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Cognitive and Motor Functioning in Gene Carriers for Huntington's Disease

A Baseline Study

Received May 21, 2001; revised October 12, 2001; accepted December 14, 2001. From Leiden University Medical Center, Departments of Neurology, Clinical Genetics, Medical Statistics, and Human Genetics, Leiden; and the Department of Neuropsychology, Rijnland Hospital, Leiderdorp, the Netherlands. Address correspondence to M.N.W. Witjes-Ané, Leiden University Medical Center, Department of Neuropsychology J3-R, P.O. Box 9600, 2300 RC Leiden, the Netherlands. E-mail: m.n.w.witjes-ane{at}lumc.nl

The aim of this study was to investigate the first changes in cognitive and motor functioning in Huntington's disease. Forty-six gene carriers, not clinically diagnosed for HD, were compared with 88 non–gene carriers. Gene carriers performed significantly worse on the Benton Visual Retention Test. This result was due to a minority of participants who had already developed cognitive impairment. Marginal differences appeared on the motor times of single reaction time measures after correction for motor signs. The findings are discussed in the context of inconsistencies in previous studies and underscore the need for longitudinal research.

Key Words: Huntington's Disease • Asymptomatic Gene Carriers • Neuropsychological Assessment

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