Mitsuyama Y
Department of Psychiatry, Miyazaki Medical College, Japan.
Dementia. 1993 May-Aug;4(3-4):137-42. doi: 10.1159/000107312.
Seventy-one Japanese cases of presenile dementia with motor neuron disease were reviewed. The clinico-pathological features were: (1) progressive dementia with insidious onset, mostly in the presenile period: (2) neurogenic muscular wasting in the course of illness (ALS- or SPMA-like symptoms); (3) duration from the onset of the illness to death: 2-5 years (average 30.6 months); (4) extrapyramidal symptoms and definite sensory deficits are less commonly present; (5) no characteristic abnormalities in the CSF or EEG; (6) no known consanguinity or familial occurrence; (7) non-specific mild to moderate degenerative changes in the fronto-temporal cerebral cortex, hypoglossal nuclei and spinal cord, and frequently in the substantia nigra. The author was interested in discovering whether the frequency and topology of lesions in the brain of patients with presenile dementia and motor neuron disease differed characteristically from the distribution found in cases of Alzheimer's disease, Pick's disease, Creutzfeldt-Jakob disease or progressive subcortical gliosis. Presenile dementia with motor neuron disease might be a new disease entity.
对71例患有运动神经元病的早老性痴呆日本病例进行了回顾。临床病理特征如下：(1) 隐匿起病的进行性痴呆，大多发生在早老期；(2) 病程中出现神经源性肌肉萎缩（肌萎缩侧索硬化症或脊髓性肌萎缩样症状）；(3) 从发病到死亡的病程：2至5年（平均30.6个月）；(4) 锥体外系症状和明确的感觉障碍较少见；(5) 脑脊液或脑电图无特征性异常；(6) 无已知的近亲结婚或家族性发病；(7) 额颞叶大脑皮质、舌下神经核和脊髓有非特异性轻度至中度退行性改变，黑质也常受累。作者感兴趣的是，患有早老性痴呆和运动神经元病的患者大脑中的病变频率和部位是否与阿尔茨海默病、匹克病、克雅氏病或进行性皮质下胶质增生症病例中的病变分布有特征性差异。患有运动神经元病的早老性痴呆可能是一种新的疾病实体。
