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Neuralgic amyotrophy

Last updated: December 13, 2017

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Neuralgic amyotrophy (also referred to as brachial neuritis or Parsonage-Turner syndrome) is a self-limiting inflammatory disorder of the brachial plexus, that mainly affects males between 20–30 years of age. There are two clinically similar, yet etiologically distinct forms of neuralgic amyotrophy (NA). Idiopathic NA is more common, often unilateral, and non-recurrent. It is thought to be an immune-mediated process triggered by preceding viral infection, immunization, or trauma. Hereditary NA is a rare, autosomal dominant condition that often affects both shoulders and is characterized by recurrent symptoms. In both types, patients present with acute onset of excruciating shoulder pain that lasts for weeks followed by patchy lower motor neuron paresis of the proximal muscles in the affected arm. Diagnosis is clinical, with nerve conduction studies and needle electromyography performed to identify which nerves are affected. Treatment is mainly supportive and involves analgesia and physical therapy. Most patients recover complete muscle strength within 2 years.

  • Incidence: 1–3 cases per 100,000 population per year
  • Peak age: 20–30 years
  • Sex: >

References:[1][2][3][4]

Epidemiological data refers to the US, unless otherwise specified.

References:[1][3][4][5][6]

The exact pathophysiology is unknown, but it is believed to be multifactorial.

  • Environmental factors (e.g., viral infection, immunization, surgery) → activation of the immune system
  • Mechanical factors (e.g., repetitive strain, recent strenuous exercise) → repeated stretching of the brachial plexus nerves → predisposition of the brachial plexus to immune-mediated injury

A combination of the above factors → patchy inflammation of the brachial plexus → axonal injury of the affected nerves → severe burning pain, followed by paresis of the muscles supplied by the affected nerve

References:[3]

References:[1][3][4][5][6][7][8][9]

NA is mainly a clinical diagnosis.

References:[3][4][6][7][8]

References:[1][3][4][5]

The differential diagnoses listed here are not exhaustive.

There is no specific causative treatment for NA. The condition is self-limiting and is treated symptomatically.

References:[2][3][4][6][7][10][11]

  • Prognosis is variable
    • Some patients recover complete muscle strength and are pain-free within 1–2 years
    • Some may have pain and paresis for many years, causing significant functional disability
  • Recurrence
    • Idiopathic NA: often non-recurrent
    • Hereditary NA: ∼ 75% of patients will have recurrent NA

References:[1][3][4][9]

  1. Hereditary Neuralgic Amyotrophy. https://rarediseases.org/rare-diseases/hereditary-neuralgic-amyotrophy/. Updated: January 1, 2017. Accessed: May 17, 2017.
  2. Gupta A, Winalski CS, Sundaram M. Neuralgic amyotrophy (Parsonage Turner syndrome). Orthopedics. 2014; 37 (2): p.75-133. doi: 10.3928/01477447-20140124-01 . | Open in Read by QxMD
  3. Hussey AJ, O'Brien CP, Regan PJ. Parsonage–Turner syndrome—case report and literature review. Hand. 2007; 2 (4): p.218-221. doi: 10.1007/s11552-007-9059-x . | Open in Read by QxMD
  4. van Alfen N. The neuralgic amyotrophy consultation. J Neurol. 2007; 254 (6): p.695-704. doi: 10.1007/s00415-006-0246-4 . | Open in Read by QxMD
  5. Sathasivam S, Lecky B, Manohar R, Selvan A. Neuralgic amyotrophy. Bone and Joint Journal. 2008; 90 (5): p.550-553. doi: 10.1302/0301-620X.90B5.20411 . | Open in Read by QxMD
  6. Feinberg JH, Radecki J. Parsonage-Turner Syndrome. HSS J . 2010; 6 (2): p.199-205. doi: 10.1007/s11420-010-9176-x . | Open in Read by QxMD
  7. Miller JD, Pruitt S, McDonald TJ. Acute brachial plexus neuritis: An uncommon cause of shoulder pain. Am Fam Physician. 2000; 62 (9): p.2067-2072.
  8. McEnery T, Walsh R, Burke C, McGowan A, Faul J, Cormican L. Phrenic nerve palsy secondary to Parsonage-Turner syndrome: a diagnosis commonly overlooked. Lung. 2017; 195 (2): p.173-177. doi: 10.1007/s00408-016-9972-2 . | Open in Read by QxMD
  9. Gonzalez-Alegre P, Recober A, Kelkar P. Idiopathic brachial neuritis. Iowa Orthop J. 2002; 22 : p.81-85.
  10. Treatment for Neuralgic Amyotrophy. http://www.cochrane.org/CD006976/NEUROMUSC_treatment-for-neuralgic-amyotrophy. Updated: July 8, 2009. Accessed: May 17, 2017.
  11. Ashworth NL. Brachial Neuritis. In: Klein MJ, Brachial Neuritis. New York, NY: WebMD. http://emedicine.medscape.com/article/315811. Updated: January 18, 2017. Accessed: May 17, 2017.