Kurachi M, Koizumi T, Matsubara R, Isaki K, Oiwake H
Folia Psychiatr Neurol Jpn. 1979;33(2):205-15. doi: 10.1111/j.1440-1819.1979.tb00192.x.
Clinical and neuropathological findings are reported on a 48-year-old man who developed progressive bulbar palsy, muscle atrophy of four extremities, and hyperreflexia. Duration of the illness was about 19 months. Moderate personality changes were observed during his hospitalization. Neuropathological examination revealed the presence of an ALS: severe loss of the large motoneurons in the spinal anterior horns, and degeneration of the corticospinal tract, more prominent on the left side. The hypoglossal, the facial, and the motor trigeminal nuclei were also involved, but the oculomotor, the trochlear and the abducens nuclei were well preserved. There was bilateral, but more pronounced on the right, atrophy of the temporal poles consistent with the lesions of the temporal types of Pick's disease. This case indicates the simultaneous occurrence of degenerative diseases of the CNS, and the correspondence of laterality between the temporal lobe and the spinal cord may suggest a common etiology of these two types of disease processes.
报告了一名48岁男性的临床和神经病理学检查结果，该患者出现进行性延髓麻痹、四肢肌肉萎缩和反射亢进。病程约19个月。住院期间观察到中度人格改变。神经病理学检查显示存在肌萎缩侧索硬化：脊髓前角大型运动神经元严重缺失，皮质脊髓束变性，左侧更明显。舌下神经核、面神经核和运动三叉神经核也受累，但动眼神经核、滑车神经核和展神经核保存完好。双侧颞极萎缩，但右侧更明显，与Pick病颞叶型病变一致。该病例表明中枢神经系统退行性疾病同时发生，颞叶和脊髓之间的偏侧性对应可能提示这两种疾病过程有共同的病因。
