Racial Differences in the Presentation and Progression of Huntington's Disease

Background: Huntington's disease (HD) is an autosomal dominant neurodegenerative disease that predominantly impacts a Caucasian population, but few efforts have explored racial differences in presentation and progression.

Objective: The aim was to assess the presentation and progression of HD across race groups using the Enroll-HD longitudinal observational study.

Methods: We applied propensity score matching for cytosine-adenine-guanine age product score, and age, to identify White, Hispanic, Asian, and Black participants from the Enroll-HD database. We compared clinical presentations at baseline, and progression over time, using White participants as a control cohort.

Results: Black participants were more severe at baseline across all clinical measures. No significant differences in progression were observed between race groups.

Conclusions: We consider the factors driving clinical differences at baseline for Black participants. Our data emphasize the necessary improvement in underrepresented minority recruitment for studies of rare diseases. © 2023 International Parkinson and Movement Disorder Society.