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Please be advised that this information was generated on 2016-09-18 and may be subject to change. Extrapolating the figures from a previous study on FSHD in a province of The Netherlands to the entire Dutch population suggests that at present a nearly complete overview is obtained of all symptomatic kin dred. In 139 families, dominant inheritance was observed in 97, a pat tern compatible with germline mosaicism in 6, while sporadic cases were found in 36 families. A mutation frequency of 9.6 % was calculated. Mentai retardation and severe retina! vasculopathy were reported in low frequencies (1%). Early onset was seen more frequently in sporadic cases. Chromosome 4 linkage appeared excluded in 3 of 22 autosomal-dominant families. The clinical pictures in the linked and nonlinked families were identical. © 1995 John Wiley & Sons, Inc. Key words: facioscapulohumeral muscular dystrophy • populatio