Nakano R, Sato S
Department of Neurology, Niigata University.
Nihon Rinsho. 1994 Nov;52(11):3000-5.
Paraneoplastic cerebellar degeneration (PCD) is a rare manifestation of cancer, characterized clinically by subacute progressive ataxia, dysarthria and nystagmus. The pathological hallmark of PCD is a severe, diffuse loss of Purkinje cells. PCD occurs most frequently in association with small cell carcinoma of the lung and adenocarcinoma of the ovary, but it has also developed in patients with carcinoma of the breast, malignant lymphoma, and various cancers. Autoantibodies against cerebellar Purkinje cells have been frequently observed in the serum or cerebrospinal fluid (CSF) from patients with PCD. The cause of PCD is unknown, but the presence of these autoantibodies in some patients suggests that the pathogenesis may be immune mediated. The potential role of the autoantibody in the pathogenesis of PCD is discussed.
副肿瘤性小脑变性（PCD）是癌症的一种罕见表现，临床特征为亚急性进行性共济失调、构音障碍和眼球震颤。PCD的病理标志是浦肯野细胞严重弥漫性丧失。PCD最常与肺癌小细胞癌和卵巢腺癌相关，但也见于乳腺癌、恶性淋巴瘤及各种癌症患者。在PCD患者的血清或脑脊液（CSF）中经常观察到针对小脑浦肯野细胞的自身抗体。PCD的病因尚不清楚，但一些患者体内存在这些自身抗体提示其发病机制可能是免疫介导的。本文讨论了自身抗体在PCD发病机制中的潜在作用。
