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

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.

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

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.

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).

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.