Tremblay-Tymczuk S, Mathieu J, Morgan K, Bouchard J P, De Braekeleer M
Département des sciences humaines, Université du Québec, Chicoutimi, Canada.
Rev Neurol (Paris). 1992;148(10):601-4.
The genealogies of 28 proband affected with oculopharyngeal dystrophy (OPD) distributed in 28 families from Saguenay-Lac-St-Jean, a geographically isolated region of northeastern Quebec, were reconstructed. The analysis of these genealogies showed that the Montmagny and L'Islet counties are the most likely center of diffusion of OPD among the French Canadians, as suggested by Barbeau in 1966. A unique founder couple, J.E.-M.B., common to all 28 genealogies was found. The children of this couple emigrated in the 17th century from Poitou (France), a region where several families with OPD were recently ascertained.
重建了来自魁北克东北部地理上孤立的萨格奈-拉克-圣让地区28个家庭中28名患有眼咽型肌营养不良症（OPD）的先证者的家系。对这些家系的分析表明，正如1966年巴博所指出的，蒙特马尼县和伊勒县最有可能是OPD在法裔加拿大人中传播的中心。发现了所有28个家系共有的一对独特的奠基夫妇J.E.-M.B.。这对夫妇的子女于17世纪从法国普瓦图地区移民而来，最近在该地区发现了几个患有OPD的家庭。
