eMedicine Specialties > Physical Medicine and Rehabilitation > Peripheral Neuropathy

Brachial Neuritis

Author: Nigel L Ashworth, MBChB, MSc, FRCPC, Professor and Chief, Division of Physical Medicine and Rehabilitation, Glenrose Rehabilitation Hospital, University of Alberta
Contributor Information and Disclosures

Updated: Sep 16, 2009

Introduction

Background

Brachial neuritis (BN), also known as neuralgic amyotrophy, 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.1,2 (See images below and Images 1-2.)

The patient is a 43-year-old farmer, shown 6 mont...

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.

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The patient is a 43-year-old farmer, shown 6 mont...

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.


Same patient as above. Note again severe supraspi...

Same patient as above. Note again severe supraspinatus and infraspinatus wasting on the right.

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Same patient as above. Note again severe supraspi...

Same patient as above. Note again severe supraspinatus and infraspinatus wasting on the right.


Pathophysiology

Brachial neuritis (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 system – mediated inflammatory reaction against nerve fibers of the brachial plexus.3,4,5 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.6 The inherited form is autosomal dominant and has been linked to mutations in the SEPT9 gene on chromosome 17q.7,8,9 Septins are involved in the formation of the cytoskeleton and in cell division, but how these mutations result in BN is unknown.

Frequency

United States

The incidence of brachial neuritis is approximately 1-2 cases per 100,000 person-years.

International

In the United Kingdom, the incidence of brachial neuritis (BN) is approximately 3 per 100,000 person-years.10 BN has also been described in many countries around the world, although specific rates of incidence have not been reported.

Mortality/Morbidity

Brachial neuritis 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%.11

Sex

Brachial neuritis occurs predominantly in males, with the male-to-female ratio for the condition ranging from 2:1 to 4:1.

Age

Brachial neuritis (BN) has been reported in individuals from age 3 months to 74 years; however, the condition's prevalence is highest in young to middle-aged adults. Onset in childhood should be considered suggestive of hereditary BN.12

Clinical

History

  • The onset of pain in brachial neuritis (BN) is often abrupt and may follow recent illness, surgery,13 immunization, or even trauma (see Causes, below). Up to two thirds of cases begin during the nighttime.
  • The pain usually is localized to the right shoulder region, but it may be bilateral in 10-30% of cases.
  • The pain's intensity is very high (9+/10) and is maximal at onset.
  • Usually, the pain is described as sharp or throbbing in nature.
  • The 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 from a few hours to several weeks and requires opiate analgesia.
  • Low-grade pain may persist for up to a year.
  • As the pain subsides, weakness becomes apparent.
    • In most cases of BN, 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.
  • Numbness may occur, depending on the particular nerves affected, and usually is found in the nerve distribution corresponding to 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.14,15,16
  • Variants of BN can present with isolated or multiple cranial neuropathies (IX, X, XI, XII).17
  • 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 with brachial neuritis 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 a 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 (in particular, loss of axillary nerve sensation), depending on the specific nerves affected.

Causes

The exact cause of brachial neuritis is unknown, but the condition has been linked to many antecedent events or illnesses, as follows:

  • Viral infection (particularly of the upper respiratory tract)
  • Bacterial infection (eg, pneumonia, diphtheria, typhoid)
  • Parasitic infestation
  • Surgery13
  • 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, 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, often bilateral attacks. Dysmorphic facial features (eg, hypotelorism, long nasal bridge, facial asymmetry) can also be present.

More on Brachial Neuritis

Overview: Brachial Neuritis
Differential Diagnoses & Workup: Brachial Neuritis
Treatment & Medication: Brachial Neuritis
Follow-up: Brachial Neuritis
Multimedia: Brachial Neuritis
References
Further Reading
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References

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Keywords

brachial neuritis, shoulder pain, plexus, neuritis, brachial plexus, brachial plexopathy, plexus neuritis, Parsonage-Turner syndrome, 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, serum neuritis, shoulder girdle neuritis, shoulder girdle syndrome

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Contributor Information and Disclosures

Author

Nigel L Ashworth, MBChB, MSc, FRCPC, Professor and Chief, Division of Physical Medicine and Rehabilitation, Glenrose Rehabilitation Hospital, University of Alberta
Nigel L Ashworth, MBChB, MSc, FRCPC 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
Disclosure: Nothing to disclose.

Medical Editor

Benjamin M Sucher, DO, FAAPMR, FAOCPMR, Medical Director, EMG Labs of AARA (Arizona Arthritis & Rheumatology Associates)
Benjamin M Sucher, DO, FAAPMR, FAOCPMR is a member of the following medical societies: American Academy of Physical Medicine and Rehabilitation, American Association of Neuromuscular and Electrodiagnostic Medicine, American Osteopathic Association, and American Osteopathic College of Physical Medicine and Rehabilitation
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Michael T Andary, MD, MS, Residency Program Director, Professor, Department of Physical Medicine and Rehabilitation, Michigan State University College of Osteopathic Medicine
Michael T Andary, MD, MS is a member of the following medical societies: American Academy of Physical Medicine and Rehabilitation, American Association of Neuromuscular and Electrodiagnostic Medicine, American Medical Association, and Association of Academic Physiatrists
Disclosure: allergan Honoraria Speaking and teaching; Pfizer Honoraria Speaking and teaching

CME Editor

Kelly L Allen, MD, Regional Medical Director, IMX-Medical Management Services
Disclosure: Nothing to disclose.

Chief Editor

Robert H Meier III, MD, Director, Amputee Services of America; Active Medical Staff, Presbyterian/St Luke's Hospital, Spalding Rehabilitation Hospital, Select Specialty Hospital; Consulting Staff, Kindred Hospital
Robert H Meier III, MD is a member of the following medical societies: American Academy of Physical Medicine and Rehabilitation and Association of Academic Physiatrists
Disclosure: Nothing to disclose.

 
 
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