Neuralgic amyotrophy.

Abstract

This review focuses on the current insights and developments in neuralgic amyotrophy (NA), an auto-immune multifocal peripheral nervous system disorder that leaves many patients permanently impaired if not recognized and treated properly. NA is not as rare as previously thought. The phenotype is broad, and recent nerve imaging developments suggest that NA is the most common cause of acute anterior or posterior interosseous nerve palsy. Phrenic nerve involvement occurs in 8% of all NA patients, often with debilitating consequences. Acute phase treatment of NA with steroids or i.v. immunoglobulin may benefit patients. Long-term consequences are the rule, and persisting symptoms are mainly caused by a combination of decreased endurance in the affected nerves and an altered posture and movement pattern, not by the axonal damage itself. Patients benefit from specific rehabilitation treatment. For nerves that do not recover, surgery may be an option. NA is not uncommon, and has a long-term impact on patients' well-being. Early immunomodulating treatment, and identifying phrenic neuropathy or complete nerve paralysis is important for optimal recovery. For persistent symptoms a specific treatment strategy aiming at regaining an energy balance and well-coordinated scapular movement are paramount.