Huntington's Chorea in South Wales A genetic and epidemiological study

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Abstract
A study of Huntington''s Chorea in South Wales, UK has shown a prevalence of 7.61/100,000 in the counties of Gwent and Glamorgan, with a total population of 1.7 million. Heterozygote frequency is close to 1 in 5000. Total ascertainment within this area was attempted; no patients evidently were omitted apart from those originating outside the area and those new cases with no living affected relatives. Analysis of migration patterns suggests that around 20% of cases in each generation arise from outside Glamorgan and Gwent and that around 12% of 1st-degree relatives of indigenous cases have left the area. The survey has formed the initial phase of a long-term prospective study of the disease involving systematic genetic counseling of all high-risk individuals and regular surveillance to monitor possible trends in future prevalence.