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Chiari malformation
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== Co-morbidities == Common comorbidites of Chiari malformation include: * [[Hydrocephalus]]<ref name=":1" /> - an excessive buildup of cerebrospinal fluid in the brain. It can cause [[intracranial hypertension]]. * [[Spina bifida]]<ref name=":1" /> - an incomplete closing of the backbone and membranes around the spinal cord. * [[Syringomyelia]]<ref name=":1" /> -a disorder in which a cerebrospinal-filled cyst or syrinx forms within the spinal cord’s central canal. * [[Tethered cord syndrome]]<ref name=":1" /> * Spinal curvature<ref name=":1" /> - the spine either may bend to the left or right ([[scoliosis]]) or may bend forward ([[kyphosis]]). * [[Craniocervical instability]] === Ehlers-Danlos syndrome === === Misdiagnosis === Patients with Chiari I malformation may be misdiagnosed with [[migraine]], [[multiple sclerosis]], [[fibromyalgia]], [[chronic fatigue syndrome]] or [[mental illness]]es.<ref name="misdiagnosis">https://pubmed.ncbi.nlm.nih.gov/10232534/</ref><ref name="CFS-misdiagnosis">https://onlinelibrary.wiley.com/doi/abs/10.1111/j.1745-7599.2001.tb00239.x?sid=nlm%3Apubmed</ref> === Fibromyalgia === In the 1990s, there was a controversy surrounding whether Chiari malformation could cause the symptoms of fibromyalgia or chronic fatigue syndrome,<ref name="wsj">{{Cite news |url =https://www.wsj.com/articles/SB942280927444238502 | title = Some Doctors Operate on People Diagnosed With Chronic Fatigue | last = Burton | first = Thomas M. | website = Wall Street Journal|language=en-US|access-date=2019-07-07}}</ref><ref name="mass">{{Cite web | url = https://www.massmecfs.org/more-resources-for-differential-diagnosis/172-cfidsfm-and-chiari-malformation-surgery | title = CFS/FM and Chiari Malformation Surgery | website = Massachusetts ME/CFS & FM Association|access-date=2019-07-07}}</ref> and some patients had unnecessary and potentially harmful surgery for Chiari I malformation in the hope of curing or improving ME/CFS or fibromyalgia, when their Chiara I malformation may have been asymptomatic or unsuitable for surgery, or the malformation did not meet the Chiari malformation criteria.<ref name="mass" /><ref name="Bradley2001">https://doi.org/10.1002/1529-0131(199912)42:12%3C2731::AID-ANR36%3E3.0.CO;2-K</ref><br> There has also been discussion about whether Chiari I malformation was more or less common in fibromyalgia patients. A 2004 study of a non-random sample of 270 patients with [[fibromyalgia]] diagnoses found that 46% had [[cervical spinal stenosis]] and 20% chiari malformation.<ref>{{Cite journal | last = Heffez|first = Dan S. | last2 = Ross | first2 = Ruth E. | last3 = Shade-Zeldow | first3 = Yvonne | last4 = Kostas | first4 = Konstantinos | last5 = Shah | first5 = Sagar | last6 = Gottschalk | first6 = Robert | last7 = Elias | first7 = Dean A. | last8 = Shepard | first8 = Alan | last9 = Leurgans | first9 = Sue E. | date = 2004-04-09 | title = Clinical evidence for cervical myelopathy due to Chiari malformation and spinal stenosis in a non-randomized group of patients with the diagnosis of fibromyalgia | url = https://link.springer.com/article/10.1007/s00586-004-0672-x | journal = European Spine Journal|language=en | volume = 13 | issue = 6 | pages = 516–523|doi=10.1007/s00586-004-0672-x|issn=0940-6719|pmc=3476600|pmid=15083352}}</ref> However, a 2011 paper which compared a random sample of fibromyalgia patients to a control group failed to find any increased rate of Chiari malformation among people with fibromyalgia.<ref>{{Cite journal | last = Ellenbogen | first = Richard G. | last2 = Guan | first2 = Qingyan | last3 = Noonan | first3 = Carolyn | last4 = Maravilla | first4 = Kenneth R. | last5 = Goldberg | first5 = Jack | last6 = Buchwald | first6 = Dedra | last7 = Watson | first7 = Nathaniel F. | date = 2011-02-01 | title = Is Chiari I Malformation Associated With Fibromyalgia? | url = https://academic.oup.com/neurosurgery/article/68/2/443/2606332 | journal = Neurosurgery|language=en | volume = 68 | issue = 2 | pages = 443–449|doi=10.1227/NEU.0b013e3182039a31|issn=0148-396X}}</ref> Chiari malformation is associated with [[Ehlers-Danlos syndrome]],<ref>{{Cite journal | last = Castori|first = Marco | last2 = Camerota | first2 = Filippo | last3 = Celletti | first3 = Claudia | last4 = Danese | first4 = Chiara | last5 = Santilli | first5 = Valter | last6 = Saraceni | first6 = Vincenzo Maria | last7 = Grammatico | first7 = Paola | date = 2010 | title=Natural history and manifestations of the hypermobility type Ehlers–Danlos syndrome: A pilot study on 21 patients |url =https://onlinelibrary.wiley.com/doi/abs/10.1002/ajmg.a.33231 | journal = American Journal of Medical Genetics Part A|language=en | volume = 152A | issue = 3 | pages = 556–564|doi=10.1002/ajmg.a.33231|issn=1552-4833}}</ref><ref>{{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>{{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> which is sometimes misdiagnosed as fibromyalgia, or with which there may be diagnostic overlap.<ref>{{Cite journal | last = Rombaut | first = Lies | last2 = Malfait | first2 = Fransiska | last3 = Paepe | first3 = Anne De | last4 = Rimbaut | first4 = Steven | last5 = Verbruggen | first5 = Gust | last6 = Wandele | first6 = Inge De | last7 = Calders | first7 = Patrick | date = 2011 | title = Impairment and impact of pain in female patients with Ehlers-Danlos syndrome: A comparative study with fibromyalgia and rheumatoid arthritis |url =https://onlinelibrary.wiley.com/doi/abs/10.1002/art.30337 | journal = Arthritis & Rheumatism|language=en | volume = 63 | issue = 7 | pages = 1979–1987|doi=10.1002/art.30337|issn=1529-0131}}</ref><ref>{{Cite journal | last = Miller | first = Victor J. | last2 = Zeltser | first2 = Rephael | last3 = Yoeli | first3 = Zvi | last4 = Bodner | first4 = Lipa | date = 1997-07-01 | title = Ehlers-Danlos Syndrome, Fibromyalgia and Temporomandibular Disorder: Report of an Unusual Combination | url =https://doi.org/10.1080/08869634.1997.11746020 | journal = CRANIO® | volume = 15 | issue = 3 | pages = 267–269|doi=10.1080/08869634.1997.11746020|issn=0886-9634}}</ref> In 2018, an American chart review of patients undergoing surgery for Chiari I malformation found that 14% had fibromyalgia and 14% had chronic fatigue syndrome, although it did not report how many patients had both fibromyalgia and CFS.<ref name=":4">{{Cite journal | title = A morphometric assessment of type I Chiari malformation above the McRae line: A retrospective case-control study in 302 adult female subjects | date = 2018-02-01 | url = https://www.researchgate.net/profile/Soroush-Heidari-Pahlavian/publication/318239474_A_Morphometric_Assessment_of_type_I_Chiari_Malformation_above_the_McRae_line_A_Retrospective_Case-Control_Study_in_302_Adult_Female_Subjects/links/5a5254f30f7e9bbc10549cbd/A-Morphometric-Assessment-of-type-I-Chiari-Malformation-above-the-McRae-line-A-Retrospective-Case-Control-Study-in-302-Adult-Female-Subjects.pdf | journal=Journal of Neuroradiology | volume = 45 | issue = 1 | pages = 23–31 | last = Houston | first = James R. | authorlink = | last2 = Eppelheimer | first2 = Maggie S. | author-link2 = | last3 = Pahlavian | first3 = Soroush Heidari | author-link3 = | last4 = Biswas | first4 = Dipankar | author-link4 = | last5 = Urbizu | first5 = Aintzane | author-link5 = | last6 = Martin | first6 = Bryn A. | author-link6 = | last7 = Bapuraj | first7 = Jayapalli Rajiv | last8 = Luciano | first8 = Mark | last9 = Allen | first9 = Philip A. | last10 = Loth | first10 = Francis|language=en|doi=10.1016/j.neurad.2017.06.006|pmc=|pmid=|access-date=|issn=0150-9861|quote=|via=}}</ref> === ME/CFS === A Swedish study of 234 ME/CFS patients meeting the [[Canadian Consensus Criteria]] found that a substantial proportion of patients had signs on their brain MRIs suggestive of [[intracranial hypertension]]: 55% had increased diameter of the optic nerve sheath. In addition 80% had cerebellar tonsillar descent that could potentially cause disruptions to spinal fluid flow, and thus increased intracranial pressure. 13.2% had tonsillar herniations severe enough to be considered a Chiari Malformation.<ref name="Bragee2020">{{Cite journal | title = Signs of Intracranial Hypertension, Hypermobility, and Craniocervical Obstructions in Patients With Myalgic Encephalomyelitis/Chronic Fatigue Syndrome | date = 2020 | url=https://www.frontiersin.org/article/10.3389/fneur.2020.00828 | journal = Frontiers in Neurology | volume = 11 | pages = 828 | last = Bragée | first = Björn | last2 = Michos | first2 = Anastasios | last3 = Drum | first3 = Brandon | last4 = Fahlgren | first4 = Mikael | last5 = Szulkin | first5 = Robert | last6 = Bertilson | first6 = Bo C.|doi=10.3389/fneur.2020.00828|issn=1664-2295}}</ref> In 2018, an American chart review of patients undergoing surgery for Chiari I malformation found that 14% had chronic fatigue syndrome.<ref name=":4" />
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