The epidemiology of Huntington's disease - PubMed (original) (raw)

Review

The epidemiology of Huntington's disease

P S Harper. Hum Genet. 1992 Jun.

Abstract

The available information on the world distribution of Huntington's disease (HD) from population surveys and death rate analysis is summarised and discussed in the light of genetic studies. It is concluded that most European populations, both Northern and Southern, show a relatively high prevalence (4-8 per 100,000), and that the disorder may also be frequent in India and parts of central Asia. HD is notably rare in Finland and in Japan, but data for Eastern Asia and Africa are inadequate. The disorder may have been underestimated in the American black population. Populations derived from recent European immigration show frequencies and origins of HD comparable to those expected from their own origins and expansion; there is no evidence to suggest that the HD gene has spread disproportionally and its selective effect may be close to neutral. Multiple separate introductions of the gene have been the rule in large populations. Several major foci of HD exist as the result of rapid population expansion. It is likely that a number of separate mutations for HD will be shown to be responsible for the disease, but that the high frequency of HD in European populations will prove to be the result of one or a very small number of mutations, probably of great antiquity.

PubMed Disclaimer

Similar articles

Cited by

References

    1. Nervenarzt. 1987 Jul;58(7):424-7 - PubMed
    1. Scott Med J. 1967 Apr;12(4):152-6 - PubMed
    1. S Afr Med J. 1988 Apr 16;73(8):489-94 - PubMed
    1. East Afr Med J. 1956 Jun;33(6):209-23 - PubMed
    1. Brain. 1967 Mar;90(1):203-32 - PubMed

Publication types

MeSH terms

LinkOut - more resources