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X-linked recessive inheritance of ataxia and adult-onset dementia

Clinical features and preliminary linkage analysis

Departments of Neurology (Drs. Farlow and DeMyer) and Medical Genetics (Drs. Dlouhy and Hodes), Indiana University School of Medicine, Indianapolis, IN.

Three generations of a family exhibit a unique syndrome of X-linked ataxia, pyramidal tract signs, and adult-onset dementia. Initial signs, manifested by 2 to 3 years of age, are delayed walking and tremor. During their teens, the patients develop mild but progressive ataxia and pyramidal tract signs. Memory problems in the third decade initiate a progressive dementia, leading to death in the sixth decade. Laboratory investigations failed to disclose a biochemical basis for the syndrome. Preliminary molecular linkage studies have been conducted, and although the specific position of the responsible gene on the X chromosome has not yet been determined, the q26-qter region and much of the p arm are unlikely sites for this gene. The linkage studies are continuing.

Address correspondence and reprint requests to Dr. Farlow, Department of Neurology, 545 Barnhill Drive ~ EM 125, Indiana University School of Medicine, Indianapolis, IN 46223.

Received April 10, 1986. Accepted for publication in final form August 5, 1986.

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