Neurology Department, Huashan Hospital, Fudan University, China.
Neurology Department, Huashan Hospital, Fudan University, China.
J Clin Neurosci. 2019 Oct;68:73-79. doi: 10.1016/j.jocn.2019.07.049. Epub 2019 Jul 19.
To evaluate the clinical characteristics, treatment outcomes, prognosis and potential risk factors of patients in East China with seizure secondary to autoimmune encephalitis.
From February 2014 to June 2016, 113 patients diagnosed with autoimmune encephalitis in Huashan Hospital, Fudan University, were enrolled in our study. After at least two years of follow-up, we retrospectively analyzed the patients' clinical details, electroencephalograph performance, brain MRI findings, and the therapeutic outcome. Patients underwent clinical evaluation every 3 months. We compared the clinical characteristics and epileptic prognosis of autoimmune encephalitis per antibody type. The association of the epileptic prognosis and EEG abnormalities was evaluated. GTE (Grand Total EEG) Score was used to evaluate EEG abnormalities. Statistic methods included ANOVA, Bonferroni correction test.
Treatment outcomes were assessabled in 103 patients (10 patients died or withdrew), including anti-GABAR encephalitis (11), anti-LGI1 encephalitis (16), anti-NMDAR encephalitis (73), Caspr2 antibody encephalitis (3). 83 patients had seizures, who underwent both immunotherapy and anti-epileptic drugs therapy. In terms of seizure type, 57 (68.7%) patients exhibited focal to bilateral tonic-clonic seizure (FBTCS), 51 (61.4%) patients exhibited focal-impaired awareness seizure (FIAS) or focal aware seizure (FAS). 18 (21.7%) patients developed to status epilepticus. 30 (36%) patients had multiple types of seizures. 39 (47%) patients had daily seizures. 80.7% (67/83) of patients with epilepsy had seizure remission. During the 24 months of follow-up, 11 (11%) patients had clinical relapses. GTE scores were significantly different between the group with seizure reduction < 75% and the group with seizure remission (p = 0.009). Imaging abnormalities existed in 53% of the patients in our cohort, but lacked specificity during the acute phase.
Autoimmune encephalitis (AE) presents with large seizure burden with differing seizure semiology among different antibody types. Except for anti-GABAb receptor encephalitis, it may not be necessary for other AE types to apply long-term use of anti-epileptic drugs (AEDs). The GTE Score can be used to evaluate the EEG abnormalities and may be a predictor of seizure outcomes. MRI findings during the acute phase are non-specific. Long-term follow-up MRIs may be much more meaningful in evaluating prognosis.
评估华东地区自身免疫性脑炎继发癫痫患者的临床特征、治疗结局、预后及潜在危险因素。
本研究纳入 2014 年 2 月至 2016 年 6 月期间在复旦大学华山医院确诊为自身免疫性脑炎的 113 例患者。经过至少 2 年的随访，我们回顾性分析了患者的临床资料、脑电图表现、脑 MRI 结果和治疗结局。患者每 3 个月进行一次临床评估。我们比较了不同抗体类型的自身免疫性脑炎的临床特征和癫痫预后，并评估了脑电图异常与癫痫预后的相关性。采用 GTE（总脑电图）评分评估脑电图异常。统计方法包括方差分析、Bonferroni 校正检验。
103 例患者（10 例死亡或退出）的治疗结局可评估，包括抗 GABAR 脑炎（11 例）、抗 LGI1 脑炎（16 例）、抗 NMDAR 脑炎（73 例）、Caspr2 抗体脑炎（3 例）。83 例患者有癫痫发作，均接受免疫治疗和抗癫痫药物治疗。在癫痫发作类型方面，57 例（68.7%）患者表现为局灶性双侧强直-阵挛发作（FBTCS），51 例（61.4%）患者表现为局灶性意识障碍发作（FIAS）或局灶性意识发作（FAS）。18 例（21.7%）患者发展为癫痫持续状态。30 例（36%）患者有多种类型的癫痫发作。39 例（47%）患者有每日发作。80.7%（67/83）的癫痫患者癫痫发作缓解。在 24 个月的随访期间，11 例（11%）患者出现临床复发。脑电图 GTE 评分在癫痫发作缓解<75%的患者与癫痫发作缓解的患者之间有显著差异（p=0.009）。我们队列中有 53%的患者存在影像学异常，但在急性期缺乏特异性。
自身免疫性脑炎（AE）表现为大的癫痫发作负荷，不同抗体类型的癫痫发作半侧不同。除抗 GABAb 受体脑炎外，其他 AE 类型可能不需要长期使用抗癫痫药物（AEDs）。GTE 评分可用于评估脑电图异常，并可能是癫痫发作结局的预测因子。急性期 MRI 表现无特异性。长期随访 MRI 可能更有意义，有助于评估预后。
