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Hereditary motor-sensory neuropathy (HMSN)

Possible X-linked dominant inheritance

Departments of Neurology (Drs. Phillips and Parker) and Pediatrics (Dr. Kelly and Ms. Schnatterly), University of Virginia Medical Center, Charlottesville, VA.

The inheritance of the hereditary motor and sensory neuropathies (HMSN) is usually autosomal dominant. We studied a kinship with a pattern of X-linked dominant inheritance. The phenotype was similar to HMSN of the "intermediate" type. Men were more severely affected than women, and hypertrophic nerves were not found. Nerve conduction was very slow in men, but it was mildly slow or normal in women. No male-to-male transmission was found in six generations.

Address correspondence and reprint requests to Dr. Phillips, Box 394, Department of Neurology, University of Virginia Medical Center, Charlottesville. VA 22908.

Presented in part at the thirty-fifth annual meeting of the American Academy of Neurology, San Diego, CA, April 1983.

Accepted for publication July 30, 1984.

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