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.