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Origin and Diffusion of the Myotonic Dystrophy Gene in the Saguenay Region (Quebec)

Published online by Cambridge University Press:  18 September 2015

Gérard Bouchard ,
Raymond Roy ,
Manon Declos ,
Jean Mathieu  and
Kevork Kouladjian
Gérard Bouchard*
Affiliation:
SOREP, University of Quebec at Chicoutimi
Raymond Roy
Affiliation:
SOREP, University of Quebec at Chicoutimi
Manon Declos
Affiliation:
Statistics Canada, Ottawa
Jean Mathieu
Affiliation:
Chicoutimi Hospital Muscular Dystrophy Clinic
Kevork Kouladjian
Affiliation:
SOREP, University of Quebec at Chicoutimi
*
SOREP, Université du Québec à Chicoutimi, 555 Boulevard de l'Université, Chicoutimi, Quebec, Canada G7H 2B1
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Abstract:

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A very high prevalence (approximately 1/475 in 1985) of myotonic dystrophy (Steinert disease) is observed in the Saguenay region, which is located in the north-east part of the Province of Quebec. For various reasons, however, the literature on the subject generally associates a high degree of selective disadvantage with this gene, which seems to contradict the Saguenay data. Using a computerized regional population register, we have reconstituted patients' genealogies and family biographies. We have thus been able to study the origin of the gene and to compare the demographic behavior of patients and controls. On the whole, patients seem to be very little disadvantaged compared to controls, in terms of reproduction as well as of geographical and occupational mobility.

Type
SPECIAL SUPPLEMENT Dystrophie myotonique au Québec
Information
Canadian Journal of Neurological Sciences , Volume 16 , Issue 1 , February 1989 , pp. 119 - 122
Copyright
Copyright © Canadian Neurological Sciences Federation 1989

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