Craniocervical instability explained

Craniocervical instability (CCI) is a medical condition characterized by excessive movement of the vertebra at the atlanto-occipital joint and the atlanto-axial joint located between the skull and the top two vertebra, known as C1 and C2. The condition can cause neural injury and compression of nearby structures, including the brain stem, spinal cord, vagus nerve, and vertebral artery, resulting in a constellation of symptoms.

Craniocervical instability is more common in people with a connective tissue disease, including Ehlers-Danlos syndromes,[1] osteogenesis imperfecta, and rheumatoid arthritis.[2] It is frequently co-morbid with atlanto-axial joint instability, Chiari malformation,[3] or tethered spinal cord syndrome.

The condition can be brought on by physical trauma, including whiplash, laxity of the ligaments surrounding the joint, or other damage to the surrounding connective tissue.

Symptoms and signs

The impact of craniocervical instability can range from minor symptoms to severe disability in which patients are bed-bound. The constellation of symptoms caused by craniocervical instability is known as "cervico-medullary syndrome"[4] and includes:[5] [6] [7]

Symptoms are frequently worsened by a Valsalva maneuver, or by being upright for long periods of time. The reason that being upright is problematic is that gravity allows increased interaction between the brain stem and the top of the spinal column, increasing symptoms.

Lying in the supine position can bring short-term relief. Lying supine eliminates the downward gravitational pull, reducing symptoms to some degree. Lying with the feet somewhat higher and head lower allows gravity can be helpful in symptom reduction.

Diagnosis

Craniocervical instability is usually diagnosed through neuro-anatomical measurement using radiography. Digital Motion X-ray is considered the most accurate method. Upright magnetic resonance imaging, supine magnetic resonance imaging, CT scan, and flexion and extension x-rays may also be used but are far less accurate and have a much higher potential for false negatives.

The measurements to diagnose craniocervical instability are:

Alternatively, craniocervical instability can be diagnosed if a trial of cervical traction, typically using a halo fixation device, results in a significant alleviation of symptoms.

Treatment

Conservative treatment of craniocervical instability includes physical therapy[10] [11] and the use of a cervical collar to keep the neck stable. Prolotherapy, including with stem cells, is a treatment option, but there is no scientific evidence supporting the success of this approach.[12]

Cervical spinal fusion is performed on patients with more severe symptoms.

See also

Notes and References

  1. 10.1002/ajmg.c.31549. 28220607. Neurological and spinal manifestations of the Ehlers-Danlos syndromes. American Journal of Medical Genetics Part C: Seminars in Medical Genetics. 175. 1. 195–211. 2017. Henderson. Fraser C.. Austin. Claudiu. Benzel. Edward. Bolognese. Paolo. Ellenbogen. Richard. Francomano. Clair A.. Ireton. Candace. Klinge. Petra. Petra Klinge. Koby. Myles. Long. Donlin. Patel. Sunil. Singman. Eric L.. Voermans. Nicol C.. free.
  2. 10.1136/ard.52.9.629. 8239756. Neuropathology of the brainstem and spinal cord in end stage rheumatoid arthritis: Implications for treatment. Annals of the Rheumatic Diseases. 52. 9. 629–637. 1993. Henderson. F. C.. Geddes. J. F.. Crockard. H. A.. 1005138. free.
  3. 10.2531/spinalsurg.23.168. Occipito-atlanto-axial Hypermobility : Clinical Features and Dynamic Analysis of Cranial Settling and Posterior Gliding of Occipital Condyle. Part 1 : Findings in Patients with Hereditary Disorders of Connective Tissue and Ehlers-Danlos Syndrome. Spinal Surgery. 23. 2. 168–175. 2009. Nishikawa. Misao. h. Milhorat. Thomas. a. Bolognese. Paolo. b. Mcdonnell. Nazli. a. Francomano. Clair. free.
  4. Batzdorf U, Henderson F, Rigamonti D 2015. "Consensus statement on Cervico-Medullary Syndrome." In Co-morbidities that complicate the treatment and outcomes of Chiari malformation. Ulrich Batzdorf.
  5. 10.1155/2015/794829. 26770824. 4681798. The Role of the Craniocervical Junction in Craniospinal Hydrodynamics and Neurodegenerative Conditions. Neurology Research International. 2015. 1–20. 2015. Flanagan. Michael F.. free .
  6. 10.1111/head.12417. 24958300. Joint Hypermobility and Headache: The Glue That Binds the Two Together - Part 2. Headache: The Journal of Head and Face Pain. 54. 8. 1403–1411. 2014. Martin. Vincent T.. Neilson. Derek. 40251988 .
  7. 10.1111/j.1468-2982.2006.01187.x. 16961783. Cervical Spine Joint Hypermobility: A Possible Predisposing Factor for New Daily Persistent Headache. Cephalalgia. 26. 10. 1182–1185. 2006. Rozen. TD. Roth. JM. Denenberg. N.. 25434393.
  8. Book: 10.1159/000365467 . The Cranial Cervical Syndrome Defined: New Hope for Postwhiplash Migraine Headache Patients - Cervical Digital Motion X-Ray, FONAR Upright® Weight-Bearing Multi-Position™ MRI and Minimally Invasive C1-C2 Transarticular Lag Screw Fixation Fusion . The Craniocervical Syndrome and MRI . 2015 . Franck . Joel Ira . Perrin . Pamela . 9–21 . 978-3-318-02696-2 .
  9. 10.1002/ajmg.c.31549. 28220607. Neurological and spinal manifestations of the Ehlers-Danlos syndromes. American Journal of Medical Genetics Part C: Seminars in Medical Genetics. 175. 1. 195–211. 2017. Henderson. Fraser C.. Austin. Claudiu. Benzel. Edward. Bolognese. Paolo. Ellenbogen. Richard. Francomano. Clair A.. Ireton. Candace. Klinge. Petra. Koby. Myles. Long. Donlin. Patel. Sunil. Singman. Eric L.. Voermans. Nicol C.. free.
  10. Chu . ECP . Cervicogenic Dizziness Associated With Craniocervical Instability: A Case Report . Journal of Medical Cases . November 2021 . 12 . 11 . 451–454 . 10.14740/jmc3792 . 34804305 . 8577610 .
  11. Chu . ECP . Craniocervical instability associated with rheumatoid arthritis: a case report and brief review . AME Case Reports . April 2021 . 5 . 12 . 12 . 10.21037/acr-20-131 . 33912801 . 8060151 . free .
  12. 10.2174/1874325001408010326 . free. 25328557. 4200875. Chronic Neck Pain: Making the Connection Between Capsular Ligament Laxity and Cervical Instability. The Open Orthopaedics Journal. 8. 326–345. 2014. Steilen. Danielle. Hauser. Ross. Woldin. Barbara. Sawyer. Sarah.

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