Mortality trends and disparities in adults with Huntington's disease in the United States.

Background: Huntington's disease (HD), an autosomal dominant disorder, is characterized by progressive neurodegeneration, psychiatric issues, dementia, and worsening chorea over time. Its prevalence varies by ethnicity and region.

Objective: This study aims to analyze mortality trends and disparities in adults with HD in the United States (US).

Methods: This study analyzed death certificates from 1999 to 2020 for deaths due to HD (ICD-10 code G10) in individuals aged 25 and older. Age-adjusted mortality rates (AAMRs) and annual percent change (APC) were calculated by year, gender, age groups, race/ethnicity, geographics and urbanization status.

Results: Between 1999 and 2020, there were 24,121 reported deaths among patients with HD. During this period, the AAMR increased from 4.3 to 6.0 per 1,000,000 population, with a notable surge from 2018 to 2020 (APC: 9.88; 95% CI: 5.45 to 13.20). Older adults exhibited the highest AAMRs at 10.4 per 1,000,000 when analyzed by age-group. Men and women had comparable AAMRs (5.2 vs. 5.0). By race, non-Hispanic (NH) Whites had the highest AAMRs (6.0), followed by NH African Americans (3.3) and Hispanics (2.8). Additionally, non-metropolitan areas experienced higher AAMRs compared to metropolitan areas (6.6 vs. 4.8).

Conclusions: Since 1999, mortality from HD has increased, particularly from 2018 to 2020, with higher rates in older adults, men, NH Whites, and non-metropolitan areas. Further research is essential to consolidate data, standardize reporting practices, and address disparities to improve outcomes.