Britton Jeffrey
Department of Neurology, Mayo Clinic, Rochester, MN, USA.
Handb Clin Neurol. 2016;133:219-45. doi: 10.1016/B978-0-444-63432-0.00013-X.
Seizures are a common manifestation of autoimmune limbic encephalitis and multifocal paraneoplastic disorders. Accumulating evidence supports an autoimmune basis for seizures in the absence of syndromic manifestations of encephalitis. The autoimmune epilepsies are immunologically mediated disorders in which recurrent seizures are a primary and persistent clinical feature. When other etiologies have been excluded, an autoimmune etiology is suggested in a patient with epilepsy upon detection of neural autoantibodies and/or the presence of inflammatory changes on cerebrospinal fluid (CSF) or magnetic resonance imaging. In such patients, immunotherapy may be highly effective, depending on the particular autoimmune epilepsy syndrome present. In this chapter, several autoimmune epilepsy syndromes are discussed. First, epilepsies secondary to other primary autoimmune disorders will be discussed, and then those associated with antibodies that are likely to be pathogenic, such as voltage-gated potassium channel-complex and N-methyl-d-aspartate receptor, gamma-aminobutyric acid A and B receptor antibodies. For each syndrome, the typical clinical, imaging, electroencephaloram, CSF, and serologic features, and pathophysiology and treatment are described. Finally, suggested guidelines for the recognition, evaluation, and treatment of autoimmune epilepsy syndromes are provided.
癫痫发作是自身免疫性边缘叶脑炎和多灶性副肿瘤性疾病的常见表现。越来越多的证据支持在没有脑炎综合征表现的情况下，癫痫发作存在自身免疫基础。自身免疫性癫痫是免疫介导的疾病，其中反复发作的癫痫是主要且持续的临床特征。当排除其他病因后，在癫痫患者中，若检测到神经自身抗体和/或脑脊液（CSF）或磁共振成像上存在炎症改变，则提示存在自身免疫病因。在此类患者中，免疫治疗可能非常有效，这取决于所呈现的特定自身免疫性癫痫综合征。在本章中，将讨论几种自身免疫性癫痫综合征。首先，将讨论继发于其他原发性自身免疫性疾病的癫痫，然后讨论与可能具有致病性的抗体相关的癫痫，如电压门控钾通道复合物抗体和N-甲基-D-天冬氨酸受体、γ-氨基丁酸A和B受体抗体。对于每种综合征，将描述典型的临床、影像学、脑电图、脑脊液和血清学特征，以及病理生理学和治疗方法。最后，提供了自身免疫性癫痫综合征的识别、评估和治疗建议指南。
