The high frequency of juvenile Huntington's chorea in South Africa

Abstract

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.