GENETIC EPIDEMIOLOGY OF CONGENITAL MUSCULAR-DYSTROPHY IN A SAMPLE FROM NORTHEAST ITALY

Congenital muscular dystrophy (CMD) is a heterogenous disease with aut osomic recessive transmission. In an epidemiological study in four pro vinces of Veneto (region of 2586830 inhabitants in north-east Italy), the recorded incidence rate for the period 1979-1993 was 4.65 x 10(-5) ; the prevalence rate in the year 1993 was 6.8 x 10(-6). The incidence and the prevalence rates that we have obtained during the course of o ur investigation represent the first estimates for CMD in Europe and s how that this myopathy is among the most frequent neuromuscular diseas es with autosomic recessive transmission.