Craniocervical Instability

Craniocervical Instability (CCI), also known as the Syndrome of Occipitoatlantialaxial Hypermobility, is a structural instability of the craniocervical junction which may lead to apathological deformation of the brainstem, upper spinal cord, and cerebellum. It primarily occurs in patients with Ehlers-Danlos Syndrome and other hereditary disorders of connective tissue.

Symptoms of Craniocervical Instability: 

  • Other symptoms include:
    • neck pain
    • central or mixed sleep apnea
    • facial pain or numbness
    • balance problems
    • muscle weakness
    • dizziness and vertigo
    • vision problems
    • reduced gag reflux and difficulty swallowing
    • ringing in the ears and hearing loss
    • nausea and vomiting
    • impaired coordination
    • downward nystagmus (irregular eye movements)
    • paralysis
    • and more
  • a heavy headache: a constant to near constant headache that can be described as feeling like the head is too heavy for the neck to support (feeling like a “bobble-head”)
  • a pressure headache: an impairment of CSF flow causes intracranial pressure which would be aggravated by “valsalva maneuvers” such as yawning, laughing, crying, coughing, sneezing or straining.
  • Dysautonomia: brainstem compression can lead to a dysfunctional autonomic nervous system (the involuntary regulator of all body functions). Symptoms of this include, but are not limited to:
    • tachycardia (rapid heart)
    • heat intolerance
    • orthostatic intolerance (low blood pressure when standing)
    • syncope (fainting)
    • polydipsia (extreme thirst)
    • delayed gastric emptying
    • chronic fatigue

courtesy of The Pain Relief Foundation 

Cranio-cervical Instability in Patients with Hypermobility Connective Disorders

Fraser C. Henderson Sr. M.D. - Journal of Spine, 2016

Abstract: Cranio-cervical instability is well documented in connective tissue disorders such as rheumatoid arthritis, systemic lupus, and genetic disorders such as Down’s syndrome and Osteogenesis Imperfecta. However, less understood are the more than fifty genetic disorders of collagen characterized by joint laxity, and of course, laxity of the ligaments of the spine. Given the propensity in these patients for spinal instability, it is not surprising that the most severe symptoms arise in the most mobile part of the spine, the cranio-cervical junction. The increased recognition of hypermobility syndromic disorders, of which Ehlers Danlos Syndome (EDS) is emblematic, has prompted questions and concern as to what constitutes pathological instability in this category of patient, and how best to diagnose this instability.


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