Mizutani T, Maeda S, Hayakawa K, Tanaka U, Hirahata S, Kamoshita H, Taketani T, Morimatsu Y
Department of Clinical Pathology, Tokyo Metropolitan Institute of Gerontology, Japan.
Acta Neuropathol. 1988;77(2):206-12.
A case is described of paraneoplastic cortical cerebellar degeneration in a patient with a small cell carcinoma of the lung. Following therapy, clinical improvement of cerebellar ataxia had been observed. The most severe degeneration was found in the superior aspects of the vermis and in the anterior and simple lobes as well as in the inferior aspects of the hemisphere. In addition to this distribution of degenerative lesions, uneven loss of Purkinje cells was apparent. Such distribution patterns in this case were apparently compatible with those of alcoholic cortical cerebellar degeneration (ACD), although the lesions were less severe than in ACD. Furthermore, dendritic changes in the Purkinje cells including loss of the spiny branchlets, focal swelling of the dendrites, and disappearance of secondary and tertiary branches were remarkable. It is noteworthy that these cells showed various stages of degeneration before cell loss occurred. These data suggest that the degree of vulnerability varies among Purkinje cells, and that this could be related to the uneven loss of these cells. It is proposed that, although this case and cases of ACD have both similarities and differences in their neuropathological aspects, it is apparent that both conditions have some common morphopathogenetic factor.
本文描述了一例患有小细胞肺癌的患者发生副肿瘤性皮质小脑变性的病例。经过治疗后，观察到小脑共济失调有临床改善。最严重的变性见于蚓部上半、前叶和单纯叶以及半球下半。除了这种变性病变的分布外，浦肯野细胞的不均匀丢失也很明显。尽管该病例中的病变不如酒精性皮质小脑变性（ACD）严重，但这种分布模式显然与ACD的模式相符。此外，浦肯野细胞的树突变化显著，包括棘状小分支的丢失、树突的局灶性肿胀以及二级和三级分支的消失。值得注意的是，这些细胞在细胞丢失发生之前呈现出不同程度的变性阶段。这些数据表明，浦肯野细胞的易损程度各不相同，这可能与这些细胞的不均匀丢失有关。有人提出，尽管该病例与ACD病例在神经病理学方面既有相似之处也有不同之处，但显然这两种情况都有一些共同的形态病理发生因素。
