It is difficult to tell how common HD is in a population. In Western countries it's estimated that about five to seven people per 100,000 are affected by HD (1). There are a few isolated populations in Western Europe where HD is unusually common.
A very high concentration of HD has also been found in the Lake Maracaibo region of Venezuela where the prevalence of HD is about 700 per 100,000 (2).
In Australia about 1,200 people now have HD and approximately 6,000 are at risk (3). The prevalence of HD in Tasmania is about 12 people per 100,000 (4).
A study released in 1999 (5) identified 380 people with definite HD in NSW in 1996, giving a prevalence of 6.29 per 100,000 population. The number of people with a 50% risk of inheriting the HD gene was estimated at 25.2 per 100,000 population.
1. S. E. Folstein Huntington's Disease. A Disorder
of Families Baltimore: Johns Hopkins University Press,
1989
2. R. Avila-Giron Medical and Social Aspects of
Huntington's Chorea in the State of Zulia, Venezuela
in: Advances in Neurology, Vol 1 (eds A. Barbeau,
T.N. Chase and G.W. Paulson) New York: Raven Press, 1973, pp.
261-266
3. P.M. Conneally Huntington's Disease: Genetics and
Epidemiology American Journal of Human Genetics
1984: 36, pp.506-526
4. S.A. Pridmore The Prevalence of Huntington's Disease
in Tasmania Medical Journal of Australia 1990:
153, pp.137-139
5. Elizabeth A. McCusker et al Prevalence of Huntington
Disease in New South Wales in 1996 Medical Journal of
Australia 2000: 173, pp.187-190
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