Centre de référence, de diagnostic et de traitement des syndromes neurologiques paranéoplasiques, hôpital neurologique Pierre-Wertheimer, 69677 Bron cedex, France.
Rev Neurol (Paris). 2011 May;167(5):408-17. doi: 10.1016/j.neurol.2010.07.032. Epub 2010 Nov 4.
Accumulation of recent clinical evidence indicates that the immune system plays an important role in some central nervous system diseases usually regarded as degenerative. The most striking example is paraneoplastic cerebellar ataxia (PCA), which is characterized by autoimmune cross-reaction between tumoral and nervous system antigens.
In the past 20 years, several antibodies directed against neuronal and tumoral antigens have been described in association with PCA, leading to the description of different subtypes of PCA based on the associated antibodies, the clinical course and the type of tumor. In some subtypes, cerebellar ataxia occurs in isolation, whereas in others, cerebellar ataxia is a syndrome that occurs in conjunction with extensive nervous system disease. Circulating antibodies have also been described in patients with non-paraneoplastic cerebellar ataxia (N-PCA), suggesting that the immune system may be involved in certain cases of sporadic cerebellar ataxia.
Immune-mediated cerebellar ataxia does not seem to be limited to paraneoplastic neurological syndromes. Further studies are however necessary to understand the exact pathophysiology of these disorders and offer effective treatments.
In this review, the clinical presentation of the different subtypes of potentially immune-mediated PCA and N-PCA will be described, and the associated tumors will be discussed.
最近的临床证据表明，免疫系统在某些通常被认为是退行性的中枢神经系统疾病中起着重要作用。最引人注目的例子是副肿瘤性小脑共济失调（PCA），其特征是肿瘤和神经系统抗原之间的自身免疫交叉反应。
在过去的 20 年中，已经描述了几种针对神经元和肿瘤抗原的抗体与 PCA 相关联，从而根据相关抗体、临床过程和肿瘤类型描述了不同类型的 PCA。在某些亚型中，小脑共济失调是孤立发生的，而在其他亚型中，小脑共济失调是与广泛神经系统疾病同时发生的综合征。在非副肿瘤性小脑共济失调（N-PCA）患者中也描述了循环抗体，这表明免疫系统可能参与某些散发性小脑共济失调病例。
免疫介导性小脑共济失调似乎不仅限于副肿瘤性神经综合征。然而，需要进一步研究以了解这些疾病的确切病理生理学，并提供有效的治疗方法。
在这篇综述中，将描述不同亚型的潜在免疫介导性 PCA 和 N-PCA 的临床表现，并讨论相关肿瘤。
