Neuralgic amyotrophy. An update.

A century after the first description of neuralgic amyotrophy (NA), its pathophysiology remains unknown. An inflammatory (auto)immune pathophysiology is presumed, with mechanical or infectious precipitating conditions, which triggers attacks. Clinically, NA is an acute and painful unique or multiple mononeuropathy that causes palsy, amyotrophy and sensory loss in an asymmetric and patchy distribution. It involves the upper brachial plexus rather than the other parts but also may involve the cervical plexus, lumbosacral plexus and cranial nerves. The impairment can be restricted to one fascicule of one nerve, plexus or root; limited to a few ones; or extensive, involving both upper limbs. Its evolution is usually monophasic and auto-limited and never leads to generalized polyneuropathy. Electrodiagnostically, NA is characterized by severe axonal damage. The recovery is usually good after 6 months to 3 years in 80% of cases. Persistent disability is present in 20% of idiopathic NA cases and is more frequent in hereditary NA, with frequent recurrences, more frequent bilateral impairment, and more atypical distribution (cervical plexus, lumbosacral plexus or cranial nerves) than with idiopathic NA. Hereditary NA is mainly linked to a mutation in the gene of the Septin-9 protein. When the patient is seen early after disease onset, treatment with corticosteroids for 2 weeks seems to shorten the pain duration and the delayed recovery. With diagnosis during the palsy period, treatment is based on pharmacologic and non-pharmacologic therapies according to the complaints of the patient.