Mitsuyama Y, Takamiya S
Arch Neurol. 1979 Sep;36(9):592-3. doi: 10.1001/archneur.1979.00500450086021.
A 61-year-old woman suffered the gradual onset of difficulty with memory, concentration, and cognition at age 58. Progressively more severe dementia was accompanied by muscle wasting and fasciculation prominent in hand and bulbar muscles. An electromyogram and a muscle biopsy specimen demonstrated denervation patterns, and a computerized tomographic scan showed considerable cerebral atrophy. This report reviews cases of presenile dementia with motor neuron disease reported in Japan and discusses the possibility of a new clinicopathologic entity.
一名61岁女性在58岁时逐渐出现记忆、注意力和认知方面的困难。逐渐加重的痴呆伴有肌肉萎缩和手部及延髓肌肉明显的肌束震颤。肌电图和肌肉活检标本显示有去神经支配模式，计算机断层扫描显示有明显的脑萎缩。本报告回顾了日本报道的早老性痴呆伴运动神经元病的病例，并讨论了一种新的临床病理实体的可能性。
