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Hereditary neuropathy with upper mlotor-neuron, visual pathway, and autonomic disorders

Department of Neurology, Albert Einstein College of Medicine, Bronx, NY.

A 42-year-old man had progressive distal weakness and muscle atrophy, stocking-type sensory loss, upper motor-neuron and visual pathway lesions, and dysautonomia. Electrodiagnostic tests revealed a generalized sensorimotor peripheral neuropathy that largely involved axons. Low recumbent and upright norepinephrine levels implied a peripheral autonomic defect. Sural nerve biopsy showed mild abnormalities of medium and small size fibers. The patient's mother and two brothers were also affected. Other causes of peripheral motor, sensory, and autonomic failure were eliminated. This kinship does not fit any generally accepted classification of hereditary neuropathies.

Address correspondence and reprint requests to Dr. Rechthand, Department of Neurology, Neuromuscular Unit, Johns Hopkins Hospital, Meyer 5–119, Baltimore, MD 21205.

Accepted for publication February 23, 1983.