Black D N, Watters G V, Andermann E, Dumont C, Kabay M E, Kaplan P, Meagher-Villemure K, Michaud J, O'Gorman G, Reece E
Department of Neurogenetics, Montreal Neurological Institute, Quebec.
Ann Neurol. 1988 Oct;24(4):483-9. doi: 10.1002/ana.410240402.
We report a neurological disease among Cree Indian children in a northern Quebec village. The disease manifests as severe mental retardation, cerebral atrophy with white matter changes and calcifications, and systemic immunological abnormalities. Eleven cases are known in five families. The familial incidence of cases and the high degree of parental consanguinity suggest a genetic contribution. We propose that this entity may be caused by an unusual viral infection in a genetically vulnerable host.
我们报告了魁北克北部一个村庄克里印第安儿童中出现的一种神经疾病。该疾病表现为严重智力迟钝、伴有白质改变和钙化的脑萎缩以及全身免疫异常。已知五个家族中有11例病例。病例的家族发病率以及较高程度的父母近亲结婚表明存在遗传因素。我们认为这种病症可能是由基因易损宿主中一种不寻常的病毒感染引起的。
