Department of Neurology and Experimental Neurology, Epilepsy-Center Berlin-Brandenburg, Charité - Universitätsmedizin Berlin, Berlin, Germany.
Department of Neurology and Experimental Neurology, Charité - Universitätsmedizin Berlin, Berlin, Germany; German Center for Neurodegenerative Diseases (DZNE) Berlin, Berlin, Germany.
Seizure. 2021 Mar;86:138-143. doi: 10.1016/j.seizure.2021.02.010. Epub 2021 Feb 13.
To determine long-term seizure outcome, use of antiseizure medication (ASM) and seizure recurrence risk after its withdrawal in patients with autoimmune encephalitis (AE) due to neuronal surface and GAD antibodies.
In patients from a specialized AE outpatient clinic, we assessed seizure manifestation, ASM and immunotherapy at onset of AE as well as seizure occurrence, development of autoimmune-associated epilepsy (AAE) and use of ASM in the long-term. Data were collected from patients via telephone interviews and medical records.
Out of 94 AE patients, 75 were analyzed; 47 patients had NMDAR, 17 LGI1, 7 GAD, 3 CASPR2 and 1 mGluR5 antibodies. Fifty-three of the 75 patients (71 %) experienced seizures, all of which for the first time occurred at AE onset. After a median follow-up of 6 years (range, 1-15), 47 of the 53 AE patients had 1-year terminal seizure remission, median duration of terminal seizure freedom was 5 years. Rate of 1-year terminal seizure remission was significantly higher in patients with neuronal surface antibodies (NMDAR 97 %, LGI1 93 %, CASPR2 100 %) compared to patients with GAD antibodies (20 %, p < 0.001). In seizure-free patients, ASM was withdrawn after 13 months (median) without any relapse seizures.
Seizures are common in most forms of AE manifesting at disease onset in all cases. However, the development of AAE is rare and typically occurs in patients with GAD antibodies. Thus, in most AE cases with neuronal surface antibodies, ASM can be withdrawn after the acute phase of AE with low risk of seizure relapse.
确定自身免疫性脑炎（AE）患者因神经元表面和 GAD 抗体导致的长期癫痫发作结局、抗癫痫药物（ASM）的使用以及停药后的复发风险。
在专门的 AE 门诊患者中，我们评估了 AE 发病时的癫痫发作表现、ASM 和免疫治疗，以及长期癫痫发作、自身免疫性相关癫痫（AAE）的发生和 ASM 的使用。通过电话访谈和病历收集患者的数据。
94 例 AE 患者中，75 例进行了分析；47 例患者存在 NMDAR 抗体，17 例存在 LGI1 抗体，7 例存在 GAD 抗体，3 例存在 CASPR2 抗体，1 例存在 mGluR5 抗体。75 例患者中有 53 例（71%）出现癫痫发作，所有癫痫发作均首次发生在 AE 发病时。中位随访时间为 6 年（范围 1-15 年）后，53 例 AE 患者中有 47 例在 1 年时达到癫痫发作终点缓解，终点无癫痫发作的中位持续时间为 5 年。与 GAD 抗体患者（20%，p<0.001）相比，神经元表面抗体患者（NMDAR 抗体 97%，LGI1 抗体 93%，CASPR2 抗体 100%）1 年时达到癫痫发作终点缓解的比例明显更高。在无癫痫发作的患者中，ASM 在 13 个月（中位数）后停药，无癫痫复发。
癫痫发作在大多数 AE 类型中较为常见，在所有病例中均在疾病发病时出现。然而，AAE 的发生较为罕见，且通常发生在 GAD 抗体患者中。因此，在大多数存在神经元表面抗体的 AE 病例中，AE 急性期过后可以停用 ASM，癫痫复发的风险较低。
