Department of Neurology, The First Hospital of Jilin University, Changchun, Jilin 130021, China.
Department of Neurology, The First Hospital of Jilin University, Changchun, Jilin 130021, China.
Epilepsy Behav. 2019 May;94:198-203. doi: 10.1016/j.yebeh.2018.10.038. Epub 2019 Apr 8.
The objective of this study was to report seizure characteristics, long-term outcome, and potential factors associated with persistent seizures in patients with autoimmune synaptic encephalitis (ASE).
Clinical data and courses of 52 patients with ASE who presented with seizures at the Department of Neurology of the First Hospital of Jilin University from January 2015 to August 2017 were reviewed. Seizure outcomes were assessed with a median follow-up duration of 30 months (8-40 months).
Most patients (71.2%) presented with seizure at initial consultation; focal to bilateral tonic-clonic seizures (50.0%) were the most common type. The temporal lobe (73.5%) was the prominent region of seizure origin, which was incident with hippocampal lesions on magnetic resonance imaging (MRI) in 62.1% of the patients. Status epilepticus, subclinical seizures, and nonepileptic events were observed in 28.9%, 36.8%, and 28.9% of the patients, respectively. Twenty-seven out of the 43 followed-up patients (62.8%) exhibited seizure remission after initial immunotherapy. Others (37.2%) developed persistent seizures to different extents. Six out of 9 patients experienced additional seizure freedom because of antiepileptic drugs (AEDs); however, the seizures of the other three patients, with serious conditions, showed poor response. Patients with anti-N-methyl-d-aspartate receptor antibodies had a lower risk of developing persistent seizures than those with anti-leucine-rich glioma-inactivated 1 (LGI1) or anti-γ-aminobutyric acid receptor type B receptor (GABAR) antibodies (P = 0.001).
A complex of clinical and subclinical seizures, and nonepileptic events characterize ASE. Patients with anti-LGI1 or anti-GABAR antibodies have a higher risk of developing persistent seizures; AEDs are suitable for achieving additional seizure freedom, but not for patients with serious conditions. A few patients present with super-refractory epilepsy despite multiple treatments.
本研究旨在报告自身免疫性突触脑炎(ASE)患者癫痫发作的特征、长期预后以及与持续性癫痫相关的潜在因素。
回顾 2015 年 1 月至 2017 年 8 月吉林大学第一医院神经内科就诊的 52 例癫痫发作的 ASE 患者的临床资料和病程。采用中位数随访 30 个月(8-40 个月)评估癫痫发作结局。
大多数患者(71.2%)在初次就诊时出现癫痫发作;局灶性至双侧强直阵挛性发作(50.0%)是最常见的类型。颞叶(73.5%)是癫痫发作的主要起源区域,62.1%的患者在 MRI 上有海马病变。28.9%、36.8%和 28.9%的患者分别出现癫痫持续状态、亚临床发作和非癫痫性事件。43 例随访患者中有 27 例(62.8%)在初始免疫治疗后癫痫发作缓解。其余(37.2%)患者不同程度地出现持续性癫痫发作。9 例中有 6 例因抗癫痫药物(AED)而再次无癫痫发作,但另外 3 例病情严重的患者癫痫发作反应较差。与抗 N-甲基-D-天冬氨酸受体抗体相比,抗富亮氨酸胶质瘤失活 1(LGI1)或抗γ-氨基丁酸 B 型受体(GABAR)抗体的患者发生持续性癫痫的风险较低(P=0.001)。
ASE 以复杂的临床和亚临床癫痫发作以及非癫痫性事件为特征。抗 LGI1 或抗 GABAR 抗体的患者发生持续性癫痫的风险较高;AED 适用于实现额外的无癫痫发作,但不适用于病情严重的患者。尽管经过多次治疗,仍有少数患者出现超难治性癫痫。