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[伴有特别不良病程的肌阵挛-失张力发作癫痫(Lennox-Gastaut综合征)]

[Epilepsy with myoclonic-astatic attacks (Lennox-Gastaut syndrome) with particularly unfavorable course].

作者信息

Michałowicz R, Ignatowicz R, Karkowska B, Kmieć T, Potakiewicz W, Wymysłowska D

出版信息

Neurol Neurochir Pol. 1985 Mar-Apr;19(2):98-107.

PMID:3930980
Abstract

Out of over 100 children with the Lennox-Gastaut syndrome observed in the Outpatient Clinic and Department of Paediatric Neurology, Children's Health Center the authors present 6 cases in which the course of the disease was progressive and devastating. The main signs were astatic-myoclonic and generalized seizures, regression of psychomotor development, followed by mental regression, pareses of extremities, decortication symptoms and somatic cachexia. The authors suggest that the clinical course, the character of epileptic seizures very poor prognosis in certain children with the Lennox-Gastaut syndrome make the syndrome similar in its clinical aspects to subacute sclerosing panencephalitis and require careful differential diagnosis for excluding the latter disease.

摘要

在儿童健康中心门诊和儿科神经科观察到的100多名患有伦诺克斯 - 加斯东综合征的儿童中,作者介绍了6例疾病呈进行性且严重的病例。主要症状为站立不能性肌阵挛和全身性癫痫发作、精神运动发育倒退,随后出现智力衰退、肢体瘫痪、去皮层症状和身体恶病质。作者认为,某些患有伦诺克斯 - 加斯东综合征儿童的临床病程、癫痫发作特征及预后极差,使得该综合征在临床方面与亚急性硬化性全脑炎相似,需要仔细进行鉴别诊断以排除后者。

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