Craniocervical instability

Craniocervical instability is a medical condition where there is excessive laxity of the ligaments at the atlanto-occipital joint and the atlanto-axial joint, between the skull and the top two vertebrae (C1 and C2). This results in excessive movement of the vertebrae which can cause neuronal injury and compression of nearby structures including the spinal cord, 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 is often brought on by a trauma, frequently whiplash.

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
  • 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.

Diagnosis

Craniocervical instability is usually diagnosed through neuro-anatomical measurement using radiography. Upright magnetic resonance imaging is considered the most accurate method, and supine magnetic resonance imaging, CT scan or digital motion X-ray, or Digital X-ray are also used.

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 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 [10] but there is limited scientific evidence to date.

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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. 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.
  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.
  8. Smith FW, Dworkin JS (eds): The Craniocervical Syndrome and MRI. Basel, Karger, 2015, pp 9-21 (DOI:10.1159/000365467)
  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. 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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