Craniocervical instability

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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 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 (EDS) (itself a common co-morbidity with 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.[1][2][3][4] 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[edit | edit source]

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

Symptoms[edit | edit source]

There can be a wide range of clinical presentations. Symptoms[5] of craniocervical instability and atlantoaxial instability, particularly in the context of a connective tissue disorder, can and may include:

Treatment[edit | edit source]

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.[7] 25% of patients later required fusion at another level.[7] 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.[19]

Risk factors[edit | edit source]

Patients with Ehlers-Danlos syndrome, Down Syndrome, dwarfism and Rheumatoid arthritis are at greater risk.

Co-morbid conditions[edit | edit source]

CCI can co-occur in patients with EDS and other connective tissue disorders with intracranial hypertension[7] or hypotension[7], abnormalities of intracranial venous drainage due to sinus stenosis or jugular vein stenosis,[7] Migraine headaches, temporomandibular joint dysfunction[7][12], and scoliosis.[12]

See also[edit | edit source]

Learn more[edit | edit source]

References[edit | edit source]

  1. "Have you ruled out Chiari as a cause of your CFS". Phoenix Rising. Cite has empty unknown parameter: |dead-url= (help)
  2. Johnson, Cort (May 21, 2019). "Jennifer Brea's Amazing ME/CFS Recovering Story: the Spinal Series - Pt. II". Health Rising. Retrieved June 1, 2019.
  3. rsci.app.link https://rsci.app.link/3LpJOxcTaX?_p=f3542c5bfc29c2616780177b27. Retrieved June 1, 2019. Missing or empty |title= (help)
  4. "Craniocervical instability, Atlantoaxial Instability, Myalgic Encephalomyelitis, ME, CFS". MEchanical Basis. Retrieved June 1, 2019.
  5. "Craniocervical Instability". The Zebra Network. Cite has empty unknown parameter: |dead-url= (help)
  6. 6.0 6.1 6.2 6.3 6.4 6.5 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. 7.00 7.01 7.02 7.03 7.04 7.05 7.06 7.07 7.08 7.09 7.10 7.11 7.12 7.13 7.14 7.15 7.16 Henderson, Fraser C.; Francomano, C. A.; Koby, M.; Tuchman, K.; Adcock, J.; Patel, S. (January 9, 2019). "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". Neurosurgical Review. doi:10.1007/s10143-018-01070-4. ISSN 1437-2320.
  8. 8.0 8.1 8.2 8.3 Rebbeck, Trudy; Liebert, Ann (December 1, 2014). "Clinical management of cranio-vertebral instability after whiplash, when guidelines should be adapted: A case report". Manual Therapy. 19 (6): 618–621. doi:10.1016/j.math.2014.01.009. ISSN 1356-689X.
  9. 9.0 9.1 9.2 9.3 9.4 9.5 9.6 "MRI video diagnosis and surgical therapy of soft tissue trauma to the craniocervical junction - ProQuest". search.proquest.com. Retrieved June 1, 2019.
  10. 10.0 10.1 10.2 10.3 10.4 Mathers, K. Sean; Schneider, Michael; Timko, Michael (2011-06). "Occult Hypermobility of the Craniocervical Junction: A Case Report and Review". Journal of Orthopaedic & Sports Physical Therapy. 41 (6): 444–457. doi:10.2519/jospt.2011.3305. ISSN 0190-6011. Check date values in: |date= (help)
  11. 11.0 11.1 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.
  12. 12.00 12.01 12.02 12.03 12.04 12.05 12.06 12.07 12.08 12.09 12.10 Francomano, Clair A.; McDonnell, Nazli B.; Nishikawa, Misao; Bolognese, Paolo A.; Milhorat, Thomas H. (December 1, 2007). "Syndrome of occipitoatlantoaxial hypermobility, cranial settling, and Chiari malformation Type I in patients with hereditary disorders of connective tissue". Journal of Neurosurgery: Spine. 7 (6): 601–609. doi:10.3171/SPI-07/12/601.
  13. Henderson, Fraser C.; Henderson, Fraser C.; Wilson, William A.; Mark, Alexander S.; Koby, Myles (January 1, 2018). "Utility of the clivo-axial angle in assessing brainstem deformity: pilot study and literature review". Neurosurgical Review. 41 (1): 149–163. doi:10.1007/s10143-017-0830-3. ISSN 1437-2320. PMC 5748419. PMID 28258417.
  14. Lua error in Module:Citation/CS1/Identifiers at line 1131: attempt to index local 'id' (a nil value).
  15. 15.0 15.1 15.2 15.3 15.4 15.5 15.6 15.7 Bergholm, Ulla; Johansson, Bengt H.; Johansson, Hakan (January 1, 2004). "New Diagnostic Tools Can Contribute to Better Treatment of Patients with Chronic Whiplash Disorders". Journal of Whiplash & Related Disorders. 3 (2): 5–19. doi:10.3109/J180v03n02_02. ISSN 1533-2888.
  16. Ghanem, Ismat; El Hage, Samer; Rachkidi, Rami; Kharrat, Khalil; Dagher, Fernand; Kreichati, Gabi (March 1, 2008). "Pediatric cervical spine instability". Journal of Children's Orthopaedics. 2 (2): 71–84. doi:10.1007/s11832-008-0092-2. ISSN 1863-2521. PMC 2656787. PMID 19308585.
  17. Montazem, Abbas (2000). "Secondary tinnitus as a symptom of instability in the upper cervical spine: Operative management" (PDF). International Tinnitus Journal.
  18. Janjua, M. Burhan; Hwang, Steven W.; Samdani, Amer F.; Pahys, Joshua M.; Baaj, Ali A.; Härtl, Roger; Greenfield, Jeffrey P. (January 1, 2019). "Instrumented arthrodesis for non-traumatic craniocervical instability in very young children". Child's Nervous System. 35 (1): 97–106. doi:10.1007/s00381-018-3876-9. ISSN 1433-0350.
  19. Wang, Chao; Passias, Peter G.; Wang, Shenglin; Zhao, Deng (May 1, 2015). "Craniocervical Instability in the Setting of Os OdontoideumAssessment of Cause, Presentation, and Surgical Outcomes in a Series of 279 Cases". Neurosurgery. 76 (5): 514–521. doi:10.1227/NEU.0000000000000668. ISSN 0148-396X.