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[儿童非典型良性部分性癫痫综合征的诊断标准]

[Diagnostic criteria of atypical benign partial epilepsy syndrome in childhood].

作者信息

Mukhin K Iu, Glukhova L Iu, Petrukhin A S, Iukhalina N S, Gaman O V

出版信息

Zh Nevrol Psikhiatr Im S S Korsakova. 2001;101(1):13-21.

Abstract

The paper presents an analysis of clinical-neuropsychological peculiarities of the syndrome of atypical benign partial childhood epilepsy (pseudolennox syndrome) of 6 patients (3 boys, 3 girls). An age of the onset of the disease was between 1.5-4 years. There was polymorphism of paroxysms, their high frequency with an obligate presence of hemifacial fits and atypical absences. Night generalized tonic-clonic attacks and the falling attacks were found in 67% of the patients. Spectrum of the neurological disorders included disorders of speech and a slight cerebella symptomatology. Regional "rolandic" activity and diffuse epileptiformed disorders, increasing into a phase of a slow sleep, were registered by EEG. Resistance to anticonvulsive therapy was revealed. The authors had demonstrated a nosologic independence of pseudolennox syndrome and had considered worth while to pick it out in a group of cryptogenic partial forms of epilepsy together with the epileptic aphasia of Landu-Kleffner and an epilepsy with the continuous peak-waves during the slow sleep.

摘要

本文对6例(3男3女)非典型良性部分性儿童癫痫综合征(假性 Lennox 综合征)的临床神经心理学特点进行了分析。发病年龄在1.5至4岁之间。发作具有多形性,发作频率高,必有半侧面部发作和非典型失神发作。67%的患者出现夜间全身性强直阵挛发作和跌倒发作。神经系统疾病谱包括言语障碍和轻微的小脑症状。脑电图显示有局灶性“中央区”活动和弥漫性癫痫样障碍,在慢波睡眠期加重。发现对抗惊厥治疗有抵抗性。作者证实了假性 Lennox 综合征在病因学上的独立性,并认为值得将其与 Landau-Kleffner 癫痫性失语症以及慢波睡眠期持续性棘波癫痫一起,在一组隐源性部分性癫痫形式中区分出来。

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