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[中央脑性肌阵挛-无动性癫痫小发作。52例患者的临床与脑电图长期随访研究(作者译)]

[Centrencephalic myoclonic-astatic petit mal. Clinical and electroencephalographic long-term follow-up study in 52 patients (author's transl)].

作者信息

Langenstein I

出版信息

EEG EMG Z Elektroenzephalogr Elektromyogr Verwandte Geb. 1978 Jun;9(2):86-96.

PMID:97076
Abstract

In this report 52 patients meeting the criteria of centrencephalic myoclonic-astatic petit mal (10) at the beginning of petit mal are included. The results of clinical and encephalographic follow-up examinations are as follows: 1) The type reported here apparently has a petit mal course with peculiar characteristics, it therefore must be separated from Lennox syndrome: centrencephalic myoclonicastatic petit mal, pyknolepsia, bilateral myoclonus (impulsive petit mal). It should file under generalized primary petit mal epilepsy. 2) It is primarily defined by its EEG marker: "centrencephalic" EEG pattern (irregular and/or regular spike-wave groups, photosensibility and abnormal theta- and/or delta-rhythm). Rarely (33%) minor cerebral organic lesions as additional pathogenetic factors are uncovered by clinical and electroencephalographic examinations. 3) The clinical picture is characterized, aside from myoclonic and/or astatic seizures, by frequent absences (80%), rare tonic seizures (6%), petit mal status (25%) and mostly generalized grand mal seizures (62%). 4) There are changes of the course of the disease to Lennox syndrome (N = 6) in in children suffering from marked cerebral organic lesions at the onset of petit mal and in development of severe epilepsy. 5) Least favorite markers with respect to prognosis are concomitant grand mal seizures (p = 0,05), petit mal status (p = 0.008), additional 2/sec spike wave-pattern (spike wave Variant) in the EEG (p = 0.002) and previous seizures with focal signs. Favourite outcome of epilepsy are frequently connected to missing cerebral organic lesions (p = 0.05).

摘要

本报告纳入了52例在失神发作初期符合中央脑性肌阵挛 - 无张力性小发作(10例)标准的患者。临床和脑电图随访检查结果如下:1)此处报告的类型显然具有失神发作病程且有独特特征,因此必须与Lennox综合征区分开来:中央脑性肌阵挛 - 无张力性小发作、密集性癫痫、双侧肌阵挛(冲动性小发作)。它应归类于全身性原发性小发作癫痫。2)其主要由脑电图标志物定义:“中央脑性”脑电图模式(不规则和/或规则的棘波 - 慢波群、光敏性以及异常的θ和/或δ节律)。临床和脑电图检查很少(33%)发现轻微脑器质性病变作为额外的致病因素。3)临床表现的特征是,除了肌阵挛和/或无张力性发作外,频繁失神发作(80%)、罕见的强直发作(6%)、小发作持续状态(25%)以及大多为全身性大发作(62%)。4)在失神发作初期患有明显脑器质性病变且发展为严重癫痫的儿童中,疾病进程会转变为Lennox综合征(N = 6)。5)关于预后最不利的标志物是伴有大发作(p = 0.05)、小发作持续状态(p = 0.008)、脑电图中额外的2次/秒棘波模式(棘波变体,p = 0.002)以及既往有局灶性体征的发作。癫痫的良好预后常与无脑器质性病变相关(p = 0.05)。

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