Brachial neuritis

Nens van Alfen MD PhD (Dr. van Alfen of the Radboud University Nijmegen Medical Center received speaker fees from Ipsen as an ultrasound trainer.)
Raymond P Roos MD, editor. (Dr. Roos of the University of Chicago owns stock in Amgen, Best Doctors, Express Scripts, Isis, and Merck.)
Originally released May 14, 1996; last updated September 19, 2016; expires September 19, 2019

This article includes discussion of brachial neuritis, acute brachial neuropathy, acute brachial plexitis, acute brachial radiculitis, acute scapulohumeral paralysis, acute shoulder neuritis, brachial plexus neuropathy, cryptogenic brachial neuropathy, inflammatory brachial plexus neuropathy, localized neuritis of shoulder girdle, long thoracic neuropathy, multiple neuritis of shoulder girdle, neuralgic amyotrophy, paralytic brachial neuritis, Parsonage-Turner syndrome, and serratus magnus palsy. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.

Overview

Brachial neuritis, also known as neuralgic amyotrophy, has a characteristic clinical presentation with sudden onset of pain in the shoulder or arm region followed by weakness and atrophy of shoulder girdle or arm muscles. Clinical variants may include upper limb mononeuropathies, lumbosacral plexus involvement, phrenic neuropathy with diaphragm dysfunction, and concomitant recurrent laryngeal or accessory nerve involvement. These variants are important to recognize to avoid unnecessary work-up or interventions and to direct appropriate treatment. In this article, the author discusses typical and variant clinical features, diagnostic approach, and the role of immune therapy and targeted rehabilitation in patients with brachial neuritis.

Key points

 

• Brachial neuritis, also known as neuralgic amyotrophy or Parsonage-Turner syndrome, is clinically characterized in its classic form by acute onset of severe pain in the shoulder or arm, followed within days and weeks by weakness, reduced endurance, and wasting of affected muscles as well as variable sensory impairment due to involvement of the brachial plexus or its component nerves (van Eijk et al 2016).

 

• Although the exact pathophysiology is unclear, available evidence points to an autoimmune pathogenesis, superimposed on a mechanically-induced vulnerability of the nerves with an underlying genetic susceptibility.

 

• Diagnosis of the classic form is mainly clinical based on the typical history and symptoms, which usually include abnormal scapular posture and movement and signs of interosseus anterior nerve palsy. EMG can help localize the lesion to the brachial plexus and distinguish the syndrome from other multifocal peripheral neuropathies and cervical radiculopathy. Imaging with MRI or ultrasound can show multifocal nerve inflammation.

 

• A familial form of brachial neuritis, hereditary neuralgic amyotrophy, exists. Hereditary neuralgic amyotrophy accounts for about 10% of all brachial neuritis cases. It is clinically indistinguishable from idiopathic neuralgic amyotrophy, but with a median age of onset around 20 years of age whereas idiopathic neuralgic amyotrophy usually occurs around the age of 40 years, and recurrences tend to occur more often in hereditary neuralgic amyotrophy. In 30% to 50% of the families, it is associated with a mutation or duplication of the septin-9 (SEPT9) gene on chromosome 17q (van Alfen 2012).

 

• Management of acute brachial neuritis consists of pain control with strong analgesics (eg, long-acting opiods combined with a long-acting NSAID), adequate education of the patient to manage expectations, and, when tolerated, physical therapy to help preserve scapular control and prevent both disuse and overuse of the affected limbs.

 

• There is level 4 evidence for the benefit of corticosteroid or intravenous immunoglobulin treatment in the acute phase.

Historical note and terminology

Brachial neuritis, characterized by acute pain and weakness with variable atrophy and sensory loss around the shoulder girdle, is a fairly well-defined clinical entity. Typical cases were reported as early as the end of the 19th century (Dreschfeld 1886), when the condition was often described as "serratus palsy." During the Second World War, an increased incidence of brachial neuritis was encountered in the civil population as well as in army personnel (Spillane 1943; Parsonage and Turner 1948). A large number of reports during that period helped to establish the full clinical spectrum of the disease and also gave rise to a variety of synonyms for this syndrome: "multiple neuritis of shoulder girdle," "localized neuritis of shoulder girdle," "acute brachial radiculitis," "acute brachial plexitis," and "acute scapulohumeral paralysis" (Spillane 1943; Tsairis et al 1972). In their landmark paper, Parsonage and Turner analyzed 136 cases of brachial neuritis and noted that the essential clinical picture was simple but subject to modification (Parsonage and Turner 1948). They used the descriptive term "neuralgic amyotrophy" to avoid any assumptions about etiology or site of lesion. The syndrome received little attention in North American literature until Magee and Dejong reported 23 cases of paralytic brachial neuritis (Magee and Dejong 1960). Subsequently, Tsairis and colleagues reviewed a large series of patients with brachial neuritis to determine the natural history of the disease and advocated the term "brachial plexus neuropathy" (Tsairis et al 1972). Other reports have further refined the clinical features and phenotypic variability of both idiopathic neuralgic amyotrophy and hereditary neuralgic amyotrophy (Byrne 1987; Cruz-Martinez 2002; van Alfen 2006). The entity continues to be recognized by different terms; however, "neuralgic amyotrophy," "brachial neuritis," "brachial plexus neuropathy," or the eponym "Parsonage-Turner syndrome" are most commonly employed.

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