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Atlantoaxial instability
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'''Atlantoaxial instability''' or '''Atlanto-axial instability''' '''(AAI)''' is characterized by excessive movement at the junction between the atlas (C1) and axis (C2) as a result of either a bone or ligament abnormality.<ref name="emedicine" /><ref name="Lacy2019" /> ==Causes == Atlantoaxial instability can be caused by hereditary conditions including Marfan Syndrome, neurofibromatosis, connective tissue conditions like [[rheumatoid arthritis]],or [[Ehlers-Danlos Syndrome]]s (EDS), as a result of physical trauma, or infection.<ref name="emedicine"/><ref name="Henderson2020">https://link.springer.com/article/10.1007/s10143-020-01345-9</ref> It has been associated with Down syndrome, Morquio syndrome,<ref name="Li2013">{{Cite journal | last = Li | first = Ming-Feng | last2 = Chiu | first2 = Pao-Chin | last3 = Weng | first3 = Mei-Jui | last4 = Lai | first4 = Ping-Hong | date = 2010-12-13 | title = Atlantoaxial Instability and Cervical Cord Compression in Morquio Syndrome | url =https://jamanetwork.com/journals/jamaneurology/fullarticle/801729 | journal = Archives of Neurology|language=en | volume = 67 | issue = 12 | pages = 1530–1530|doi=10.1001/archneurol.2010.308|issn=0003-9942}}</ref> [[Marfan syndrome]],<ref name=":3">{{Cite journal | last = MacKenzie | first = James MacKintosh | last2 = Rankin | first2 = Rosslyn | date = Dec 2003 | title = Sudden Death Due to Atlantoaxial Subluxation in Marfan Syndrome | url =https://journals.lww.com/amjforensicmedicine/Abstract/2003/12000/Sudden_Death_Due_to_Atlantoaxial_Subluxation_in.12.aspx | journal = The American Journal of Forensic Medicine and Pathology|language=en-US | volume = 24 | issue = 4 | pages = 369|doi=10.1097/01.paf.0000097853.26115.bb|issn=0195-7910|pmc=|pmid=|quote=|access-date=|via=}}</ref> and [[Ehlers-Danlos syndrome]].<ref>{{Cite web | url = https://europepmc.org/article/med/8596160 | title = Europe PMC | website = europepmc.org | access-date = 2019-12-10}}</ref><ref name="Henderson2017">{{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>{{Cite journal | last = Castori | first = Marco | last2 = Morlino | first2 = Silvia | last3 = Ghibellini | first3 = Giulia | last4 = Celletti | first4 = Claudia | last5 = Camerota | first5 = Filippo | last6 = Grammatico | first6 = Paola | date = 2015 | title=Connective tissue, Ehlers–Danlos syndrome(s), and head and cervical pain | url =https://onlinelibrary.wiley.com/doi/abs/10.1002/ajmg.c.31426 | journal = American Journal of Medical Genetics Part C: Seminars in Medical Genetics|language=en | volume = 169 | issue = 1 | pages = 84–96|doi=10.1002/ajmg.c.31426|issn=1552-4876}}</ref><ref>{{Cite journal | last = Lane | first = D. | date = 2006-08-01 | title = Anaesthetic Implications of Vascular Type Ehlers-Danlos Syndrome | url =https://doi.org/10.1177/0310057X0603400412 | journal = Anaesthesia and Intensive Care|language=en | volume = 34 | issue = 4 | pages = 501–505|doi=10.1177/0310057X0603400412|issn=0310-057X}}</ref><ref>{{Cite journal | last = Dordoni | first = Chiara | last2 = Ciaccio | first2 = Claudia | last3 = Venturini | first3 = Marina | last4 = Calzavara‐Pinton | first4 = Piergiacomo | last5 = Ritelli | first5 = Marco | last6 = Colombi | first6 = Marina | date = 2016 | title=Further delineation of FKBP14-related Ehlers–Danlos syndrome: A patient with early vascular complications and non-progressive kyphoscoliosis, and literature review | url =https://www.onlinelibrary.wiley.com/doi/abs/10.1002/ajmg.a.37728 | journal = American Journal of Medical Genetics Part A|language=en | volume = 170 | issue = 8 | pages = 2031–2038|doi=10.1002/ajmg.a.37728|issn=1552-4833}}</ref><ref>{{Cite journal | last = Giunta | first = Cecilia | last2 = Baumann | first2 = Matthias | last3 = Fauth | first3 = Christine | last4 = Lindert | first4 = Uschi | last5 = Abdalla | first5 = Ebtesam M. | last6 = Brady | first6 = Angela F. | last7 = Collins | first7 = James | last8 = Dastgir | first8 = Jahannaz | last9 = Donkervoort | first9 = Sandra | date = Jan 2018 | title = A cohort of 17 patients with kyphoscoliotic Ehlers–Danlos syndrome caused by biallelic mutations in FKBP14 : expansion of the clinical and mutational spectrum and description of the natural history | url = https://www.nature.com/articles/gim201770 | journal = Genetics in Medicine|language=en | volume = 20 | issue = 1 | pages = 42–54|doi=10.1038/gim.2017.70|issn=1530-0366|pmc=|pmid=|quote=|access-date=|via=}}</ref><ref>{{Cite web | url = https://europepmc.org/article/med/2387821 | title = Europe PMC | website = europepmc.org | access-date = 2019-12-10}}</ref><ref>{{Cite journal | last = Galan | first = Enrique | last2 = Kousseff | first2 = Boris G. | date = 1995-04-01 | title = Peripheral neuropathy in Ehlers-Danlos syndrome | url =http://www.sciencedirect.com/science/article/pii/088789949500003X | journal = Pediatric Neurology | volume = 12 | issue = 3 | pages = 242–245|doi=10.1016/0887-8994(95)00003-X|issn=0887-8994}}</ref><ref>{{Cite journal | last = Wills | first = Brian P.D. | last2 = Dormans | first2 = John P. | date = Apr 2006 | title = Nontraumatic Upper Cervical Spine Instability in Children | url =https://journals.lww.com/jaaos/Citation/2006/04000/Nontraumatic_Upper_Cervical_Spine_Instability_in.5.aspx | journal = JAAOS - Journal of the American Academy of Orthopaedic Surgeons|language=en-US | volume = 14 | issue = 4 | pages = 233|issn=1067-151X| doi = | pmc = | pmid = | quote = |access-date=|via=}}</ref><ref>{{Cite journal | last = Herzka | first = Andrea | last2 = Sponseller | first2 = Paul D. | last3 = Pyeritz | first3 = Reed E. | date = 2000-02-15 | title = Atlantoaxial Rotatory Subluxation in Patients With Marfan Syndrome: A Report of Three Cases |url =https://journals.lww.com/spinejournal/Abstract/2000/02150/Atlantoaxial_Rotatory_Subluxation_in_Patients_With.22.aspx | journal = Spine|language=en-US | volume = 25 | issue = 4 | pages = 524|issn=0362-2436}}</ref> AAI often co-occurs with [[craniocervical instability]] (CCI). In individuals without predisposing conditions, it is thought to be extremely rare.<ref name="Lacy2019">{{Cite book | last = Lacy | first = Jordan | last2 = Gillis | first2 = Christopher C. | date = 2019 | title=Atlantoaxial Instability | url = http://www.ncbi.nlm.nih.gov/books/NBK519563/|location=Treasure Island (FL)| publisher = StatPearls Publishing|pmid=30137847}}</ref> == Symptoms == * Neck pain<ref name="Lacy2019" /> * Neck stiffness, torticollis<ref name="Cohen1998">{{Cite journal | last = Cohen | first = William I. | date = 1998-02-01 | title = Atlantoaxial Instability: What's Next? | url = https://jamanetwork.com/journals/jamapediatrics/fullarticle/189216 | journal = Archives of Pediatrics & Adolescent Medicine|language=en | volume = 152 | issue = 2 | pages = 119–122|doi=10.1001/archpedi.152.2.119|issn=1072-4710}}</ref> * Spasticity<ref name="Lacy2019" /> * Radicular symptoms<ref name="Lacy2019" /> * Lack of coordination<ref name="Lacy2019" /> * Clumsiness<ref name="Lacy2019" /> * Gait changes, difficulty with gait<ref name="Lacy2019" /><ref name="Cohen1998" /> * Sensory deficits<ref name="Lacy2019" /> * Neurogenic bladder<ref name="Lacy2019" /> * Clonus, hyperreflexia<ref name="Lacy2019" /><ref name="Cohen1998" /> * Paraplegia, quadriplegia<ref name="Lacy2019" /> * Muscle weakness, which is not a constant feature<ref name="Henderson2017" /> * Blurred vision<ref name=":3" /> * Occipital headache<ref name=":3" /> Symptoms can often be exacerbated by rotation of the neck. == Risk factors and causes == === Congenital causes === === Rheumatoid arthritis === Chronic inflammation can cause laxity and stretching of the transverse ligament, the formation of a pannus, as well as bone erosion.<ref name="Lacy2019" /> === Grisel’s syndrome === Grisel’s syndrome is AAI that occurs following inflammation of the soft tissue as a consequence of surgery or infection,<ref name="emedicine" /> frequently an upper respiratory infection. It is primarily seen in patients ages 5-12 but can also be seen in adults.<ref name="emedicine">{{Cite web | date = 2019-11-10 | title = Atlantoaxial Instability: Background, Pathophysiology, Etiology | url = https://emedicine.medscape.com/article/1265682-overview#a3}}</ref> It usually presents with torticollis, neck pain, neck tilt, and stiffness.<ref name="Fernandez">{{Cite journal | last = Fernández Cornejo | first = Víctor J. | last2 = Martínez-Lage | first2 = Juan F. | last3 = Piqueras | first3 = Claudio | last4 = Gelabert | first4 = Amparo | last5 = Poza | first5 = Máximo | date = 2003-06-01 | title = Inflammatory atlanto-axial subluxation (Grisel's syndrome) in children: clinical diagnosis and management | url = https://doi.org/10.1007/s00381-003-0749-6 | journal = Child's Nervous System|language=en | volume = 19 | issue = 5 | pages = 342–347|doi=10.1007/s00381-003-0749-6|issn=1433-0350}}</ref> It can often be treated with conservative therapies such as physical therapy, traction, immobilization, anti-inflammatories and treatment of any underlying infection.<ref name="Fernandez" /> A handful of cases of concomitant non-traumatic CCI and AAI following upper respiratory infections have also been documented.<ref name="Washington1998">{{Cite journal | title = Nontraumatic Atlanto-occipital and Atlantoaxial Rotatory Subluxation: Case Report | url = https://academic.oup.com/neurosurgery/article/43/1/162/2856810 | journal = Neurosurgery | date = 1998-07-01|issn=0148-396X | pages = 162–164 | volume = 43 | issue = 1|doi=10.1097/00006123-199807000-00110|language=en | first = Shehan | last = Hettiaratchy | first2 = Chou | last2 = Ning | first3 = Ian | last3 = Sabin}}</ref><ref>{{Cite journal | title = Non-Traumatic Atlanto-Occipital and Atlanto-Axial Dislocation: A Case Report | url = https://journals.lww.com/jbjsjournal/Citation/1959/41020/Non_Traumatic_Atlanto_Occipital_and_Atlanto_Axial.15.aspx | journal = JBJS | date = Mar 1959|issn=0021-9355 | pages = 341–344 | volume = 41 | issue = 2|language=en-US| first = Eleby R. | last = Washington | last2 = | first2 = | doi = | pmc = | pmid = | quote = |access-date=|via=}}</ref> == Co-morbid conditions == AAI can cause vertebrobasilar insufficiency.<ref name="Vinchon">{{Cite journal | last = Vinchon | first = Matthieu | last2 = Assaker | first2 = Richard | last3 = Leclerc | first3 = Xavier | last4 = Lejeune | first4 = Jean-Paul | date = 1995-04-01 | title = Vertebrobasilar Insufficiency Resulting from Traumatic Atlantoaxial InstabilityCase Report | url = https://academic.oup.com/neurosurgery/article/36/4/839/2753933 | journal = Neurosurgery|language=en | volume = 36 | issue = 4 | pages = 839–841|doi=10.1227/00006123-199504000-00027|issn=0148-396X}}</ref> == Diagnosis == AAI diagnosis is based on a neurological exam, reported symptoms, and radiological measurements, generally a CTSCAN with rotation. An atlantodental (or atlantodens or atlas-dens) interval (ADI) of greater than 3 mm in adults and of greater than 5 mm in children as measured on plain radiography is considered indicative of AAI. The ADI is the distance between the odontoid process and the posterior border of the anterior arch of the atlas. An abnormal degreeof rotation of the atlas (C1) on C2 has also been determined to indicate instability. == Treatment== == See also == * [[Craniocervical instability]] == Learn more == == References == {{Reflist}} [[Category:Diagnoses]]
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