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Atlantoaxial instability
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==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>
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