Atlantoaxial instability: Difference between revisions

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Atlantoaxial 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.
Atlantoaxial 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.


It can be caused by congenital conditions, inflammatory conditions like [[rheumatoid arthritis]], as a result of physical trauma, or infection.<ref>{{Cite journal|date=2019-11-10|title=Atlantoaxial Instability: Background, Pathophysiology, Etiology|url=https://emedicine.medscape.com/article/1265682-overview}}</ref> It has been associated with Down’s Syndrome, Morquio's syndrome<ref>{{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>{{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><ref name=":3">{{Cite journal|last=MacKenzie|first=James MacKintosh|last2=Rankin|first2=Rosslyn|date=2003-12|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}}</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>{{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=2018-01|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}}</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=2006-04|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}}</ref>
It can be caused by congenital conditions, inflammatory conditions like [[rheumatoid arthritis]], as a result of physical trauma, or infection.<ref>{{Cite journal|date=2019-11-10|title=Atlantoaxial Instability: Background, Pathophysiology, Etiology|url=https://emedicine.medscape.com/article/1265682-overview}}</ref> It has been associated with Down’s Syndrome, Morquio's syndrome,<ref>{{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>{{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><ref name=":3">{{Cite journal|last=MacKenzie|first=James MacKintosh|last2=Rankin|first2=Rosslyn|date=2003-12|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}}</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>{{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=2018-01|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}}</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=2006-04|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}}</ref>


It often co-occurs with [[craniocervical instability]] (CCI).  
It often co-occurs with [[craniocervical instability]] (CCI).  
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== See also ==
== See also ==
* [[Craniocervical instability]]
* [[Craniocervical instability]]
Learn more
 
== Learn more ==


== References ==
== References ==
<references />
<references />

Revision as of 06:07, December 10, 2019

Atlantoaxial 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.

It can be caused by congenital conditions, inflammatory conditions like rheumatoid arthritis, as a result of physical trauma, or infection.[1] It has been associated with Down’s Syndrome, Morquio's syndrome,[2] Marfan syndrome,[3][4] and Ehlers-Danlos syndrome.[5][6][7][8][9][10][11][12][13]

It often co-occurs with craniocervical instability (CCI).

In individuals without predisposing conditions, it is thought to be extremely rare.[14]

Symptoms[edit | edit source]

  • Neck pain[14]
  • Neck stiffness, torticollis[15]
  • Myelopathy[14]
  • Spasticity[14]
  • Radius are symptoms[14]
  • Lack of coordination[14]
  • Clumsiness[14]
  • Gait changes, difficulty with gait[14][15]
  • Sensory deficits[14]
  • Neurogenic bladder[14]
  • Spasticity, clonus, hyperreflexia[14][15]
  • Paraplegia, quadriplegia[14]
  • Muscle weakness[16]
  • Decreased hand dexterity[16]
  • Blurred vision[4]
  • Occipital headache[4]

Risk factors and causes[edit | edit source]

Congenital causes[edit | edit source]

Rheumatoid arthritis[edit | edit source]

Chronic inflammation can cause laxity and stretching of the transverse ligament, the formation of a pannus, as well as bone erosion.[14]

Grisel’s syndrome[edit | edit source]

Grisel’s syndrome occurs following inflammation of the soft tissue as a consequence of surgery or infection,[17] frequently an upper respiratory infection. It is primarily seen in patients ages 5-12 but can also be seen in adults.[17] It usually presents with torticollis, neck pain, neck tilt, and stiffness.[18] It can often be treated with conservative therapies such as physical therapy, traction, immobilization, antiinflammatories and treatment of any underlying infection.[18]

Co-morbid conditions[edit | edit source]

AAI can cause vertebrobasilar insufficiency.[19]

Diagnosis[edit | edit source]

AAI is defined as 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. The ADI is the distance between the odontoid process and the posterior border of the anterior arch of the atlas.

Treatment[edit | edit source]

See also[edit | edit source]

Learn more[edit | edit source]

References[edit | edit source]

  1. "Atlantoaxial Instability: Background, Pathophysiology, Etiology". November 10, 2019. Cite journal requires |journal= (help)
  2. Li, Ming-Feng; Chiu, Pao-Chin; Weng, Mei-Jui; Lai, Ping-Hong (December 13, 2010). "Atlantoaxial Instability and Cervical Cord Compression in Morquio Syndrome". Archives of Neurology. 67 (12): 1530–1530. doi:10.1001/archneurol.2010.308. ISSN 0003-9942.
  3. Herzka, Andrea; Sponseller, Paul D.; Pyeritz, Reed E. (February 15, 2000). "Atlantoaxial Rotatory Subluxation in Patients With Marfan Syndrome: A Report of Three Cases". Spine. 25 (4): 524. ISSN 0362-2436.
  4. 4.0 4.1 4.2 MacKenzie, James MacKintosh; Rankin, Rosslyn (2003-12). "Sudden Death Due to Atlantoaxial Subluxation in Marfan Syndrome". The American Journal of Forensic Medicine and Pathology. 24 (4): 369. doi:10.1097/01.paf.0000097853.26115.bb. ISSN 0195-7910. Check date values in: |date= (help)
  5. "Europe PMC". europepmc.org. Retrieved December 10, 2019.
  6. Henderson, Fraser C.; Austin, Claudiu; Benzel, Edward; Bolognese, Paolo; Ellenbogen, Richard; Francomano, Clair A.; Ireton, Candace; Klinge, Petra; Koby, Myles (2017). "Neurological and spinal manifestations of the Ehlers–Danlos syndromes". American Journal of Medical Genetics Part C: Seminars in Medical Genetics. 175 (1): 195–211. doi:10.1002/ajmg.c.31549. ISSN 1552-4876.
  7. Castori, Marco; Morlino, Silvia; Ghibellini, Giulia; Celletti, Claudia; Camerota, Filippo; Grammatico, Paola (2015). "Connective tissue, Ehlers–Danlos syndrome(s), and head and cervical pain". American Journal of Medical Genetics Part C: Seminars in Medical Genetics. 169 (1): 84–96. doi:10.1002/ajmg.c.31426. ISSN 1552-4876.
  8. Lane, D. (August 1, 2006). "Anaesthetic Implications of Vascular Type Ehlers-Danlos Syndrome". Anaesthesia and Intensive Care. 34 (4): 501–505. doi:10.1177/0310057X0603400412. ISSN 0310-057X.
  9. Dordoni, Chiara; Ciaccio, Claudia; Venturini, Marina; Calzavara‐Pinton, Piergiacomo; Ritelli, Marco; Colombi, Marina (2016). "Further delineation of FKBP14-related Ehlers–Danlos syndrome: A patient with early vascular complications and non-progressive kyphoscoliosis, and literature review". American Journal of Medical Genetics Part A. 170 (8): 2031–2038. doi:10.1002/ajmg.a.37728. ISSN 1552-4833.
  10. Giunta, Cecilia; Baumann, Matthias; Fauth, Christine; Lindert, Uschi; Abdalla, Ebtesam M.; Brady, Angela F.; Collins, James; Dastgir, Jahannaz; Donkervoort, Sandra (2018-01). "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". Genetics in Medicine. 20 (1): 42–54. doi:10.1038/gim.2017.70. ISSN 1530-0366. Check date values in: |date= (help)
  11. "Europe PMC". europepmc.org. Retrieved December 10, 2019.
  12. Galan, Enrique; Kousseff, Boris G. (April 1, 1995). "Peripheral neuropathy in Ehlers-Danlos syndrome". Pediatric Neurology. 12 (3): 242–245. doi:10.1016/0887-8994(95)00003-X. ISSN 0887-8994.
  13. Wills, Brian P. D.; Dormans, John P. (2006-04). "Nontraumatic Upper Cervical Spine Instability in Children". JAAOS - Journal of the American Academy of Orthopaedic Surgeons. 14 (4): 233. ISSN 1067-151X. Check date values in: |date= (help)
  14. 14.00 14.01 14.02 14.03 14.04 14.05 14.06 14.07 14.08 14.09 14.10 14.11 14.12 Lacy, Jordan; Gillis, Christopher C. (2019). "Atlantoaxial Instability". Treasure Island (FL): StatPearls Publishing. PMID 30137847. Cite journal requires |journal= (help)
  15. 15.0 15.1 15.2 Cohen, William I. (February 1, 1998). "Atlantoaxial Instability: What's Next?". Archives of Pediatrics & Adolescent Medicine. 152 (2): 119–122. doi:10.1001/archpedi.152.2.119. ISSN 1072-4710.
  16. 16.0 16.1 "Atlantoaxial Instability - Spine - Orthobullets". www.orthobullets.com. Retrieved December 10, 2019.
  17. 17.0 17.1 "Atlantoaxial Instability: Background, Pathophysiology, Etiology". November 10, 2019. Cite journal requires |journal= (help)
  18. 18.0 18.1 Fernández Cornejo, Víctor J.; Martínez-Lage, Juan F.; Piqueras, Claudio; Gelabert, Amparo; Poza, Máximo (June 1, 2003). "Inflammatory atlanto-axial subluxation (Grisel's syndrome) in children: clinical diagnosis and management". Child's Nervous System. 19 (5): 342–347. doi:10.1007/s00381-003-0749-6. ISSN 1433-0350.
  19. Vinchon, Matthieu; Assaker, Richard; Leclerc, Xavier; Lejeune, Jean-Paul (April 1, 1995). "Vertebrobasilar Insufficiency Resulting from Traumatic Atlantoaxial InstabilityCase Report". Neurosurgery. 36 (4): 839–841. doi:10.1227/00006123-199504000-00027. ISSN 0148-396X.