The Prevalence of Huntington’s Disease

Background: Reviews of the epidemiology of Huntington’s disease (HD) suggest that its worldwide prevalence varies widely. This review was undertaken to confirm these observations, to assess the extent to which differences in caseascertainment and/or diagnosis might be responsible, and to investigate whether the prevalence pattern has changed over the past 50 years. Methods: Eighty two relevant studies were identified from Medline and Embase, previous reviews, scrutiny of references from included and excluded studies and enquiry among those interested in the field. Results: The lowest rates were among the Asians and the highest among the Caucasians. The differences are not fully explained by varying approaches to case-ascertainment or diagnosis. There was evidence of an increasing prevalence of between 15 and 20% per decade in studies from Australia, North America and Western Europe. Conclusions: The prevalence of HD varies more than tenfold between different geographical regions. This variation can in part be attributed Received: September 11, 2015 Accepted: December 27, 2015 Published online: January 30, 2016 Michael D. Rawlins Department of Non-Communicable Disease Epidemiology London School of Hygiene and Tropical Medicine Keppel St., London WC1E 7HT (UK) E-Mail michael.rawlins @ nice.org.uk © 2016 S. Karger AG, Basel 0251–5350/16/0462–0144$39.50/0