Clinical characteristics of Huntington disease cohort.

*Chorea subscore 28 maximal, worst, dystonia subscore 20 maximal, worstThe values represent the mean±standard deviations. Ranges are in parentheses. Comparisons of means (ANOVA) were made among the 3 HD groups. UHDRS = Unified Huntington disease rating scale, 120 is the maximal score of severity, up to 4 is considered as normal. The HD group included 15 strictly presymptomatic carriers, 10 patients at an early stage of the disease and 7 mildly affected patients. Ages at examination did not differ significantly among groups; presymptomatic HD carriers were not younger than affected patients. Total functional score was close to maximal in all groups, even in mildly affected patients, in whom the mean TFC was greater than 10. However, the depression score was pathological, even in presymptomatic carriers, and worsened with disease progression.