Investigations on Huntington's disease in the Canadian Prairies

I. Prevalence

Authors

  • M. H. K. Shokeir

    Corresponding author
    1. Division of Genetics, Department of Paediatrics, University of Manitoba, and Health Sciences Children's Centre, Winnipeg, Canada
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  • 1

    Queen Elizabeth II Scientist.

3 Health Sciences Children's Centre 685 Bannatyne Avenue Winnipeg, Manitoba Canada R3E OW1

Abstract

One hundred and sixty-two confirmed cases of Huntington's disease have been ascertained in the provinces of Saskatchewan and Manitoba in the Canadian Prairies. They were encountered in 97 sibships which were derived from 58 kindreds. The prevalence of the disorder was estimated to be 8.4 per 100,000 individuals. Taking into account the mean age of onset of the disorder, 23.3 per 100,000 individuals are expected to be heterozygous for the Huntington's disease allele, the frequency of which is thus computed to be 0.0001168.

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