Honnorat J, Antoine J C, Derrington E, Aguera M, Belin M F
INSERM U 433, department of Neuropathology, Hôpital Neurologique, Lyon, France.
J Neurol Neurosurg Psychiatry. 1996 Sep;61(3):270-8. doi: 10.1136/jnnp.61.3.270.
Paraneoplastic neurological syndromes (PNS) are inflammatory disorders that probably depend on autoimmune processes. Several autoantibodies (anti-Hu, anti-Ri, and anti-Yo) have been characterised in PNS and proved to be helpful in the diagnosis. However, these do not account for all the cases and the possibility that other types of antibodies could be detected was investigated.
Of 45 patients with PNS whose serum was probed on paraformaldehyde fixed rat brain sections, 11 patients were identified whose serum samples recognised a cytoplasmic antigen in a subpopulation of glial cells in the white matter of adult rat brainstem, cerebellum, and spinal cord that were double labelled with a monoclonal antibody specific for oligodendrocytes. All serum samples reacted with a 66 kDa protein of newborn rat brain on western blot analysis. These antibodies were designated as anti-CV2 antibodies. Only one of the 11 patients had one of the well characterised autoantibodies (anti-Hu). Five patients had cerebellar degeneration, three had limbic encephalitis, two had encephalomyelitis, and one had Lambert-Eaton myasthenic syndrome. The tumours were small cell lung cancer or undifferentiated mediastinal cancer in seven patients, uterine sarcoma in two, and malignant thymoma in two. Among 1061 control serum samples, only two patients had anti-CV2 antibodies. One had small cell lung cancer and the other malignant thymoma.
The detection of anti-CV2 antibodies in patients with neurological disorders should be considered as an indication of the presence of an occult cancer.
副肿瘤性神经系统综合征（PNS）是可能依赖自身免疫过程的炎症性疾病。几种自身抗体（抗Hu、抗Ri和抗Yo）已在PNS中得到鉴定，并被证明有助于诊断。然而，这些并不能解释所有病例，因此对是否能检测到其他类型抗体的可能性进行了研究。
在45例PNS患者中，用其血清检测经多聚甲醛固定的大鼠脑切片，发现11例患者的血清样本能识别成年大鼠脑干、小脑和脊髓白质中胶质细胞亚群的一种胞质抗原，这些胶质细胞用少突胶质细胞特异性单克隆抗体进行了双重标记。所有血清样本在蛋白质印迹分析中均与新生大鼠脑的一种66 kDa蛋白发生反应。这些抗体被命名为抗CV2抗体。11例患者中只有1例具有一种已明确的自身抗体（抗Hu）。5例患者有小脑变性，3例有边缘叶脑炎，2例有脑脊髓炎，1例有兰伯特-伊顿肌无力综合征。肿瘤在7例患者中为小细胞肺癌或未分化纵隔癌,2例为子宫肉瘤，2例为恶性胸腺瘤。在1061份对照血清样本中，只有2例患者有抗CV2抗体。1例患有小细胞肺癌，另1例患有恶性胸腺瘤。
在神经系统疾病患者中检测到抗CV2抗体应被视为隐匿性癌症存在的指征。
