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The high frequency of juvenile Huntington's chorea in South Africa
  1. M R Hayden*,
  2. J M Macgregor,
  3. D S Saffer,
  4. P H Beighton*
  1. *The Department of Human Genetics, University of Cape Town, Medical School, Observatory 7925
  2. Department of Neurology, Groote Schuur Hospital, Observatory 7925
  3. Department of Neurology and Neurosurgery, Baragwanath Hospital and University of the Witwatersrand, Johannesburg, South Africa


    During a national investigation concerning all patients with Huntington's chorea in South Africa, 17 affected children, comprising 7·7% of the patients in the survey, were identified. Although the frequency of juvenile Huntington's chorea in the white community was equal to that reported from around the world, the frequency was much higher in the population of mixed ancestry. It is possible that this unique situation is related to the genetic constitution of this latter group.

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