Early onset Cockayne's syndrome: case reports with neuropathological and fibroblast studies.

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Abstract
A survey of the mentally retarded children with an IQ between 30 and 55 born in a 10-year period (1955-64) and now of school age was carried out in New South Wales. The number of propositi who had a similarly affected sib of the same sex was ascertained; 58 boys had a similarly affected brother(s) and 22 girls had a similarly affected sister(s). It is suggested that the excess of affected brothers represents X-linked forms of mental retardation. An estimate of prevalence rate was calculated from the brother pair excess and was found to be 0·74/1000 males. The calculated incidence of X-linked forms of mental retardation appeared to account for most of the male excess found in the survey and suggests that 1 in every 5 of the mentally retarded boys in the IQ range in this survey may be retarded on the basis of genes on the X chromosome.