Abstract

Numerous retrospective studies have reported the presence of psychiatric disorders at the prodromal or early stages of Huntington's disease (HD). However, most of the studies comparing gene carriers with non-carriers before the clinical manifestation of the illness have failed to reveal differences in the psychiatric manifestation. The objective of the present study was to detect behavioral and psychological features that differentiate gene carriers from non-carriers. Eighty-one Greek patients at 50% risk for HD were recruited prospectively and examined by means of a semi-structured interview and four self-rated questionnaires. The study focused predominantly on hostility/irritability and obsessional behavior. Gene carriers had significantly higher extroverted hostility than non-carriers (p = .005). The elevated level of hostility was unaffected by the proximity to the estimated age of onset. The remainder of the scales did not reveal significant differences. Extroverted hostility, in particular criticism of others and delusional hostility, is increased in gene carriers well before the onset of clinical HD. Hostility is regarded as a personality dimension rather than as a behavioral pattern.

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