The Prevalence of Huntington's Disease

Abstract

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 case-ascertainment 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 to differences in case-ascertainment and/or diagnostic criteria, but there is consistent evidence of a lower incidence in Asian populations. There is also evidence that in Australia, North America and in Western Europe (including the United Kingdom), prevalence has increased over the past 50 plus years.

Two Ethnic Clusters with Huntington Disease in Israel: The Case of Mountain Jews and Karaites

“…The high proportion of Mountain Jews among our HD patients is in striking contrast to epidemiological studies on HD in Asia. Recent meta-analysis studies on the incidence and prevalence of HD detected higher prevalence rates in North America, Europe, and Australia (5.7 per 100,000) compared to Asia and Africa (0.4 per 100,000) [22,23]. This is in part due to the difference in CAG tract size and HTT haplotypes between the populations, with the average CAG tract size in Europeans significantly larger than that of Asians and Africans [24].…”

Implication of Epigenetic Modifications in Neurodegenerative Disorders: Traces and Imprints

“…The prevalence of this disease shows a tenfold variation across various geographic regions of the world [23]. Generally, this disease initiates in the middle years ranging from 35-45 years although juvenile cases [24] of the same have been reported (Figure 1).…”

Genetic Modifiers of CAG.CTG Repeat Instability in Huntington's Disease Mouse Models

“…The estimation of prevalence varies according to haplogroups studied: it is estimated from 2 to 7 per 100,000 in the Caucasians and only 0.1-1 per 100,000 in Asians and Africans [5,6]. Adult-onset Huntington disease is the most common form of HD and usually presents in early middle life.…”