PRESENILE-DEMENTIA WITH MOTOR NEURON DISEASE IN JAPAN - CLINICOPATHOLOGICAL REVIEW OF 26 CASES
PRESENILE-DEMENTIA WITH MOTOR NEURON DISEASE IN JAPAN - CLINICOPATHOLOGICAL REVIEW OF 26 CASES
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DOI：
10.1136/jnnp.47.9.953
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发表时间：
1984-01-01
影响因子：
11
通讯作者：
MITSUYAMA, Y
中科院分区：
文献类型：
--
作者：
MITSUYAMA, Y
本文回顾了日本26例伴运动神经元病的早老性痴呆的临床病理资料。特征性特征包括：在早老期缓慢进行性发作的进行性痴呆;在疾病过程中神经源性肌肉消耗;疾病至死亡的持续时间为1-3年;没有锥体外系症状和明确的感觉缺陷; CSF或EEG没有特征性异常;没有已知的家族发生的父母血缘关系;整个CNS的非特异性轻度退行性变化，无脑血管疾病或原发性退行性痴呆的证据，但存在运动神经元疾病的病理学发现。这是一个新的疾病实体的可能性建议。
The clinico-pathological findings of 26 cases of presenile dementia with motor neuron disease in Japan are reviewed. The characteristic features include: progressive dementia with slowly progressive onset in the presenile period; neurogenic muscular wasting during the course of illness; a duration of illness to death of from 1-3 yr; absence of extrapyramidal symptoms and definite sensory deficits; no characteristic abnormalities in the CSF or EEG; no known parental consanguinity of familial occurrence; 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.