免疫球蛋白对Landau-Kleffner综合征疗效分析(附1例报道)

目的：分析Landau—Kleffner综合征（LKS）的临床特点，并探讨静脉注射免疫球蛋白（IVIG）治疗LKS的作用机制。方法：对1例以反复抽搐5月，语言功能减退1月为主诉入院的16个月患儿进行临床和实验室总结，行单-IVIG治疗。结果：单用IVIG治疗后癫痫无再次发作，语言功能恢复。3个月后复发，再次治疗1周病情再度控制，随访10个月未复发。结论：IVIG不仅可作为治疗LKS的一线药物，而且为该病的免疫学机制假说提供了依据。

Efficacy of Intravenous Immunoglobulin Treatment in a Case of Landau-Kleffner Syndrome

Objective： To analyze the clinical characteristics of Landau Kleffner syndrome （LKS）and to investigate the relationship between LKS and immune mechanism, and possible effect mechanism of intravenous immunoglobulin （IVIG）. Methods： The clinical features and laboratory data of a 16-month-old girl with experience language regression with verbal auditory agnosia,abnormal epileptiform activity, behavioral disturbances,and sometimes overt seizures admitted in March 2007 was analyzed. To treat only with IVIG, and therapeutic response and long-term outcome were followed up. Results： She received two courses of intravenous （i.v.） gamma-globulin, after initial course, clinical and immune factors improvement lasted 3 months but was followed by recurrence of the seizures and by speech deterioration. Her last remission has been continuous for the past 10 months. Conclusions： Based on our experience with this patient and on other investigators＇ experience, we believe that IVIG showed favorable effects on LKS, further research into immunologic mechanisms and therapies of this syndrome are warranted.