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[儿童恶性癫痫:大剂量静脉注射免疫球蛋白治疗]

[Malignant epilepsy in children: therapy with high doses of intravenous immunoglobulin].

作者信息

Sterio M, Gebauer E, Felle D, Vucicević G, Zalisevskij G

机构信息

Institut za zdravstvenu zastitu majke i deteta, Medicinski fakultet, Novi Sad.

出版信息

Med Pregl. 1992;45(5-6):220-4.

PMID:1365062
Abstract

22 children with intractable childhood epilepsy (ICE) showing no response to conventional drugs of hormone (ACTH, Synacten) therapy were administered i.v. immunoglobulin (ENDOBULIN immuno) at a dosage of 400 mg/kg on the first and 15th day and subsequently every 3 weeks for 6 months. 12/22 patients showed IgG2 subclass deficiency. A significant reduction in attacks, or even absence of attacks was observed in 13/22 children after 6 months of i.v. immunoglobulin therapy. Most of this children showed IgG2 subclass deficiency. The reduction of attacks after i.v. immunoglobulin therapy correlated with the improvement or normalization of the EEG finding. As for the psychomotor development, no major changes were noticed with respect to the condition prior to the therapy, but in children with IgG2 deficiency, there is no further psychomotor deterioration. 6 months after the last i.v. immunoglobulin dose positive therapeutic effect remained in 5/22 children, with 3 children the therapy was repeated because of recidive attacks and worse EEG findings, and proved effective. Light worsening of the EEG findings was found in 3/22 children, 2/22 dropped out, 1/22 child died of intercurrent infection, and in girl the attacks ceased entirely 4 months after the last i.v. immunoglobulin dose. With other children the condition remained unchanged. According to the authors opinion, i.v. immunoglobulin has its own place in ICE treatment, and it is evident in all cases where the classical antiepileptic and/or hormone therapy was unsuccessful, especially in children with IgG2 subclass deficiency, that is, in all the epilepsy cases where a great number of attacks is imperilling the psychomotor development in children, independently of type.

摘要

22名患有儿童难治性癫痫(ICE)且对传统激素药物(促肾上腺皮质激素、辛纳科坦)治疗无反应的儿童,在第1天和第15天静脉注射免疫球蛋白(恩多布灵免疫),剂量为400mg/kg,随后每3周注射一次,持续6个月。22名患者中有12名存在IgG2亚类缺陷。在静脉注射免疫球蛋白治疗6个月后,22名儿童中有13名发作次数显著减少,甚至不再发作。这些儿童大多数存在IgG2亚类缺陷。静脉注射免疫球蛋白治疗后发作次数的减少与脑电图结果的改善或正常化相关。至于精神运动发育,与治疗前相比没有发现重大变化,但在IgG2缺陷的儿童中,精神运动没有进一步恶化。在最后一次静脉注射免疫球蛋白剂量6个月后,22名儿童中有5名仍有积极的治疗效果,3名儿童因发作复发和脑电图结果变差而重复治疗,且证明有效。22名儿童中有3名脑电图结果轻度恶化,2名退出治疗,1名儿童死于并发感染,一名女孩在最后一次静脉注射免疫球蛋白剂量4个月后发作完全停止。其他儿童的病情保持不变。根据作者的观点,静脉注射免疫球蛋白在ICE治疗中有其自身的地位,在所有经典抗癫痫和/或激素治疗失败的病例中都很明显,尤其是在IgG2亚类缺陷的儿童中,也就是说,在所有大量发作危及儿童精神运动发育的癫痫病例中,无论癫痫类型如何。

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