Abstract

Background

Despite extensive research regarding the etiology of Huntington’s disease, relatively little is known about the epidemiology of this rare disorder, particularly in the United States where there are no national‐scale estimates of the disease.

Objectives

To provide national‐scale estimates of Huntington’s disease in a U.S. population and to test whether disease rates are increasing, and whether frequency varies by race, ethnicity, or other factors.

Methods

Using an insurance database of over 67 million enrollees, we retrospectively identified a cohort of 3,707 individuals diagnosed with Huntington’s disease between 2003 and 2016. We estimated annual incidence, annual diagnostic frequency, and tested for trends over time and differences in diagnostic frequency by sociodemographic characteristics.

Results

During the observation period, the age‐adjusted cumulative incidence rate was1.22 per 100,000 persons (95% confidence interval: 1.53, 1.65), and age‐adjusted diagnostic frequency was 6.52 per 100,000 persons (95% confidence interval: 5.31, 5.66); both rates remained relatively stable over the 14‐year period. We identified several previously unreported differences in Huntington’s disease frequency by self‐reported sex, income, and race/ethnicity. However, racial/ethnic differences were of lower magnitude than have previously been reported in other country‐level studies.

Conclusions

In these large‐scale estimates of U.S. Huntington’s disease epidemiology, we found stable disease frequency rates that varied by several sociodemographic factors. These findings suggest that disease patterns may be more driven by social or environmental factors than has previously been appreciated. Results further demonstrate the potential utility of administrative Big Data in rare disease epidemiology when other data sources are unavailable. © 2019 International Parkinson and Movement Disorder Society

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