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Parsonage Turner Syndrome

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Other Names

  • Idiopathic Brachial Neuritis
  • Idiopathic Brachial Plexitis
  • Neuralgic Amyotrophy
  • Parsonage-Turner Syndrome (PTS)
  • Paralytic Brachial Neuritis
  • Idiopathic Brachial Plexopathy (IHA)
  • Hereditary Neuralgic Amyotrophy (HNA)

Background

  • Rare disorder of complex constellation of symptoms including abrupt onset of shoulder pain, usually unilaterally, followed by progressive neurologic deficits of motor weakness, dysesthesias, and numbness
    • Primarily affects shoulder girdle musculature, upper limb muscles
  • First reported in 1948 by Parsonage and Turner, but similar clinical presentations date back to 1897[1]

Epidemiology

  • Rare disease, true incidence and prevalence is likely under-reported
  • Incidence 1.64 per 100,000[2]
  • Male > Female[3]
  • Range of 3rd - 7th decades of life, case reports between 3 months and 75 years[4]
  • Typically unilateral, bilateral in 10-30% of patients (need citation)

Pathophysiology

  • Definition
    • Sudden or abrupt unilateral shoulder or upper extremity pain
    • May begin insidiously but quickly amplifies in severity and intensity
    • As the acute period resolves, patients develop progressive weakness, reflex and sensory abnormalities

Etiology

  • Considered inflammatory, but exact pathophysiology is complex and incompletely understood
  • Antecedent event identified in around 50 percent of cases and immune mediated response shown in 20-40 percent of cases [5]
  • Mechanical processes such as position related to surgical procedures, autoimmune, genetic and infection etiologies have been proposed
  • Also associated with coronary artery bypass, oral surgery, hysterectomy and a variety of orthopedic procedures [6]
    • Usually occur 24 hours to 1 week after procedure

Pathoanatomy

Risk Factors

  • Recent viral illness (Most common, up to 25%) [7]
  • Recent immunization including tetanus, hepatitis B (15%)
  • Recent surgical procedure
    • Orthopedic procedures
    • Coronary artery bypass surgery
    • Hysteroscopy
    • Oral surgery
  • Other Iatrogenic (need citation)
    • Interscalene Block
    • Lumbar puncture
    • Irradiation
  • Hereditary[8]
  • Rheumatic disease
    • Connective tissue disorders (i.e., Ehlers-Danlos Syndrome)
    • Systemic lupus erythematosus
    • Temporal arteritis
    • Polyarteritis nodosa
  • Trauma (remote or recent)
  • Strenuous exercise
  • Pregnancy and childbirth (up to 14%)
  • Pharmacologic (need citation)
    • Abacavir
    • Streptokinase
    • Heroin
    • Infliximab
    • Interferon

Differential Diagnosis

Clinical Features

  • General: Physical Exam Shoulder
    • Cervical spine should be examined including strength, sensation and reflexes
  • History
    • Thorough physical examination can show weakness or atrophy in musculature, especially once the acute phase has subsided
    • Presentation can be variable but patterns do exist
  • Phase 1 (pain)
    • Acute onset of severe pain that can last several days to weeks (usually 1-2 weeks)
    • Pain typically located in or around the shoulder and radiates down the arm or proximally
    • Occurs in both dominant and non dominant arm, although typically unilateral
    • Pain is not positional in nature, typically worse at night
  • Phase 2 (neurologic)
    • Weakness and muscle atrophy follow the pain phase in the following days to weeks
    • Most commonly the upper part of the brachial plexus and more than one nerve branch
  • Physical Exam
  • Special Tests
    • Winging of scapula evaluation and wall push ups should be performed due to possible long thoracic nerve involvement

Evaluation

Radiographs

EMG/NCS

  • Studies performed 3-4 weeks after onset, as test can be normal if performed within 3 weeks

MRI

  • MRI Brachial Plexus
    • Can show diffuse high signal intensity on T2-weighted images, involving one or more muscles innervated by the brachial plexus [12]
    • Like the EMGs, these findings may not appear for the first few weeks
    • Recent reports show hourglass constriction in peripheral nerves [13]
  • MRI Cervical Spine to rule out cervical disc disease or nerve root compression
  • MRI Shoulder can rule out rotator cuff pathology, labral tears, nerve entrapment or mass lesions

Laboratory

  • Normal: CBC, ESR
  • Other findings non-specific
  • May see: elevated liver enzymes
  • Positive antiganglioside antibodies
  • Positive antinuclear antibody (ANA) test
  • CSF: usually normal (may show elevated protein, pleocytosis, oligoclonal bands)

Classification

  • Idiopathic Brachial Plexopathy (IHA)
  • Hereditary Neuralgic Amyotrophy (HNA)

Management

Prognosis

  • Prognosis good for most cases and most make full functional recovery
  • Recurrence is rare in non-hereditary casees
  • Poor prognosis:
    • Female gender
    • Lower trunk involvement
    • Persistant pain, loss of motor function > 3 mo
  • Timeline (need citation)
    • 66% demonstrate some recovery of motor funcion at 1 month
    • "Excellent" recovery at 1 year (36%), 2 years (75%), 3 years (89%)
    • Case reports of up to 8 years for full recovery

Nonpharmacologic

Pharmacologic

Operative

  • Indications
    • Mass effect etiology
    • Lack of improvement at 6-9 months of conservative therapy you can consider surgery
  • Technique
    • Nerve exploration, neurolysis, neurorrhaphy, nerve grafting, nerve transfer or muscle/tendon transfers[16]

Rehab and Return to Play

Rehabilitation

  • No clearly defined rehabilitation protocol
  • One case report discusses 9 months of rehabilitation[17]
    • PT: education, pain management, active and passive ROM of the shoulder and other affected joints in all planes, strengthening exercises
    • OT: focused on regaining fine motor control and functional activities

Return to Play

  • Unknown

Complications

  • Chronic pain, functional deficits
    • One out of three patients continue to experience chronic pain and persistent functional deficits after 6 years [18]
    • Around one in three patients show excellent recovery after one year
    • Prolonged pain and weakness associated with poor prognosis
    • Recurrent or subsequent attacks can occur, but are typically not as severe

See Also

References

  1. ↑ Parsonage MJ, Turner JWA: The shoulder girdle syndrome. Lancet 1: 973–978, 1948
  2. ↑ Beghi E, Kurland LT, Mulder DW, Nicolosi A. Brachial plexus neuropathy in the population of Rochester, Minnesota, 1970–1981. Ann Neurol. 1985;18(3):320–3.
  3. ↑ Martin WA, Kraft GH: Shoulder girdle neuritis: a clinical and electrophysiologic evaluation. Mil Med 139: 21–25, 1974
  4. ↑ Tsairis, P, Dyck PJ, Mulder DW: Natural history of brachial plexus neuropathy. Report on 99 patients. Arch Neurol 27: 109–117, 1972
  5. ↑ van Alfen N, van Engelen BG. The clinical spectrum of neuralgic amyotrophy in 246 cases. Brain 2006;129(2):438–50
  6. ↑ Feinberg JH, Radecki J. Parsonage-turner syndrome. HSS J. 2010;6(2):199–205. doi:10.1007/s11420-010-9176-x
  7. ↑ Fibuch EE, Mertz J, Geller B. Postoperative onset of idiopathic brachial neuritis. Anesthesiology. 1996;84:455–458.
  8. ↑ Feinberg, Joseph H., and Jeffrey Radecki. "Parsonage-turner syndrome." HSS journal 6.2 (2010): 199-205.
  9. ↑ Rubin DI. Neuralgic amyotrophy: clinical features and diagnostic evaluation. Neurologist 2001;7:350–6.
  10. ↑ Dumitru D. Brachial plexopathies and proximal mononeuropathies Electrodiagnostic medicine. 2. New York: Mosby; 2002. pp. 623–624.
  11. ↑ Cwik VA, Wilbourn AJ, Rorick M: Acute brachial neuropathy: detailed EMG findings in a large series. Muscle Nerve 13: 859, 1990
  12. ↑ Gaskin CM, Helms CA. Parsonage Turner syndrome: MR imaging findings and clinical information of 27 patients. Radiology 2006;240:501–7.
  13. ↑ Sneag DB, Rancy SK, Wolfe SW, Lee SC, Kalia V, Lee SK, Feinberg JH. Brachial plexitis or neuritis? MRI features of lesion distribution in Parsonage-Turner syndrome. Muscle Nerve. 2018 Sep;58(3):359-366.
  14. ↑ van Alfen N, van Engelen BG, Hughes RA. Treatment for idiopathic and hereditary neuralgic amyotrophy (brachial neuritis). Cochrane Database Syst Rev 2009;(3):CD006976
  15. ↑ van Alfen N, van Engelen BG. The clinical spectrum of neuralgic amyotrophy in 246 cases. Brain 2006;129(2):438–50
  16. ↑ https://www.orthobullets.com/shoulder-and-elbow/3065/brachial-neuritis-parsonage-turner-syndrome
  17. ↑ Labrecque, LCDR Scott N. "Parsonage-Turner Syndrome: Diagnosis and Rehabilitation Strategies." FEDERAL PRACTITIONER (2013).
  18. ↑ van Alfen N, van Engelen BG, Hughes RA. Treatment for idiopathic and hereditary neuralgic amyotrophy (brachial neuritis). Cochrane Database Syst Rev 2009;(3):CD006976
Created by:
John Kiel on 14 June 2019 08:33:54
Last edited:
1 October 2022 19:13:05
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