title: Genealogical reconstruction of myotonic dystrophy in the Saguenay-Lac-Saint-Jean area (Quebec, Canada).
abstract: The prevalence of myotonic dystrophy (MyD) in the Saguenay-Lac-Saint-Jean (SLSJ) region (Quebec, Canada) is 30 to 60 times the world's prevalence. We identified 746 patients (673 still alive) distributed in 88 families. Using a population-based register of the SLSJ area and several marriage repositories from northeastern Quebec, we could trace back all patients to a couple who settled in "Nouvelle-France" in 1657. The MyD gene was then passed on over 10 to 14 generations. This genealogical reconstruction is a strong argument in favor of the genetic homogeneity of MyD in the SLSJ region.
authors.affiliation: Clinique des maladies neuro-musculaires, Hôpital de Chicoutimi, PQ, Canada.
authors.forename: J
authors.initials: J
authors.lastname: Mathieu
authors.forename: M
authors.initials: M
authors.lastname: De Braekeleer
authors.forename: C
authors.initials: C
authors.lastname: Prévost
mesh_terms: D005260:Female; D006801:Humans; D008297:Male; D009223:Myotonic Dystrophy / Q000453:epidemiology / Q000235:genetics*; D010375:Pedigree; D015995:Prevalence; D011792:Quebec / Q000453:epidemiology; D011795:Surveys and Questionnaires