66461|issn=2152-7806|pmc=PMC2940090|pmid=20847911}}</ref>
==Evidence==
Recent peer-reviewed research indicates that mechanical brainstem compression can be directly responsible for dysautonomia and fatigue.<ref name=":0" /><ref name=":1" /> When patients had a fusion surgery to correct their brainstem compression, their symptoms of brainstem compression resolved.<ref name=":1" />

There are anecdotal accounts of all ME/CFS symptoms, including POTS and fatigue, going away after a craniocervical fusion to correct the brainstem compression.<ref>{{Cite news|url=https://forums.phoenixrising.me/index.php?threads/have-you-ruled-out-chiari-as-a-cause-of-your-cfs.56908/#post-945253|title=Have you ruled out Chiari as a cause of your CFS|work=Phoenix Rising ME / CFS Forums|access-date=2018-10-26|language=en-US}}</ref>

As the brainstem is central to regulating the autonomic nervous system, it is perhaps unsurprising that dysautonomia (such as POTS) can be corrected by correcting brainstem compression.
==Diagnosis==
Brainstem compression is diagnosed via dynamic imaging, such as flexion-extension MRIs and rotational CT scans. The most common measurements used to diagnose and quantify brainstem compression are the Clivo-Axial Angle, the Grabb-Oakes Measurement, and the Basion-Dens Interval.<ref name=":1" />

==Treatment==
A cervical collar can be tried to see if it helps alleviate the neurological symptoms (even if only partially). However, a cervical collar is not an actual treatment for CCI. The standard treatment to correct brainstem compression is a craniocervical fusion surgery,<ref name=":1" /> performed by a CCI-literate neurosurgeon.
==See also==
* Craniocervical instability
* Cervical stenosis
* Autonomic control of energy metabolism

==References==
<references />

Brainstem

2018-10-26T23:17:31Z

Jeff:I created a page: Brainstem Compression

Brainstem Compression often occurs as a result of craniocervical instability (CCI).<ref name=":0">{{Cite journal|last=Henderson|first=Fraser C.|last2=Henderson|first2=Fraser C.|last3=Wilson|first3=William A.|last4=Mark|first4=Alexander S.|last5=Koby|first5=Myles|date=2017-03-03|title=Utility of the clivo-axial angle in assessing brainstem deformity: pilot study and literature review|url=https://link.springer.com/article/10.1007/s10143-017-0830-3|journal=Neurosurgical Review|language=en|volume=41|issue=1|pages=149–163|doi=10.1007/s10143-017-0830-3|issn=0344-5607|pmc=PMC5748419|pmid=28258417}}</ref><ref name=":1">{{Cite journal|last=Henderson|first=Fraser C.|last2=Wilson|first2=William A.|last3=Mott|first3=Stephen|last4=Mark|first4=Alexander|last5=Schmidt|first5=Kristi|last6=Berry|first6=Joel K.|last7=Vaccaro|first7=Alexander|last8=Benzel|first8=Edward|date=2010-07-16|title=Deformative stress associated with an abnormal clivo-axial angle: A finite element analysis|url=https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2940090/|journal=Surgical Neurology International|volume=1|doi=10.4103/2152-7806.66461|issn=2152-7806|pmc=PMC2940090|pmid=20847911}}</ref>
==Evidence==
Recent peer-reviewed academic research indicates that mechanical brainstem compression can be directly responsible for dysautonomia and fatigue.<ref name=":0" /><ref name=":1" /> When patients had their brainstem compression surgically corrected with a craniocervical fusion, their symptoms of brainstem compression resolved.<ref name=":1" />

There are also anecdotal accounts of POTS and fatigue going away after a craniocervical fusion to correct the brainstem compression.<ref>{{Cite news|url=https://forums.phoenixrising.me/index.php?threads/have-you-ruled-out-chiari-as-a-cause-of-your-cfs.56908/#post-945253|title=Have you ruled out Chiari as a cause of your CFS|work=Phoenix Rising ME / CFS Forums|access-date=2018-10-26|language=en-US}}</ref>

As the brainstem is central to regulating the autonomic nervous system, it is perhaps unsurprising that dysautonomia (such as POTS) can be corrected by correcting brainstem compression.
==Diagnosis==
Brainstem compression is diagnosed via dynamic imaging, such as flexion-extension MRIs and rotational CT scans. The most common measurements used to diagnose and quantify brainstem compression are the Clivo-Axial Angle, the Grabb-Oakes Measurement, and the Basion-Dens Interval.<ref name=":1" />

==Treatment==
A cervical collar can be tried to see if it helps alleviate the neurological symptoms (even if only partially). However, a cervical collar is not an actual treatment for CCI. The standard treatment to correct brainstem compression is a craniocervical fusion surgery,<ref name=":1" /> performed by a CCI-literate neurosurgeon.
==See also==
* Craniocervical instability
* Cervical stenosis
* Autonomic control of energy metabolism

==References==
<references />

Craniocervical instability

2018-10-24T20:37:05Z

Jeff:I discussed CCI in more detail and also linked this article to the "connective tissue" page.

'''Craniocervical instability''' (CCI), also known as the Syndrome of Occipitoatlantialaxial Hypermobility, is a structural instability of the craniocervical junction which may lead to deformation of the [[brainstem]], upper [[spinal cord]], and [[cerebellum]]. It is more common in patients with rheumatoid arthritis and [[Ehlers-Danlos Syndrome]] (itself a common co-morbidity with [[myalgic encephalomyelitis]]). CCI/AAI can occur from an injury, such as whiplash or a high-impact trauma. However, there are several known conditions where non-traumatic CCI/AAI can happen, seemingly spontaneously, for no obvious reason. This non-traumatic CCI/AAI happens with Down’s Syndrome, rheumatoid arthritis, Ehlers-Danlos Syndrome, and other inflammatory or connective tissue disorders. The ligaments of the craniocervical junction, which are made up of [[connective tissue]], simply become too lax.

There have been anecdotal reports of people with ME who meet the [[International Consensus Criteria]] who were ultimately diagnosed with CCI and/or [[Chiari malformation]], a related condition.<ref>{{Cite web|url=https://forums.phoenixrising.me/index.php?threads/have-you-ruled-out-chiari-as-a-cause-of-your-cfs.56908/|title=Have you ruled out Chiari as a cause of your CFS|last=|first=|date=|website=Phoenix Rising|archive-url=|archive-date=|dead-url=|access-date=}}</ref>

== Symptoms ==
Symptoms<ref>{{Cite web|url=http://thezebranetwork.org/craniocervical-instability/|title=Craniocervical Instability|last=|first=|date=|website=The Zebra Network|archive-url=|archive-date=|dead-url=|access-date=}}</ref> of craniocervical instability include:

a heavy headache: a constant to near constant headache that can be described as feeling like the head is too heavy for the neck to support (feeling like a “bobble-head”)

a pressure headache: an impairment of CSF flow causes intracranial pressure which would be aggravated by “valsalva maneuvers” such as yawning, laughing, crying, coughing, sneezing or straining.

Dysautonomia: brainstem compression can lead to a dysfunctional autonomic nervous system (the involuntary regulator of all body functions). Symptoms of this include, but are not limited to:

* [[tachycardia]] (rapid heart)
* [[heat intolerance]]
* [[orthostatic intolerance]] (low blood pressure when standing)
* syncope (fainting)
* [[polydipsia]] (extreme thirst)
* [[delayed gastric emptying]]
* [[chronic fatigue]]

Other symptoms include:

* [[neck pain]]
* central or mixed sleep [[apnea]]
* facial pain or [[numbness]]
* [[balance problems]]
* [[muscle weakness]]
* [[dizziness]] and [[vertigo]]
* [[vision]] problems
* reduced gag reflux and difficulty swallowing
* ringing in the ears and hearing loss
* [[nausea]] and [[vomiting]]
* impaired [[coordination]]
* downward [[nystagmus]] (irregular eye movements)
* [[paralysis]]

== Risk factors ==
Patients with [[Ehlers-Danlos syndrome|Ehlers-Danlos Syndrome]] are at greater risk.

== See also ==

* [[Ehlers-Danlos syndrome]]
* [[Chiari malformation]]

== Learn more ==

== References ==
<references />
[[Category:Diagnoses]]

Talk:Collagen

2018-10-18T08:37:14Z

Jeff:

Rheumatological presentation of Bartonella koehlerae and Bartonella henselae bacteremias: A case report
https://journals.lww.com/md-journal/Pages/articleviewer.aspx?year=2018&issue=04270&article=00032&type=Fulltext

Cross reaction of antibodies to a glycine/alanine repeat sequence of Epstein-Barr virus nuclear antigen-1 with collagen, cytokeratin, and actin.
https://ard.bmj.com/content/annrheumdis/50/11/772.full.pdf

Herpes-simplex virus encephalitis is characterized by an early MMP-9 increase and collagen type IV degradation
https://www.sciencedirect.com/science/article/pii/S0006899306029246