Bien Christian G, Vincent Angela
Epilepsy Center Bethel, Bielefeld, Germany.
Handb Clin Neurol. 2013;111:521-31. doi: 10.1016/B978-0-444-52891-9.00055-5.
Apart from Rasmussen encephalitis, other immune-mediated brain disorders have emerged that are frequently associated with recurrent epileptic seizures: (1) limbic encephalitis, (2) anti-N-methyl-D-aspartate-receptor (NMDAR) encephalitis, and (3) steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT, formerly called Hashimoto encephalopathy). Limbic encephalitis mainly affects the mediotemporal aspect of the cortex giving rise to temporal lobe seizures and memory and affective disturbances. It can occur as a paraneoplastic or, even more frequently, a nonparaneoplastic condition. Detection of specific autoantibodies may suggest underlying cancer (onconeural antibodies) and may give hints regarding the long-term prognosis. VGKC-complex or GAD antibodies have been identified in children. Anti-NMDAR encephalitis is a disorder mainly of female adolescents and young adults. Patients present with seizures in around one-third of cases, and 80% of patients develop recurrent, mainly extratemporal seizures at some stage of their disorder. The clinical course is that of a mostly monophasic, but sometimes multiphasic, severe cortico-subcortical encephalopathy. Ovarian teratomas underlie the disorder in 30-60% of adult cases but are rare in young children. Tumor removal and early intense immunotherapy are predictors for a favorable outcome. SREAT is not a disorder with definite pathogenesis but rather a "catch all" for encephalopathic immune-mediated brain diseases (often with seizures) which have in common thyroid antibodies and a good response to steroid therapy.
除了拉斯穆森脑炎外，还出现了其他免疫介导的脑部疾病，这些疾病常与反复癫痫发作相关：（1）边缘叶脑炎，（2）抗N-甲基-D-天冬氨酸受体（NMDAR）脑炎，以及（3）与自身免疫性甲状腺炎相关的类固醇反应性脑病（SREAT，以前称为桥本脑病）。边缘叶脑炎主要影响皮质的颞中部位，导致颞叶癫痫发作以及记忆和情感障碍。它可作为副肿瘤性疾病出现，或者更常见的是作为非副肿瘤性疾病出现。检测特定自身抗体可能提示潜在癌症（肿瘤神经抗体），并可能提供有关长期预后的线索。儿童中已发现电压门控性钾离子通道复合物（VGKC）或谷氨酸脱羧酶（GAD）抗体。抗NMDAR脑炎主要是青少年女性和年轻成人的疾病。约三分之一的病例会出现癫痫发作，80%的患者在疾病的某个阶段会出现反复的癫痫发作，主要是颞叶外癫痫发作。临床病程大多为单相，但有时为多相，是一种严重的皮质-皮质下脑病。30%-60%的成年病例中该疾病由卵巢畸胎瘤引起，但在幼儿中很少见。切除肿瘤和早期强化免疫治疗是预后良好的预测因素。SREAT并非一种发病机制明确的疾病，而是一种对具有甲状腺抗体且对类固醇治疗反应良好的脑病性免疫介导性脑部疾病（常伴有癫痫发作）的统称。
