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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

History

  • 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)

Introduction

Illustration of the brachial plexus

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

Differential Diagnosis Shoulder Pain


Clinical Features

Inspection of the patient’s back at rest (A) and with pressing on the wall (B). Note the prominence of the scapular border and depressed shoulder on the right side.[9]

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: Physical Exam Shoulder

  • Cervical spine should be examined including strength, sensation and reflexes
  • May see fasiculations, atrophy
  • Provactive tests: Motion and Valsalva maneuvers
  • Muscles most commonly involved: Deltoid, Rotator Cuff, Serratus Anterior, Biceps Brachii and Triceps Brachii [10]
  • Sensation: Lateral Antebrachial Cutaneous Nerve (LAC) is affected in 32% of cases [11]
  • Another study found sensory deficits in up to 66% of patients[12]
  • Autonomic dysregulation (15% of cases, need citation): trophic skin changes, temperature dysregulation, increased sweating, altered nail and hair growth

Special Tests

  • Winging of scapula evaluation and wall push ups should be performed due to possible long thoracic nerve involvement

Evaluation

MRI left shoulder without contrast shows hyperintense signal in multiple muscles predominantly involving supraspinatus, infraspinatus and part of the deltoid muscles on T2 fat sat images. No mass lesion compressing the axillary or suprascapular nerve.No atrophy or fatty infiltration.[13]

Radiographs

EMG/NCS

  • Studies performed 3-4 weeks after onset, as test can be normal if performed within 3 weeks
  • Many compare to opposite limb with special attention to Lateral Antebrachial Cutaneous Nerve

MRI

  • MRI Brachial Plexus
    • Can show diffuse high signal intensity on T2-weighted images, involving one or more muscles innervated by the brachial plexus [14]
    • Like the EMGs, these findings may not appear for the first few weeks
    • Recent reports show hourglass constriction in peripheral nerves [15]
  • 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

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[18]

Rehab and Return to Play

Rehabilitation

  • No clearly defined rehabilitation protocol
  • One case report discusses 9 months of rehabilitation[19]
    • 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/Work

  • Unknown

Prognosis and Complications

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

Complications

  • Chronic pain, functional deficits
    • One out of three patients continue to experience chronic pain and persistent functional deficits after 6 years [20]
    • 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

Internal

External


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. Image courtesy of practicalneurology.com, "How to Know it When You See It: Diagnosing Neuralgic Amyotrophy (Parsonage-Turner Syndrome)"
  10. Rubin DI. Neuralgic amyotrophy: clinical features and diagnostic evaluation. Neurologist 2001;7:350–6.
  11. Dumitru D. Brachial plexopathies and proximal mononeuropathies Electrodiagnostic medicine. 2. New York: Mosby; 2002. pp. 623–624.
  12. Cwik VA, Wilbourn AJ, Rorick M: Acute brachial neuropathy: detailed EMG findings in a large series. Muscle Nerve 13: 859, 1990
  13. Case courtesy of Abeer Ahmed Alhelali, Radiopaedia.org, rID: 49793
  14. Gaskin CM, Helms CA. Parsonage Turner syndrome: MR imaging findings and clinical information of 27 patients. Radiology 2006;240:501–7.
  15. 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.
  16. van Alfen N, van Engelen BG, Hughes RA. Treatment for idiopathic and hereditary neuralgic amyotrophy (brachial neuritis). Cochrane Database Syst Rev 2009;(3):CD006976
  17. van Alfen N, van Engelen BG. The clinical spectrum of neuralgic amyotrophy in 246 cases. Brain 2006;129(2):438–50
  18. https://www.orthobullets.com/shoulder-and-elbow/3065/brachial-neuritis-parsonage-turner-syndrome
  19. Labrecque, LCDR Scott N. "Parsonage-Turner Syndrome: Diagnosis and Rehabilitation Strategies." FEDERAL PRACTITIONER (2013).
  20. 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:
22 March 2024 13:00:51
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