Trends and Disparities in Mortality from Hereditary Ataxia in United States, 2000-2020: A Retrospective Analysis with...

Hereditary ataxias are rare cerebellar disorders. Population level mortality patterns in the United States in the previous decades remain insufficiently described. We quantified national mortality trends associated with hereditary ataxias and described stratified trends by sex, race, census region, and urbanization. We analyzed U.S. Multiple Cause of Death data from CDC WONDER for 2000 to 2020. Decedents were identified using ICD-10 code G11 for hereditary ataxias. Age-adjusted mortality rates (AAMRs) per 100,000 were computed using the 2000 U.S. standard population, with 95% confidence intervals. Temporal trends were evaluated using joinpoint regression to estimate annual percent change (APC). Forecasts through 2050 were generated using ARIMA, with sensitivity analysis using linear regression. National AAMR increased significantly from 2000 to 2020. Rising rates were observed in both sexes, with consistently higher mortality among males. The largest relative increases occurred among Black or African American individuals and in the West census region. Trends were directionally consistent across urbanization categories, and the forecast analyses also indicated increasing overall mortality. Sensitivity analyses produced qualitatively similar patterns, with wider uncertainty within strata. Mortality associated with hereditary ataxias increased in the United States between 2000 and 2020, highlighting growing clinical and public health needs related to cerebellar disease. Improved phenotyping and coding, earlier diagnosis, and registry-linked surveillance may refine estimates and support service planning.