Retrospective mortality analysis of hereditary ataxia in the United States (2000–2020) and forecast to 2050 using ARIMA

Hereditary ataxias are rare, life-limiting disorders of the cerebellum. Contemporary, population-level mortality patterns in the United States remain incompletely characterized. We quantified national trends in mortality associated with hereditary ataxias and examined differences by sex, race, census region, and urbanization. We analyzed 2000–2020 U.S. mortality data from CDC WONDER (Multiple Cause of Death). Cases were identified by ICD-10 codes G11 for hereditary ataxias. Age-adjusted mortality rates (AAMRs) were calculated per 100,000 using the 2000 U.S. standard population with 95% CIs. Trends were assessed with joinpoint regression to estimate annual percent change (APC). Forecast until 2050 was assessed through ARIMA with sensitivity analysis using linear regression. National AAMR increased significantly across 2000–2020. Increases were observed in both sexes, with persistently higher rates among males. Relative increases were largest among Black or African American individuals and in the West census region; patterns were directionally consistent across urbanization categories and increase in mortality was observed in forecast also. Sensitivity analyses yielded qualitatively similar trends, with wider uncertainty in strata. Mortality associated with hereditary ataxias rose in the United States from 2000 to 2020, underscoring growing clinical and public health needs in cerebellar disease. Improved phenotyping and coding, earlier diagnosis, and registry linked surveillance could refine estimates and inform service planning.