Craniocervical instability

Craniocervical instability is a medical condition where there is excessive movement of the vertebrae at the atlanto-occipital joint and the atlanto-axial joint, that is, between the skull and the top two vertebrae (C1 and C2). This can cause neuronal injury and compression of nearby structures including the spinal cord, brain stem, vertebral artery or vagus nerve, causing a constellation of symptoms. It is frequently co-morbid with atlanto-axial instability, Chiari malformation[1] and tethered cord syndrome.

It is more common in people with a connective tissue disease, notably Ehlers-Danlos Syndrome,[2] osteogenesis imperfecta and rheumatoid arthritis.[3] It can be brought on by a trauma, frequently 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, with some patients being bed-bound. The constellation of symptoms caused by craniocervical instability has been labelled the cervico-medullary syndrome.[4] Common symptoms include:[5][6][7]

  • Occipital headaches
  • Migraine Headaches [8]
  • neck, shoulder and jaw pain
  • difficulty swallowing, or the sensation of being choked
  • tenderness at base of skull
  • feeling of 'bobble-head', where the skull may 'fall off' the spine
  • photophobia
  • double or blurred vision
  • anxiety
  • tinnitus
  • tremors
  • orthostatic intolerance
  • vertigo or dizziness
  • palpitations
  • shortness of breath
  • nausea
  • fatigue
  • Lhermitte's sign
  • cognitive and memory decline
  • clumsiness and motor delay
  • fainting
  • weakness of the limbs

Symptoms are frequently worsened by a Valsalva maneuver or by being upright for long periods of time. Lying supine can bring short-term relief. The reason that being upright is problematic is that gravity is allowing increased interaction between the brain stem and the top of the spinal column, increasing symptoms. Lying supine eliminates the downward gravitational pull, reducing symptoms to some degree. Lying with the feet somewhat higher and head lower (Trendelenberg) allows gravity to work somewhat in the patient's favor.

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:

  • Clivo-Axial Angle equal or less than 135 degrees
  • Grabb-Oakes measurement equal or greater than 9 mm
  • Harris measurement greater than 12mm[9]
  • Spinal subluxation

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. Cervical spinal fusion is performed on patients with more severe symptoms. Prolotherapy, including with stem cells, is another treatment option used,[12] but there is limited scientific evidence on this approach.

See also
  • Hypermobility spectrum disorder

References
  1. Nishikawa, Misao; h. Milhorat, Thomas; a. Bolognese, Paolo; b. Mcdonnell, Nazli; a. Francomano, Clair (2009). "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. doi:10.2531/spinalsurg.23.168.
  2. 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. (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. PMID 28220607.
  3. Henderson, F. C.; Geddes, J. F.; Crockard, H. A. (1993). "Neuropathology of the brainstem and spinal cord in end stage rheumatoid arthritis: Implications for treatment". Annals of the Rheumatic Diseases. 52 (9): 629–637. doi:10.1136/ard.52.9.629. PMC 1005138. PMID 8239756.
  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. Flanagan, Michael F. (2015). "The Role of the Craniocervical Junction in Craniospinal Hydrodynamics and Neurodegenerative Conditions". Neurology Research International. 2015: 1–20. doi:10.1155/2015/794829. PMC 4681798. PMID 26770824.
  6. Martin, Vincent T.; Neilson, Derek (2014). "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. doi:10.1111/head.12417. PMID 24958300. S2CID 40251988.
  7. Rozen, TD; Roth, JM; Denenberg, N. (2006). "Cervical Spine Joint Hypermobility: A Possible Predisposing Factor for New Daily Persistent Headache". Cephalalgia. 26 (10): 1182–1185. doi:10.1111/j.1468-2982.2006.01187.x. PMID 16961783. S2CID 25434393.
  8. Franck, Joel Ira; Perrin, Pamela (2015). "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. pp. 9–21. doi:10.1159/000365467. ISBN 978-3-318-02696-2.
  9. 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. (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. PMID 28220607.
  10. Chu, ECP (November 2021). "Cervicogenic Dizziness Associated With Craniocervical Instability: A Case Report". Journal of Medical Cases. 12 (11): 451–454. doi:10.14740/jmc3792. PMC 8577610. PMID 34804305.
  11. Chu, ECP (April 2021). "Craniocervical instability associated with rheumatoid arthritis: a case report and brief review". AME Case Reports. 5 (12): 12. doi:10.21037/acr-20-131. PMC 8060151. PMID 33912801.
  12. Steilen, Danielle; Hauser, Ross; Woldin, Barbara; Sawyer, Sarah (2014). "Chronic Neck Pain: Making the Connection Between Capsular Ligament Laxity and Cervical Instability". The Open Orthopaedics Journal. 8: 326–345. doi:10.2174/1874325001408010326. PMC 4200875. PMID 25328557.
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