家族性肌萎缩性侧索硬化症,是运动神经元疾病中罕见一种类型的遗传病。1955年首先由Kurland报道。1983年国内首次谈氏报道3例。但对本病详细家系报道不多。本文将一家系患病2例报道如下。 1 病例与家系 例1 女性,50岁,工人。于1992年3月3日入院。1991年11月颈及左臂酸痛,日渐于该部出现进行性肌无力伴肌肉萎缩,头下垂,抬头乏力,需用颈托扶助支撑,发音含糊,张口困难,伸舌不灵活,不能进干食,进 Familial amyotrophic lateral sclerosis is a rare type of genetic disease in motor neuron disease. First reported by Kurland in 1955. In 1983, for the first time in China reported 3 cases. However, detailed reports of this disease are not many families. This article will be a family of 2 reported as follows. 1 cases and family cases 1 female, 50 years old, workers. March 3, 1992 admission. November 1991 neck and left arm pain, progressive in the Department of progressive muscle weakness with muscle atrophy, head drooping, fatigue, need to use neck support support, vague vocal, mouth opening difficulties, stretch the tongue inflexible, can not enter the dry Food, into