肌萎缩侧索硬化已被确定有三种主要类型:散发或经典型、关岛人型、家族性和假定的遗传型。家族性肌萎缩侧索硬化在临床、病理及遗传许多方面存在广泛的变异。例如病程,在某些家族不到一年,但在另一些家族可超过20年。许多家族一致显示锥体束征,但另一些家族却无。病理改变也不相同,从局限于前角细胞到锥体束、后柱、背核和脊髓小脑束受累。多数系常染色体显性传递,但也有疑为常染色体隐性遗传者。作者报导14个家族,每个家族至少两个成员患有进行性运动神经元病。诊断标准是病人表现以下运动神经元体征为主的进行性运动功能障碍,如肌 Amyotrophic lateral sclerosis has been identified as having three main types: sporadic or classic, Guam human, familial and putative genotypes. Familial amyotrophic lateral sclerosis has a wide range of clinical, pathological and genetic variants. For example, the course of disease, in some families less than a year, but in other families can be more than 20 years. Many families consistently show pyramidal tract signs, but others do not. Pathological changes are not the same, from the confined anterior horn cells to the pyramidal tract, posterior column, dorsal horn and spinocerebellar tract involvement. Most of the Department of autosomal dominant transmission, but there are also suspected autosomal recessive. The authors report 14 families, with at least two members of each family having progressive motor neuron disease. The diagnostic criteria are progressive motor dysfunction in which the patient exhibits predominantly motor neuron signs such as muscular