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Behavior in Huntington's Disease

Dissociating Cognition-Based and Mood-Based Changes

Received September 12, 2000; revised November 20, 2000; accepted December 12, 2000. From the Cerebral Function Unit, Greater Manchester Neuroscience Centre, Salford; and the Department of Clinical Genetics, St. Mary's Hospital, Manchester, UK. Address correspondence to Jennifer Thompson, Cerebral Function Unit, Greater Manchester Neuroscience Centre, Humphrey Booth Building, Hope Hospital, Stott Lane, Salford M6 8WH, United Kingdom. E-mail: jennifer.thompson{at}man.ac.uk

The authors examined the relationship of three dimensions of behavioral change (Apathy, Depression, and Irritability) measured by the Problem Behaviors Assessment for Huntington's Disease (PBA-HD) to cognitive and motor indices of disease severity. The Apathy subscale was highly correlated with both cognitive and motor impairment; the Irritability and Depression subscales were not. The findings suggest that certain behavioral alterations are intrinsic to the evolution and progression of HD, whereas others are more variable and are independent of other indices of disease progression.

Key Words: Huntington's Disease • Neuropsychiatric Disorders • Symptoms/Dimensions

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