Abstract
Objective
Juvenile Huntington's Disease (JHD) is an extremely rare autosomal dominant neurodegenerative disease with onset in childhood or teenage years. Although there are many similarities with the adult form of the disease, JHD has a clinically distinct presentation. Some common symptoms include behavioral problems and cognitive decline. However, given the rarity of juvenile presentations, limited research exists regarding sex difference performances on executive and intellectual functioning in a JHD population. Thus, exploratory research was conducted to investigate such findings.
Method
Data from the Kids HD research study was analyzed (N = 58; mean age = 15.5, 50.8% female). Preliminary bivariate partial correlations, independent t-tests, and one way ANOVA tests were used to examine differences in executive and intellectual functioning between male and female participants.
Results
Performance significantly differed on several aspects of executive and intellectual functioning between sexes, including shifting attention (p = 0.007), verbal intellect (p = 0.019), and general ability intellect (p = 0.041). Significant differences were also observed regarding inhibition (p = 0.003), verbal categorical fluency (p = 0.021), and sorting (p = < 0.001).
Conclusions
Results suggest that there are significant differences in executive and intellectual functioning between sexes. In particular, males had more difficulty shifting attention, despite higher verbal intellect and higher general ability intellect. Females demonstrated greater inhibition, while males demonstrated stronger verbal categorical fluency and abstract reasoning. Between sexes, results indicate evidence of performance differences across tasks of executive and intellectual functioning. Such findings are consistent with a subcortical neurodegenerative process, such as HD.
MRI findings in juvenile Huntington's disease
