Mitsuyama Y
J Neurol Neurosurg Psychiatry. 1984 Sep;47(9):953-9. doi: 10.1136/jnnp.47.9.953.
The clinico-pathological findings of 26 cases of presenile dementia with motor neuron disease in Japan are reviewed. The characteristic features include: (1) Progressive dementia with slowly progressive onset in the presenile period. (2) Neurogenic muscular wasting during the course of illness. (3) A duration of illness to death of from one to three years. (4) Absence of extrapyramidal symptoms and definite sensory deficits. (5) No characteristic abnormalities in the CSF or EEG. (6) No known parental consanguinity of familial occurrence. (7) Non-specific mild degenerative changes throughout the CNS without evidence of cerebrovascular disease or primary degenerative dementia, but with the presence of pathological findings of motor neuron disease. The possibility that this is a new disease entity is suggested.
本文回顾了日本26例伴有运动神经元病的早老性痴呆的临床病理研究结果。其特征包括：（1）早老期起病缓慢且呈进行性发展的痴呆。（2）病程中出现神经源性肌肉萎缩。（3）病程至死亡为1至3年。（4）无锥体外系症状及明确的感觉障碍。（5）脑脊液及脑电图无特征性异常。（6）家族性发病中无已知的父母近亲婚配情况。（7）整个中枢神经系统有非特异性轻度退行性改变，无脑血管病或原发性退行性痴呆的证据，但有运动神经元病的病理表现。提示这可能是一种新的疾病实体。
