Occipital Neuralgia and Chiari malformation

By Diane Mueller, ND, RN, FNP-BC

Occipital Neuralgia (ON) is defined by the International Headache Society (IHS) as “paroxysmal jabbing pain in the distribution of the greater or lesser occipital nerves…. sometimes accompanied by diminished sensation or dysaesthesia in the affected area” (1). The prevalence/incidence of ON among the general US population is not confirmed. Lord, Barnsley, Wallis, & Bogduk (2) documented the incidence of third occipital nerve headache associated with whiplash injury as high as 53%. A population based study from the Netherlands (3) indicated the incidence of facial pain increased with age, was more frequent among women than men, and was likely more common than previously estimated.

Causes of ON are many and can include trauma to the nerve area, compression of the nerve root, mass lesion, and Chiari I malformation (CMI) (4). Surgical procedures near the area of the Occipital nerve, often using a self-retaining retractor can produce tension on the nerve, and local scar tissue resulting in chronic nerve pain. Rhee, You, Kim and Lee (5) presented the case of a 56 year old with severe, chronic occipital nerve pain after unilateral Harms construct procedure for traumatic atlantoaxial instability. Cervical arthropathy can also be associated with ON headaches (6), and the treatments can similar- including physical therapy, medications, and injections.

Chronic head pain is a primary presenting symptom of both ON and CMI. A search of the medical literature (in English) revealed no studies that correlated the incidence/prevalence of ON with CMI. Kanpolat (7) reported the case of a 50 year old male with glossopharyngeal neuralgia in addition to MRI confirmation of CMI. The primary presenting symptom of CMI is headache- typically occurring in the posterior occipital area, and often radiating to the bitemporal or forehead areas (8,9). An MRI of the brain and/or cervical spine is the test of choice for diagnosis of CMI. Definitive diagnosis of ON is typically a combination of clinical evaluations, including tenderness in the area of the greater occipital nerve and positive response to local nerve block (4,10).

Treatments for ON consist of nerve blocks, physical therapy, cutaneous nerve stimulators (11), analgesics, and anti-inflammatory medications. In cases of refractory pain, surgical decompression of the nerve has shown promise (12). Treatment for CMI can include analgesics and posterior fossa decompression for those persons refractory to conservative management. Many patients anecdotally report occipital nerve pain after posterior fossa decompression. Surgeons who perform posterior fossa decompression for CMI should be aware of the possible association of postoperative ON. However, the true incidence and pathogenesis of occipital neuralgia after decompression for CMI remains to be determined.

Definitive diagnosis of ON concurrently with CMI may be difficult due to overlapping pain complaints. Effective pain management of both conditions remains a clinical challenge. Careful clinical appraisal may help in determining subgroups of patients with refractory chronic headache who are likely to respond to interventions specific for Occipital Neuralgia. Patients with radiographic evidence of CMI should also be screened for ON prior to contemplation of surgery. Successful long term outcome of Chiari decompression should include consideration of the co-morbid pain of Occipital Neuralgia. Consideration of both conditions concurrently may potentially provide improved relief of refractory pain. A prospective study of patients undergoing posterior fossa decompression for CMI would offer an exciting opportunity to determine multimodal treatment options for refractory headache sufferers.

  1. International Headache Society: http://www.ihsclassification.org/en/02_klassifikation/04_teil3/13.08.00_facialpain.html
  2. Lord S.M., Barnsley L., Wallis B.J., Bogduk N. Third occipital nerve headache: a prevalence study. Journal of Neurology, Neurosurgery and Psychiatry 1994; 57:1187-1190.
  3. Koopman J.S., Dieleman J.P., Huygen F.J., deMos M., Martin C.G., & Sturkenboom M.C. Incidence of facial pain in the general population. Pain 2009 Dec 15;147(1-3):122-7.
  4. Tubbs R.S., Mortazavi M.M., Loukas M., d’Antoni A.V., Shoja M.M., Chern J.J., Cohen-gadol A.A. Anatomical study of the third occipital nerve and its potential role in occipital headache/neck pain following midline dissections of the craniocervical junction. Journal Neurosurg:Spine 2011: 15, 71-75.
  5. Rhee W-T., You, S-H., Kim S-K., & Lee S-Y. Troublesome occipital neuralgia developed by C1-C2 Harms construct. Journal of Korean Neurosurgery Society 2008; 43(2):111-113.
  6. Hoppenfeld J.D. Cervical facet arthropathy and occipital neuralgia: Headache culprits. Current Pain and Headache Report. 2010; 14:418-423.
  7. Kanpolat Y., Unlu A., Savas A., & Tan F. Chiari type I malformation presenting as glossopharyngeal neuralgia: a case report. Neurosurgery; 2001: 48(1):226-228.
  8. Grazzi L., Andrasik F. Headaches and Arnold-chiari syndrome: When to suspect and how to investigate. Current Pain and Headache Report, 2012;16:350-353.
  9. Mueller, D.M., Oro, J.J. Prospective analysis of presenting symptoms among 265 patients with radiographic evidence of Chiari I Malformation with or without syringomyelia. Journal of the American Academy of Nurse Practitioners, 2004;16(3): 134-138.
  10. NINDS Occipital Neuralgia Information Page: http://www.ninds.nih.gov/disorders/occipitalneuralgia/occipitalneuralgia.htm
  11. Paemeleire K & Bartsch T. Occipital nerve stimulation for headache disorders. Neurotherapeutics: The Journal of the American Society for Experimental NeuroTherapeutics 2010; 7(2):213-219.
  12. Li F., MA Y., Zou J., LI Y., Want B., Huang, H., Wang Q., & LI L. Micro-surgical decompression for greater Occipital Neuralgia. Turk Neurosurg. 2012;22(4):427-9. DOI:10.5137/1019-5149. JTN.5234-11.1.

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