Yagishita S, Itoh Y, Iwabuchi K, Nakano T, Sakai H, Amano N
No To Shinkei. 1984 Jul;36(7):665-71.
A 46-year-old man developed sudden dysarthria and atactic gait and was noted to be unable to get up even on the bed about one year prior to his death. By following several days, he started to have scanning speech, nausea, trancal ataxia and dysmetria in succession. The cerebro-spinal fluid yielded moderate pleocytosis. There were no sensory disturbance, pathological reflexes and Romberg's sign. Half a year later, submandibular tumor was noted. The biopsy showed metastatic small cell undifferentiated carcinoma, presumably of pulmonary origin, and paraneoplastic cerebellar degeneration was suspected. He died of bronchopneumonia, superimposed on lung cancer on February 25 in 1979. The necropsy showed a large tumor in the right lung which was histologically verified small cell undifferentiated carcinoma (so-called oat-cell carcinoma). The cerebellum disclosed diffuse cortical atrophy, chiefly of Purkinje cell type. Moderate demyelination with reparative gliosis and foamy macrophages was seen in the white matter, which was considered secondary to cortical devastation. The morphometric study on Purkinje cell loss showed interesting distribution of the lesions. The severely affected portions were the central lobe and culmen in the vermis, and the ala lobuli centralis and quadrangular lobe in the hemisphere, respectively. The lingula was strikingly spared. The finding was compared with that of other cerebellar disease in reviewing the literature.
一名46岁男性在去世前约一年出现突发构音障碍和共济失调步态,甚至在床上也无法起身。数天后,他相继出现断续性言语、恶心、躯干性共济失调和辨距不良。脑脊液出现中度细胞增多。无感觉障碍、病理反射及闭目难立征。半年后发现下颌下肿物。活检显示为转移性小细胞未分化癌,推测起源于肺,怀疑为副肿瘤性小脑变性。他于1979年2月25日死于支气管肺炎,合并肺癌。尸检显示右肺有一个大肿瘤,组织学证实为小细胞未分化癌(所谓燕麦细胞癌)。小脑显示弥漫性皮质萎缩,主要为浦肯野细胞型。白质可见中度脱髓鞘伴修复性胶质增生和泡沫状巨噬细胞,认为是继发于皮质破坏。对浦肯野细胞丢失的形态计量学研究显示病变分布有趣。受累严重的部位分别是蚓部的中央叶和山顶,以及半球的中央小叶翼和四边形叶。舌叶明显未受累。在回顾文献时,将这一发现与其他小脑疾病的发现进行了比较。