Landau-Kleffner综合征二例报告及文献复习  

Landau-Kleffner Syndrome: A Report of 2 Cases and Literature Review

作　　者：古丽鲜.阿布都热依木 吐尔逊娜依.沙都 阿依江.别克道来提 库尔班江.阿布都西库尔 

机构地区：[1]乌鲁木齐儿童医院神经科,乌鲁木齐830002 [2]乌鲁木齐儿童医院门诊部,乌鲁木齐830002 [3]复旦大学附属儿科医院肝病中心,上海201102

出　　处：《临床误诊误治》2013年第12期28-31,共4页Clinical Misdiagnosis & Mistherapy

摘　　要：目的探讨Landau-Kleffner综合征的临床特征,提高对该病的认识。方法对我院收治的2例LandauKleffner综合征的临床资料及短期随访结果进行分析,并复习近5年文献。结果本组2例,包括维吾尔族(女,7.5岁)及汉族(男,7岁)患儿各1例,均表现为进行性语言功能障碍,继之出现惊厥,具有脑电图典型改变(癫痫样放电),予常规抗癫痫药物及糖皮质激素治疗效果良好,短期随访预后佳。结论 Landau-Kleffner综合征表现典型,预后一般较好,但失语症状预后不尽人意,失语或失认时间越长,预后越差。Objective To discuss clinical features of Landau-Kleffner syndrome to enhance the awareness.Methods Clinical data of 2 patients with Landau-Kleffner syndrome and short-term follow-up data were analyzed,and literature in the past 5 years was also reviewed.Results In the two patients,one was a Uighur female （aged 7.5 years） and the other was a Han male （aged 7 years）.Both had progressive language functional disturbance followed by convulsion and typical EEG findings （epileptiform discharge）.The two children were given routine treatment with anti-epileptic drugs and glucocorticoids,and had good prognosis.The short-term follow-up showed satisfactory prognosis.Conclusion Landau-Kleffner syndrome has typical clinical manifestations with good prognosis.However,prognosis of aphasia is sub-optimal,and a long history of aphasia or agnosia is associated with poor prognosis.

关 键 词：LANDAU-KLEFFNER综合征 癫痫 失语 误诊 治疗 预后 进展 

分 类 号：R742.1[医药卫生—神经病学与精神病学]