Limbic encephalitis as a precipitating event in adult-onset temporal lobe epilepsy
Limbic encephalitis as a precipitating event in adult-onset temporal lobe epilepsy
复制标题
DOI：
10.1212/01.wnl.0000276946.08412.ef
复制
发表时间：
2007-09-18
期刊：
影响因子：
9.9
通讯作者：
Elger, C. E.
中科院分区：
文献类型：
--
作者：
Bien, C. G.;Urbach, H.;Elger, C. E.
目的：颞叶癫痫伴海马硬化（TLE-HS）是尸检和手术癫痫系列中最常见的诊断。 TLE-HS 通常在儿童期或青春期开始。关于成人发病的疾病的研究很少。我们认识到，一些成年人有边缘脑炎 (LE) 的证据，这是一种成人生活中的自身免疫性疾病，我们认为这可能会直接导致这种综合征。方法：我们对 1999 年至 1999 年间癫痫发作 6 年内到本三级中心就诊的所有 TLE-HS 患者的病史、临床和临床旁发现、脑部 MRI 和手术治疗结果（包括组织病理学（如果有））进行了回顾性分析。 2005.结果：确定了 38 名患者，癫痫发作的中位年龄为 37.8 岁。 11 名患者 (29%) 被归类为继发性 HS（例如头部外伤后、热性惊厥）。七名患者 (11%) 被归类为特发性。然而，9名患者（24%）被诊断为明确的LE，另外11名患者（29%）显示出典型的LE模式MRI表现，海马肿胀演变成萎缩，FLAIR/T2信号持续增加；他们被诊断为可能的 LE。两个 LE 亚组 (60%) 的双侧异常比两个非 LE 亚组 (22%；p = 0.025) 更常见。对一名可能患有 LE 的患者在发病后不久进行组织病理学检查，结果显示典型的 T 细胞浸润和海马神经元丢失。结论：伴有海马硬化的颞叶癫痫可在成年后表现出来。大约一半的患者有与自身免疫过程一致的证据。如果得到证实，这将对诊断、预防和治疗产生影响。
Objective: Temporal lobe epilepsy with hippocampal sclerosis (TLE-HS) is the most frequent diagnosis in autopsy and surgical epilepsy series. TLE-HS usually starts during childhood or adolescence. There have been few studies of adult-onset disease. We recognized that some adult individuals have evidence of limbic encephalitis (LE), an autoimmune condition of adult life, which we proposed might lead directly to this syndrome.Methods: We performed a retrospective analysis of history, clinical and paraclinical findings, brain MRI, and outcome of surgical treatment including histopathology (if available) of all patients with TLE-HS presenting to this tertiary center within 6 years of epilepsy onset between 1999 and 2005.Results: Thirty-eight patients were identified, with median age at epilepsy onset of 37.8 years. Eleven patients (29%) were classified as having secondary HS (e.g., after head trauma, febrile seizures). Seven patients (11%) were classified as idiopathic. However, 9 patients (24%) had a diagnosis of definite LE, and another 11 individuals (29%) showed the typical LE pattern of MRI findings with hippocampal swelling evolving into atrophy with continuous FLAIR/T2 signal increase; they were diagnosed as possible LE. Bilateral abnormalities were more frequent in the two LE subgroups (60%) than in the two non-LE subgroups (22%; p = 0.025). Histopathology was performed in one patient with possible LE shortly after disease onset and showed a typical T cell infiltration and loss of hippocampal neurons.Conclusions: Temporal lobe epilepsy with hippocampal sclerosis can manifest in adult life. Around half the patients have evidence consistent with an autoimmune process. If confirmed, this should have implications for diagnosis, prevention, and treatment.