[Myotonic dystrophy: II. Marital status, fertility and gene transmission]

A sociological study, using a representative sample (N = 218) of the myotonic dystrophy population, made possible an analysis of the relationship between certain demographic characteristics (marriage, fertility) and the mechanisms involved in the transmission of the deleterious gene. The results show a clear differentiation between the marriage rate of women and men affected by myotonic dystrophy. Whereas men show a considerable decline in marriage eligibility, women continue to marry at a young age and in a proportion almost equal to that of the unaffected population. The study also indicates that the average fertility rate among married patients in the Saguenay-Lac-Saint-Jean exceeds the fertility rate reported from affected populations found in other countries. Our study shows an above-normal male fertility rate. This demographic fact explains the overrepresentation of male transmitters noted in the affected population. However, if present trends remain unchanged, women will be more likely to transmit myotonic dystrophy to future generations. These conclusions illustrate how the transmission and spread of a dominant gene follow a pattern that cannot be dissociated from the socio-cultural characteristics taken as a whole, particularly demographic characteristics. They also provide us with useful avenues for setting up future prevention programmes.