Antoine J C, Honnorat J
Service de Neurologie, Hôpital de Bellevue, Saint-Etienne.
Rev Neurol (Paris). 2000 Jan;156(1):23-33.
Antibodies against antigens found in the central nervous system have been evidenced in several neurological diseases. The most well-known are associated with paraneoplastic neurological diseases (Anti-Hu, Yo, Ri amphiphysin, Tr, CV2 and Ta antibodies). Some of these antibodies are specific for certain types of cancer or neurological syndromes and are highly useful diagnostic tools for the clinician. They have contributed to the hypothesis that these paraneoplastic neurological syndromes involve autoimmune cross reactions between tumoral and nervous system antigens. They are however most unlikely pathogenic on their own. Anti voltage-dependent calcium channel antibodies associated with Lambert-Eaton syndrome which is paraneoplastic in only 60 p. 100 of the cases are also observed in cases of paraneoplastic cerebellar atrophy. Anti-GAD antibodies are seen in non-paraneoplastic stiff man syndrome and in certain progressive cerebellar atrophies. Antibodies reacting with different glutamate receptors are detected in different neurological diseases including Rasmussen's encephalitis. Finally, antibodies are described in diverse conditions such as amyotrophic lateral sclerosis, Sydeham chorea or Gilles de la Tourette syndrome. The significance of the antibodies observed outside the context of paraneoplastic syndromes is not well understood, but the anti-GAD antibodies associated with progressive cerebellar disorders and autoimmune polyendocrinopathies could be an expression of the autoimmune nature of certain neurological degenerative processes affecting the central nervous system.
在几种神经系统疾病中已证实存在针对中枢神经系统中发现的抗原的抗体。最著名的抗体与副肿瘤性神经系统疾病相关(抗Hu、Yo、Ri抗 amphiphysin、Tr、CV2和Ta抗体)。其中一些抗体对某些类型的癌症或神经系统综合征具有特异性,是临床医生非常有用的诊断工具。它们促成了这样一种假说,即这些副肿瘤性神经系统综合征涉及肿瘤抗原与神经系统抗原之间的自身免疫交叉反应。然而,它们自身致病的可能性极小。与兰伯特-伊顿综合征相关的抗电压依赖性钙通道抗体在副肿瘤性小脑萎缩病例中也有观察到,而兰伯特-伊顿综合征仅在60%的病例中是副肿瘤性的。抗谷氨酸脱羧酶抗体见于非副肿瘤性僵人综合征和某些进行性小脑萎缩。在包括拉斯穆森脑炎在内的不同神经系统疾病中检测到与不同谷氨酸受体发生反应的抗体。最后在诸如肌萎缩侧索硬化症、小舞蹈病或 Gilles de la Tourette综合征等多种病症中也描述了抗体。在副肿瘤综合征背景之外观察到的抗体的意义尚未完全了解,但与进行性小脑疾病和自身免疫性多内分泌腺病相关的抗谷氨酸脱羧酶抗体可能是某些影响中枢神经系统的神经退行性过程的自身免疫性质的一种表现。