Feyissa Anteneh M, Lamb Christopher, Pittock Sean J, Gadoth Avi, McKeon Andrew, Klein Christopher J, Britton Jeffrey W
Department of Neurology Mayo Clinic Jacksonville Florida U.S.A.
Department of Neurology Mayo Clinic Rochester Minnesota U.S.A.
Epilepsia Open. 2018 Jun 25;3(3):348-356. doi: 10.1002/epi4.12226. eCollection 2018 Sep.
To characterize seizure semiology and the utility of antiepileptic drug (AED) therapy in leucine-rich glioma inactivated-1 ( LGI1-Ab) autoimmune epilepsy (AE).
Patients with voltage-gated potassium channel complex (VGKCc) titers higher than 0.02 nmol/L who were evaluated between May 2008 and June 2016 at the 3 Mayo Clinic sites (Arizona, Florida, or Minnesota) were identified. We then performed a retrospective review of those who were LGI1-Ab positive and were treated for seizures.
A total of 1,095 patients with VGKCc titers higher than 0.02 nmol/L were identified, in which 77 were LGI1 positive. Of these, 56 patients with seizures were included in the analysis. Mean age at symptom onset was 62.9 years; 66% (n = 37) were male. The most common seizure semiology was focal faciobrachial dystonic seizures with preserved awareness (FBDS) (n = 35, 63%), followed by focal with impaired awareness (FIA) (n = 29, 52%), generalized tonic-clonic (GTCs) (n = 28, 50%), and focal non-motor seizures with preserved awareness (n = 28, 50%). The majority had more than one seizure type (n = 49, 88%; median = 2.5). Thirty-eight patients (68%) became seizure free: 29 (76%) with immunotherapy, 3 (5%) with AEDs alone, 2 (3%) with AEDs before any immunotherapy, and 4 (7%) with AEDs after immunotherapy. Levetiracetam (n = 47, 84%) and valproic acid (n = 21, 38%) were the most commonly used AEDs, but neither were associated with seizure freedom. Sodium channel blocking (NCB) AEDs were associated with seizure freedom in 4 patients compared to none treated with non-NCB AEDs. Regardless of class, AEDs prior to or apart from immunotherapy were associated with seizure freedom in only five patients (9%). In patients with FBDS, seizure freedom was more often associated with immunotherapy than AEDs (20/30 vs. 3/34, p = 0.001).
Although FBDS are the most characteristic seizure type seen in LGI1-Ab AE, other seizure types including FIA and GTCs also occur. Immunotherapy was the treatment most frequently associated with seizure freedom in LGI1-Ab AE. In general, AEDs seemed to confer a very low chance for seizure freedom, although AEDs with NCB-blocking properties were associated with seizure freedom in a limited number. Levetiracetam in particular appears to be ineffective in this patient population.
描述富含亮氨酸胶质瘤失活-1（LGI1-Ab）自身免疫性癫痫（AE）的发作症状学及抗癫痫药物（AED）治疗的效用。
确定2008年5月至2016年6月在梅奥诊所3个院区（亚利桑那州、佛罗里达州或明尼苏达州）接受评估的电压门控钾通道复合物（VGKCc）滴度高于0.02 nmol/L的患者。然后我们对那些LGI1-Ab阳性且接受过癫痫治疗的患者进行了回顾性分析。
共确定了1095例VGKCc滴度高于0.02 nmol/L的患者，其中77例LGI1呈阳性。其中，56例有癫痫发作的患者纳入分析。症状发作时的平均年龄为62.9岁；66%（n = 37）为男性。最常见的发作症状学是意识保留的局灶性面臂肌张力障碍性发作（FBDS）（n = 35，63%），其次是意识障碍的局灶性发作（FIA）（n = 29，52%）、全身强直阵挛发作（GTCs）（n = 28，50%）和意识保留的局灶性非运动性发作（n = 28，50%）。大多数患者有不止一种发作类型（n = 49，88%；中位数 = 2.5）。38例患者（68%）实现无癫痫发作：29例（76%）接受免疫治疗，3例（5%）仅接受AEDs治疗，2例（3%）在任何免疫治疗前接受AEDs治疗，4例（7%）在免疫治疗后接受AEDs治疗。左乙拉西坦（n = 47，84%）和丙戊酸（n = 21，38%）是最常用的AEDs，但两者均与无癫痫发作无关。与未使用非钠通道阻断（NCB）AEDs治疗的患者相比，使用钠通道阻断（NCB）AEDs的4例患者实现了无癫痫发作。无论类别如何，在免疫治疗之前或之外使用AEDs仅有5例患者（9%）实现了无癫痫发作。在FBDS患者中，无癫痫发作更常与免疫治疗而非AEDs相关（20/30对3/34，p = 0.001）。
虽然FBDS是LGI1-Ab AE中最具特征性的发作类型，但其他发作类型包括FIA和GTCs也会出现。免疫治疗是LGI1-Ab AE中最常与无癫痫发作相关的治疗方法。总体而言，AEDs似乎实现无癫痫发作的机会非常低，尽管具有NCB阻断特性的AEDs在少数情况下与无癫痫发作相关。特别是左乙拉西坦在该患者群体中似乎无效。
