Longitudinal personality changes among presymptomatic Huntington disease gene carriers.

OBJECTIVE

To determine whether longitudinal changes in personality as measured by the Minnesota Multiphasic Personality Inventory (MMPI) can be detected among clinically presymptomatic individuals carrying the expanded Huntington disease (HD) allele.

BACKGROUND

Emotional symptoms are considered one of the cardinal features of HD. However, the literature is replete with conflicting reports of psychiatric symptoms in presymptomatic HD gene carriers.

METHODS

A longitudinal, case-control, double-blind study comparing presymptomatic gene carriers and nongene carriers at risk for HD evaluated with an abbreviated MMPI and a quantified neurologic rating scale examined an average of 3.7 years apart.

RESULTS

Presymptomatic gene carriers (PSGC) had a greater increase in abnormality over time for the MMPI scales, cynical hostility (repeated-measures ANOVA, = 0.04) and irritability (repeated measures ANOVA, = 0.005), when compared with the nongene carriers (NGC). Among both the PSGCs and NGCs, no significant correlation was found between the number of CAG repeats and the change in MMPI score between visits.

CONCLUSIONS

This study provides significant evidence for increasing irritability and cynical hostility in presymptomatic gene carriers before the onset of overt clinical symptoms.