| Patient Education |
|
Click here for patient education.
|
|
AUTHOR AND EDITOR INFORMATION
Section 1 of 11  
Author: Nigel L Ashworth, MB, ChB, MSc, FRCPC, Chief, Associate Professor, Division of Physical Medicine and Rehabilitation, Glenrose Rehabilitation Hospital, University of Alberta
Nigel L Ashworth is a member of the following medical societies: American Academy of Physical Medicine and Rehabilitation, American Association of Neuromuscular and Electrodiagnostic Medicine, Association of Academic Physiatrists, Australian & New Zealand Association of Neurologists, British Medical Association, Canadian Association of Physical Medicine and Rehabilitation, Canadian Medical Association, Canadian Society of Clinical Neurophysiologists, and Royal College of Physicians and Surgeons of Canada
Editors: Benjamin M Sucher, DO, FAAPMR, FAOCPMR, Medical Director, EMG Center of Arizona and Electrodiagnostic Medical Group; Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine; Michael T Andary, MD, MS, Residency Program Director, Professor, Department of Physical Medicine and Rehabilitation, Michigan State University College of Osteopathic Medicine; Kelly L Allen, MD, Consulting Staff, Department of Physical Medicine and Rehabilitation, Lourdes Regional Rehabilitation Center, Our Lady of Lourdes Medical Center; Robert H Meier III, MD, Director, Amputee Services of America, Presbyterian St Luke's Hospital; Consulting Staff, North Valley Rehabilitation Hospital, Kindred Hospital, and North Suburban Hospital
Author and Editor Disclosure
Synonyms and related keywords:
brachial neuritis, BN, acute brachial neuropathy, acute brachial plexitis, acute brachial radiculitis, acute shoulder neuritis, brachial plexus neuropathy, cryptogenic brachial neuropathy, idiopathic brachial plexopathy, idiopathic brachial neuritis, localized neuritis of the shoulder girdle, localized nontraumatic neuropathy, multiple neuritis of the shoulder girdle, neuralgic amyotrophy, paralytic brachial neuritis, Parsonage-Turner syndrome, serum neuritis, shoulder girdle neuritis, shoulder girdle syndrome
Background
Brachial neuritis (BN) is a rare syndrome of unknown etiology affecting mainly the lower motor neurons of the brachial plexus and/or individual nerves or nerve branches. BN usually is characterized by the acute onset of excruciating unilateral shoulder pain, followed by flaccid paralysis of shoulder and parascapular muscles several days later. The syndrome can vary greatly in presentation and nerve involvement.
Pathophysiology
BN exists in an inherited and an idiopathic form. In the idiopathic version, the pathophysiology is unknown, but the condition is generally thought to be an immune-mediated inflammatory reaction against nerve fibers of the brachial plexus.1 Axonopathy with subsequent Wallerian degeneration appears to predominate, but proximal conduction block has also been described in over 33% of cases in the series by Lo and Mills.2 The inherited form is autosomal dominant and has been linked to mutations in the SEPT9 gene on chromosome 17q.3 Septins are involved in the formation of the cytoskeleton and cell division but how these mutations result in BN is unknown.
Frequency
United States
The incidence is approximately 1-2 cases per 100,000 person-years.
International
The incidence is approximately 3 per 100,000 person-years in the UK (MacDonald, 2000). BN has also been described in many countries around the world, although specific rates of incidence have not been reported.
Mortality/Morbidity
BN is not a fatal condition, although the phrenic nerve may be involved. The risk of significant residual disability in the involved limb after 2 years is approximately 10-20%.
Sex
A male predominance exists with BN, with a male-to-female ratio ranging from 2:1 to 4:1.
Age
Individuals of any age from 3 months to 74 years can be affected with BN; however, the prevalence is highest in young to middle-aged adults. Onset in childhood should be considered suggestive of hereditary BN.
History
- Pain
- Onset of pain is often abrupt and may follow recent illness, surgery, immunization, or even trauma (see Causes below). Up to two thirds of cases begin during the nighttime.
- Usually localized to the right shoulder region, the pain may be bilateral in 10-30% of cases.
- Intensity of pain is very high (9+/10) and is maximal at onset.
- Usually, the pain is described as sharp or throbbing in nature.
- Pain usually is constant, but it is exacerbated by movements of the shoulder. Movements of the neck, coughing, and/or sneezing usually do not worsen the pain.
- Intense pain can last a few hours to several weeks and requires opiate analgesia.
- Low-grade pain may persist for up to a year.
- Weakness
- As the pain subsides, weakness becomes apparent. In most cases, this weakness manifests within about 2 weeks of onset.
- Weakness is maximal at onset but can progress over 1 or more weeks.
- A wide variety of muscles is affected, particularly those innervated by the upper trunk. The supraspinatus, infraspinatus, serratus anterior, and deltoid muscles are particularly susceptible, but many different single and multiple combinations of muscle involvement, including a pure distal form, have been reported.
- The patient may notice considerable atrophy and wasting, as well as a deep aching in the affected muscles.
- Other symptoms
- Numbness may occur, depending on the particular nerves affected, and usually is found in the nerve distribution corresponding to the maximal muscle weakness; however, numbness is rarely a prominent complaint.
- Phrenic nerve involvement occurs in up to 5% of cases and can result in significant shortness of breath.4, 5, 6
- Variants of BN can present with isolated or multiple cranial neuropathies (IX, X, XI, XII).
- In 25-50% of patients, the medical history indicates a viral illness or vaccination that occurred days or weeks prior to the onset of symptoms. Some patients also may note recent trauma or severe exercise, surgery, infection, or immunization.
Physical
- Due to the extreme pain involved, patients usually present acutely. Typically, the affected arm is supported by the uninvolved arm and is held in adduction and internal rotation.
- Atrophy of the affected muscles becomes prominent after approximately 2 weeks.
- Considerable muscle pain may be noted on palpation.
- Passive and active attempts at shoulder and scapular movement result in significant increase in pain. Movements of the neck are relatively pain-free.
- Muscle strength in affected muscles often is reduced severely (to 2 or less on the Medical Research Council [MRC] grading scale).
- Reflexes may be reduced or absent, depending on which nerves are involved.
- Sensory loss is not prominent but may be detectable, depending on the specific nerves affected (in particular, loss of axillary nerve sensation).
Causes
- The exact cause of BN is unknown, but the condition has been linked to many antecedent events or illnesses.
- Viral infection (particularly of the upper respiratory tract)
- Bacterial infection (eg, pneumonia, diphtheria, typhoid)
- Parasitic infestation
- Surgery
- Trauma (not related to shoulder)
- Vaccinations (eg, influenza; tetanus; diphtheria, tetanus toxoids, pertussis [DPT]; smallpox; swine flu)
- Childbirth
- Miscellaneous medical investigative procedures (eg, lumbar puncture [LP], administration of radiologic dye)
- Systemic illness (eg, polyarteritis nodosa, lymphoma, systemic lupus erythematosus, temporal arteritis, Ehlers-Danlos syndrome)
- A rarer, hereditary form of BN has been localized to the SEPT9 gene on chromosome arm 17q and should be considered a distinct disorder. This entity presents in a younger age group, affects males and females equally (autosomal-dominant inheritance), and is characterized by recurrent attacks that are often bilateral. Dysmorphic facial features (eg, hypotelorism, long nasal bridge, facial asymmetry) can also be present.
Acute Poliomyelitis
Adhesive Capsulitis
Amyotrophic Lateral Sclerosis
Cervical Disc Disease
Mononeuritis Multiplex
Neoplastic Brachial Plexopathy
Rotator Cuff Disease
Thoracic Outlet Syndrome
Other Problems to be Considered
Acute calcific tendonitis
Cervical radiculopathy
Human immunodeficiency virus (HIV)
Pack palsy
Polymyalgia rheumatica
Sarcoidosis and other granulomatous infiltrations
Spinal cord tumor
Traumatic mononeuropathies
Lab Studies
- Usually within the reference range
- Only indicated if systemic disease is suspected on clinical grounds
- CBC count and ESR as nonspecific indicators of systemic disease
- Antinuclear antibody (ANA) as a marker for connective tissue disease
- HIV serology
Imaging Studies
- MRI or CT-myelogram scan should be considered initially to rule out cervical radiculopathy (particularly C5/C6). MRI of the brachial plexus can help to rule out carcinomatous or granulomatous infiltration, if clinically indicated.
- A shoulder radiograph may be indicated to rule out specific shoulder pathologies.
- Chest radiograph is not usually part of the initial work-up; however, it can be useful to rule out sarcoidosis or other granulomatous disease, as well as Pancoast tumor.
Other Tests
- Electrodiagnosis
- Electrodiagnosis (EDX) should be considered initially to confirm neuropathic diagnosis and to rule out various other conditions (eg, radiculopathy, neuropathy, amyotrophic lateral sclerosis).
- Specific localization can be made to various nerves.
- Loss of sensory and motor amplitudes with relatively normal conduction velocity is frequent. Note: This finding only begins to fall beyond the reference range after approximately 1 week.
- Somatosensory evoked responses are not reliable for distinguishing radiculopathy from brachial plexus neuropathy, and F-waves are generally less helpful than routine conduction studies in localization.
- Needle electromyogram (EMG) shows denervation (fibrillations, positive sharp waves, and/or motor unit potential changes) in affected muscles 2-3 weeks after onset; however, clinically uninvolved muscles also may show abnormalities. Approximately 50% of patients with unilateral clinical involvement demonstrate bilateral EMG abnormalities. EMG results for the paraspinal muscles usually are within the reference range. Electromyographic exclusion of a radiculopathy may be challenging. In addition, strict anatomic localization often is difficult.
- Proximal conduction block has been reported in BN; however, this finding should suggest focal forms of inflammatory demyelinating diseases. Proximal slowing also may occur from loss of large fibers, regenerating fibers, and/or segmental demyelination.
Procedures
- Nerve biopsy usually is not indicated. Axonal loss rarely is identified with biopsies of the radial sensory branch.
- LP usually is not indicated. Analysis of cerebrospinal fluid (CSF) generally is within the reference range in individuals with BN.
Rehabilitation Program
Physical Therapy
Physical therapy (PT) should be focused on the maintenance of full range of motion (ROM) in the shoulder and other affected joints. Passive range of motion (PROM) and active range of motion (AROM) exercises should begin as soon as the pain has been controlled adequately, followed by regional conditioning of the affected areas. Strengthening of the rotator cuff muscles and scapular stabilization may be indicated. Passive modalities (eg, heat, cold, transcutaneous electrical nerve stimulation [TENS]) may be useful as adjunct pain relievers.
Occupational Therapy
Functional conditioning of the upper extremity may be helpful. Assistive devices and orthotics may be used, depending on the particular disabilities present. The occupational therapist (OT) may be involved in maintaining ROM and strengthening, particularly if the hand and wrist are involved.
Medical Issues/Complications
Treatment is largely symptomatic, and opiate analgesia often is necessary in the initial period. Immunosuppressive therapy (eg, steroids, immunoglobulin, plasma exchange) has not been shown to be beneficial.
Surgical Intervention
Nerve grafting or tendon transfers may be considered for the few patients who do not achieve good recovery by 2 years. Surgery usually is aimed at improving shoulder abduction.
Consultations
- A specialist in neuromuscular disease may be consulted to confirm diagnosis and evaluate any potentially underlying causes.
- A specialist in neuromuscular rehabilitation may be asked to provide a comprehensive rehabilitation program aimed at maintaining ROM and improving strength and function.
The goal of pharmacotherapy is to reduce morbidity and prevent complications.
Drug Category: Analgesics
Often necessary initially to control pain.
| Drug Name | Acetaminophen (Tylenol, Panadol, Aspirin Free Anacin) |
|---|
| Description | DOC for pain in patients with documented hypersensitivity to aspirin, NSAIDs, diagnosed with upper GI disease or on oral anticoagulants. |
|---|
| Adult Dose | 325-650 mg PO q4-6h or 1000 mg tid/qid; not to exceed 4 g/d |
|---|
| Pediatric Dose | <12 years: 10-15 mg/kg/dose PO q4-6h prn; not to exceed 2.6 g/d
>12 years: 325-650 mg PO q4h; not to exceed 5 doses in 24 h |
|---|
| Contraindications | Documented hypersensitivity |
|---|
| Interactions | Rifampin can reduce analgesic effects; coadministration with barbiturates, carbamazepine, hydantoins, and isoniazid may increase hepatotoxicity |
|---|
| Pregnancy | B - Usually safe but benefits must outweigh the risks.
|
|---|
| Precautions | Hepatotoxicity can occur in chronic alcoholics with various dose levels; severe or recurrent pain or high or continued fever may indicate a serious illness |
|---|
| Drug Name | Acetaminophen and codeine (Tylenol #3) |
|---|
| Description | Indicated for the treatment of mild to moderate pain. |
|---|
| Adult Dose | 30-60 mg based on codeine content PO q4-6h or 1-2 tab q4h; not to exceed 12 tab/d |
|---|
| Pediatric Dose | 0.5-1 mg/kg based on codeine PO q4-6h; 0-15 mg/kg PO based on acetaminophen content; not to exceed 2.6 g/d of acetaminophen |
|---|
| Contraindications | Documented hypersensitivity |
|---|
| Interactions | Toxicity increases with CNS depressants or tricyclic antidepressants |
|---|
| Pregnancy | C - Safety for use during pregnancy has not been established.
|
|---|
| Precautions | May result in acute withdrawal symptoms in patients dependent on opiates; caution in severe renal or hepatic dysfunction |
|---|
| Drug Name | Hydrocodone bitartrate and acetaminophen (Vicodin ES) |
|---|
| Description | Drug combination indicated for moderate to severe pain. |
|---|
| Adult Dose | 1-2 tab or cap PO q4-6h prn |
|---|
| Pediatric Dose | >12 years: 750 mg acetaminophen PO q4h; not to exceed 10 mg hydrocodone bitartrate per dose or 5 doses/24h
<12 years: 10-15 mg/kg acetaminophen PO q4-6h prn; not to exceed 2.6 g/d acetaminophen |
|---|
| Contraindications | Documented hypersensitivity; elevated intracranial pressure |
|---|
| Interactions | Coadministration with phenothiazines may decrease analgesic effects; toxicity increases with CNS depressants or tricyclic antidepressants |
|---|
| Pregnancy | C - Safety for use during pregnancy has not been established.
|
|---|
| Precautions | Tablets contain metabisulfite, which may cause allergic reactions; caution in severe renal or hepatic dysfunction |
|---|
| Drug Name | Oxycodone and acetaminophen (Percocet) |
|---|
| Description | Drug combination indicated for the relief of moderate to severe pain. DOC for aspirin hypersensitive patients. |
|---|
| Adult Dose | 1-2 tab or cap PO q4-6h prn |
|---|
| Pediatric Dose | 0.05-0.15 mg/kg oxycodone PO; not to exceed 5 mg/dose of oxycodone q4-6h prn |
|---|
| Contraindications | Documented hypersensitivity |
|---|
| Interactions | Phenothiazines may decrease analgesic effects; toxicity increases with coadministration of either CNS depressants or tricyclic antidepressants |
|---|
| Pregnancy | C - Safety for use during pregnancy has not been established.
|
|---|
| Precautions | Duration of action may increase in elderly patients; be aware of total daily dose of acetaminophen patient is receiving; do not exceed 4000 mg/24 h of acetaminophen; higher doses may cause liver toxicity |
|---|
Further Outpatient Care
- Treatment of BN usually is completed conservatively on an outpatient basis. Surgical intervention is indicated for patients who do not demonstrate good recovery after 2 years of conservative care. Please refer to the Rehabilitation section for a discussion of outpatient rehabilitation and treatment options for individuals with BN.
Complications
- Shoulder joint contractures (and potentially others)
- Sudden severe dyspnea due to phrenic nerve involvement may be noted with or without other symptoms of brachial BN; however, there should be no other evidence of lung disease.
Prognosis
- Eighty percent of patients recover functionally by 2 years; 90% recover functionally by 3 years.
- Bilateral disease has a less favorable outcome than unilateral disease.
- Lower trunk lesions have a less favorable prognosis than upper trunk lesions.
- The rate of recurrence in the inherited form is approximately 75%. The recurrence in the idiopathic form is thought to be between 5% and 26% (van Alfen, 2005).
Medical/Legal Pitfalls
- Misdiagnosis of BN as cervical radiculopathy, rotator cuff disease, or entrapment neuropathy is common.
| Media file 1:
The patient is a 43-year-old farmer, shown 6 months after presenting with severe right shoulder pain and weakness. Note severe wasting of the right infraspinatus and deltoid and winging of the scapula. |
 |  View Full Size Image | |
Media type: Photo
|
| Media file 2:
This is the same patient as in Image 1. Note again severe supraspinatus and infraspinatus wasting on the right. |
 | View Full Size Image | |
Media type: Photo
|
- Suarez GA, Giannini C, Bosch EP, et al. Immune brachial plexus neuropathy: suggestive evidence for an inflammatory-immune pathogenesis. Neurology. Feb 1996;46(2):559-61. [Medline].
- Lo YL, Mills KR. Motor root conduction in neuralgic amyotrophy: evidence of proximal conduction block. J Neurol Neurosurg Psychiatry. May 1999;66(5):586-90. [Medline].
- Kuhlenbäumer G, Hannibal MC, Nelis E. Mutations in SEPT9 cause hereditary neuralgic amyotrophy. Nat Genet. Oct 2005;37(10):1044-6.
- Kumar N, Folger WN, Bolton CF. Dyspnea as the predominant manifestation of bilateral phrenic neuropathy. Mayo Clin Proc. Dec 2004;79(12):1563-5. [Medline].
- Lahrmann H, Grisold W, Authier FJ, Zifko UA. Neuralgic amyotrophy with phrenic nerve involvement. Muscle Nerve. Apr 1999;22(4):437-42. [Medline].
- Tsao BE, Ostrovskiy DA, Wilbourn AJ, et al. Phrenic neuropathy due to neuralgic amyotrophy. Neurology. May 23 2006;66(10):1582-4. [Medline].
- Aymond JK, Goldner JL, Hardaker WT Jr. Neuralgic amyotrophy. Orthop Rev. Dec 1989;18(12):1275-9. [Medline].
- Dumitru D. Brachial plexopathies and proximal mononeuropathies. In: Electrodiagnostic Medicine. Philadelphia:. Hanley & Belfus;1994:585-642.
- England JD. The variations of neuralgic amyotrophy. Muscle Nerve. Apr 1999;22(4):435-6. [Medline].
- England JD, Sumner AJ. Neuralgic amyotrophy: an increasingly diverse entity. Muscle Nerve. Jan 1987;10(1):60-8. [Medline].
- Flaggman PD, Kelly JJ Jr. Brachial plexus neuropathy. An electrophysiologic evaluation. Arch Neurol. Mar 1980;37(3):160-4. [Medline].
- MacDonald BK, Cockerell OC, Sander JW. The incidence and lifetime prevalence of neurological disorders in a prospective community-based study in the UK. Brain. Apr 2000;123 ( Pt 4):665-76.
- Magee KR, Dejong RN. Paralytic brachial neuritis. Discussion of clinical features with review of 23 cases. JAMA. Nov 5 1960;174:1258-62. [Medline].
- Martin WA, Kraft GH. Shoulder girdle neuritis: a clinical and electrophysiological evaluation. Mil Med. Jan 1974;139(1):21-5. [Medline].
- McCarty EC, Tsairis P, Warren RF. Brachial neuritis. Clin Orthop. Nov 1999;(368):37-43. [Medline].
- Misamore GW, Lehman DE. Parsonage-Turner syndrome (acute brachial neuritis). J Bone Joint Surg Am. Sep 1996;78(9):1405-8. [Medline].
- Parsonage MJ, Aldren Turner JW. Neuralgic amyotrophy: The shoulder girdle syndrome. Lancet. 1948;1:973-8.
- Pierre PA, Laterre CE, Van den Bergh PY. Neuralgic amyotrophy with involvement of cranial nerves IX, X, XI and XII [published erratum appears in Muscle Nerve 1991 Jan;14(1):88]. Muscle Nerve. Aug 1990;13(8):704-7. [Medline].
- Stogbauer F, Young P, Kuhlenbaumer G, et al. Hereditary recurrent focal neuropathies: clinical and molecular features. Neurology. Feb 8 2000;54(3):546-51. [Medline].
- Subramony SH. AAEE case report #14: neuralgic amyotrophy (acute brachial neuropathy). Muscle Nerve. Jan 1988;11(1):39-44. [Medline].
- Tsairis P, Dyck PJ, Mulder DW. Natural history of brachial plexus neuropathy. Report on 99 patients. Arch Neurol. Aug 1972;27(2):109-17. [Medline].
- Turner JW, Parsonage MJ. Neuralgic amyotrophy (paralytic brachial neuritis); with special reference to prognosis. Lancet. Aug 3 1957;273(6988):209-12. [Medline].
- van Alfen N. The neuralgic amyotrophy consultation. J Neurol. Jun 2007;254(6):695-704. [Medline].
- van Alfen N, van Engelen BG. The clinical spectrum of neuralgic amyotrophy in 246 cases. Brain. Feb 2006;129:438-50. [Medline]. [Full Text].
- Walker M, Zunt JR, Kraft GH. Brachial neuropathy after immunosuppression and stem cell transplantation for multiple sclerosis. Mult Scler. Feb 2005;11(1):90-1. [Medline].
- Weikers NJ, Mattson RH. Acute paralytic brachial neuritis. A clinical and electrodiagnostic study. Neurology. Dec 1969;19(12):1153-8. [Medline].
- Wilbourn AJ. Brachial plexus disorders. In: Peripheral Neuropathy. Philadelphia:. WB Saunders Co;1993:911-950.
Brachial Neuritis excerpt Article Last Updated: Oct 12, 2006
|
