Neuropsychological findings from older premutation carrier males and their noncarrier siblings from families with XXXX .

@entity21883 @entity409 @entity400 @entity179 @entity2100 @entity10308 @entity11583
OBJECTIVE: Carriers of the @entity10308 premutation allele are at a significantly increased risk for a late-onset @entity179 , @entity21883 ( @entity21883 ). The primary features of @entity21883 are late-onset intention @entity400 and @entity11583 . Previous reports have shown global deficits in neuropsychological measures among males with @entity21883 , particularly those related to executive functioning. The purpose of this study was to investigate the neuropsychological profile among older males with the premutation who are at risk for @entity21883 . METHOD: Premutation carriers, 66 with motor symptoms and 23 without, and 18 noncarrier siblings were recruited from pedigrees diagnosed with @entity2100 , all over age 50. Subjects were examined with a neurological test battery to identify symptoms of @entity21883 and a neuropsychological test battery to investigate cognitive and behavioral profiles. Linear regression and ANCOVA were used to determine the effect of the premutation on outcome measures adjusting for age and education. RESULTS: We identified a significant decrease in scores of general intelligence and a marginally significant decrease in scores of logical memory among premutation carrier males with motor symptoms compared to the noncarrier male siblings. We did not identify deficits in executive functioning in our sample of premutation carrier males with motor symptoms. CONCLUSIONS: Similar to other reports, we found that the @entity10308 premutation is associated with deficits in general intelligence and memory among older males with symptoms of @entity21883 . However, our results differed in that we found no evidence of premutation-associated @entity409 . We provide possible explanations for this difference.
