Tang-Wai Richard, Mailo Janette, Rosenblatt Bernard
Division of Pediatric Neurology, University of Alberta, Canada.
Division of Pediatric Neurology, University of Western Ontario, Canada.
Seizure. 2017 Apr;47:34-41. doi: 10.1016/j.seizure.2017.03.004. Epub 2017 Mar 6.
Because immune mediated mechanisms are suspected in epileptogenesis, IVIg and corticosteroids have been used as alternatives to treat refractory seizures. We present our experience treating intractable epileptic children with IVIg and prednisone.
Children with intractable epilepsy treated with prednisone or IVIg between 2005-2016 were reviewed retrospectively. Children with infantile spasms and autoimmune epilepsy were excluded. Data analyzed include epilepsy type and etiology, duration of epilepsy prior to treatment, seizure outcome, time to best seizure outcome, and adverse effects.
Fifty-one patients were included: 26 received IVIg; 25 received prednisone. Etiologies were similar between cohorts: genetic (13 IVIg; 10 prednisone), lesional (8 IVIg; 7 prednisone), and unknown (5 IVIg; 8 prednisone). In the prednisone cohort, 92.0% had generalized epilepsy compared to 61.5% for IVIg. Among the IVIg treated, 84.6% responded (10 genetic, 4 unknown, and 8 lesional) with mean seizure reduction of 77.3% and mean time to best response of 9.8 weeks. With prednisone, 24.0% responded (2 genetic, 3 unknown, and 1 lesional) with a mean seizure reduction of 95.0% and mean time to best response of 2.7 weeks. Adverse effects occurred in 2 and 16 patients treated with IVIg and prednisone, respectively. The difference in responders and seizure reduction was statistically significant (p<0.0001 and p=0.001, respectively).
IVIg had greater responders and lower adverse effects and honeymoon effect. This response was independent of epilepsy type, etiology, and duration suggesting different mechanisms of action between prednisone and IVIg and a common, reversible, immune-mediated pathway to intractability.
由于怀疑免疫介导机制参与癫痫发生，静脉注射免疫球蛋白（IVIg）和皮质类固醇已被用作治疗难治性癫痫发作的替代方法。我们介绍了使用IVIg和泼尼松治疗难治性癫痫儿童的经验。
回顾性分析2005年至2016年间接受泼尼松或IVIg治疗的难治性癫痫儿童。排除婴儿痉挛症和自身免疫性癫痫患儿。分析的数据包括癫痫类型和病因、治疗前癫痫持续时间、癫痫发作结果、达到最佳癫痫发作结果的时间以及不良反应。
共纳入51例患者：26例接受IVIg治疗；25例接受泼尼松治疗。两组队列的病因相似：遗传性（IVIg组13例；泼尼松组10例）、病灶性（IVIg组8例；泼尼松组7例）和不明原因（IVIg组5例；泼尼松组8例）。在泼尼松组中，92.0%为全身性癫痫，而IVIg组为61.5%。在接受IVIg治疗的患者中，84.6%有反应（遗传性10例、不明原因4例、病灶性8例），癫痫发作平均减少77.3%，达到最佳反应的平均时间为9.8周。使用泼尼松时，24.0%有反应（遗传性2例、不明原因3例、病灶性1例），癫痫发作平均减少95.0%，达到最佳反应的平均时间为2.7周。接受IVIg和泼尼松治疗的患者分别有2例和16例出现不良反应。反应者和癫痫发作减少方面的差异具有统计学意义（分别为p<0.0001和p=0.001）。
IVIg有更多的反应者，不良反应和蜜月效应更低。这种反应与癫痫类型、病因和病程无关，提示泼尼松和IVIg之间作用机制不同，且存在一条共同的、可逆的、免疫介导的难治性途径。
