The Prevalence of Huntington's Disease

Area of Science:

• Neurology
• Epidemiology
• Genetics

Background:

• Huntington's disease (HD) epidemiology shows wide global prevalence variations.
• Previous reviews suggest significant differences in worldwide HD occurrence.

Purpose of the Study:

• Confirm observed variations in Huntington's disease prevalence.
• Assess the role of case-ascertainment and diagnostic criteria in prevalence differences.
• Investigate changes in Huntington's disease prevalence over the past 50 years.

Main Methods:

• Conducted a systematic review of 82 relevant studies.
• Searched Medline, Embase, and reference lists of identified studies.
• Gathered information through expert inquiry.

Main Results:

• Prevalence rates were lowest among Asian populations and highest among Caucasians.
• Differences in case-ascertainment or diagnosis do not fully explain the observed prevalence variations.
• Evidence indicates a 15-20% per decade increase in prevalence in Australia, North America, and Western Europe.

Conclusions:

• Huntington's disease prevalence differs more than tenfold across geographical regions.
• Case-ascertainment and diagnostic criteria partially explain prevalence variations, with consistently lower incidence in Asian populations.
• Prevalence of Huntington's disease has increased in Australia, North America, and Western Europe over the past 50+ years.