Benign hereditary (dominant) chorea of early onset.
Two siblings (one girl 7 1/2 and one boy 6 1/2) are described with a choreic syndrome dating from early childhood without other neurological abnormalities. The father was similarly affected but has markedly improved. Extensive neurological work-up and family history permitted to rule out numerous conditions known to cause abnormal movements in childhood. The clinical entity corresponds to the recently described syndrome of hereditary benign non progressive chorea. To our knowledge it is the first described family with this entity in Switzerland. The relevant literature is reviewed particularly as it pertains to the genetic unity of the syndrome. Its importance lies in its differentiation from the other more severe hereditary abnormal movements of childhood, its non progression and tendency to improve with time. The possible reasons for the only very recent recognition of this purely clinical syndrome are also discussed.
D000328:Adult; D002648:Child; D005260:Female; D005799:Genes, Dominant; D006801:Humans; D006816:Huntington Disease / Q000235:genetics*; D008297:Male; D010375:Pedigree; D013557:Switzerland