Craniocervical instability

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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).[citation needed] 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 , 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 .[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.

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 on this approach.

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.
  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. ^ 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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