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Hereditary Neuropathy With Liability to Pressure Palsies

By Michael Rubin, MDCM, Weill Cornell Medical College;New York Presbyterian Hospital-Cornell Medical Center

Hereditary neuropathy with liability to pressure palsies is a hereditary disorder in which nerves become increasingly sensitive to pressure, injury, and use.

In this neuropathy, nerves are susceptible to damage resulting from relatively slight pressure or injury or from repetitive use. Usually, this neuropathy starts during adolescence or young adulthood, but it may start at any age. It affects both sexes equally. It is inherited as an autosomal (not sex-linked) dominant trait. That is, only one gene from one parent is required for the disease to develop (see Dominant disorders).

Peroneal nerve palsy with footdrop, ulnar nerve palsy, and carpal tunnel syndrome (see Carpal Tunnel Syndrome) commonly develop. Numbness or weakness occurs periodically in the affected area. Symptoms vary from unnoticeable and mild to severe and incapacitating. Episodes may last several minutes to months. After an episode, about half of affected people recover completely, and most of the rest have mild symptoms.

Doctors may have difficulty diagnosing this neuropathy because the symptoms come and go. Electromyography and genetic testing help establish the diagnosis. Rarely, biopsy of a nerve is required.

Activities that cause symptoms should be avoided or modified. Supports, such as wrist splints and elbow pads, can reduce pressure, prevent reinjury, and allow the nerve to repair itself over time. Rarely, surgery is needed.

* This is the Consumer Version. *