Epidemiology

The estimated prevalence of Huntington's disease is 5.65 per 100,000 in Europe and 7.43 per 100,000 in North America.[3]​ Estimated rates in Asia and Africa are significantly lower at 0.99 per 100,000 and 0.25 per 100,000, respectively.[3]​ In the US, an insurance database review reported the lowest prevalence among Asian people, but the prevalence was not significantly different between white and African-American people.[4]​ Data from the English and Welsh Huntington’s Disease Association suggest that the prevalence may be higher than previously estimated.[5] The overall incidence in one meta-analysis was 0.47 cases per 100,000 person-years.[3]

The disease affects men and women equally; typical onset is between the age of 30 and 50 years with a range of 2 to 85 years. The duration of disease is approximately 20 years from time of diagnosis to time of death.[6]

In isolated areas, such as Tasmania or Lake Maracaibo in Venezuela, a founder's effect can be seen, where an affected early settler can significantly increase the prevalence of Huntington's disease in a concentrated population.[7][8][9][10]

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