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[特发性失神癫痫]

[Idiopathic absence epilepsy].

作者信息

Szczepanik E, Pakszys M

机构信息

Klinika Neurologii Dzieci i Młodziezy Instytut Matki i Dziecka ul. Kasprzaka 17a, 01-211 Warszawa.

出版信息

Przegl Lek. 2001;58 Suppl 1:32-6.

Abstract

UNLABELLED

The International Classification of Epilepsies and Epileptic Syndromes distinguishes four types of idiopathic generalized epilepsies with typical absences: childhood absence epilepsy (CAE), juvenile absence epilepsy (JAE), juvenile myoclonic epilepsy (JME) and epilepsy with generalized grand mal on awakening (EGMA). It is essential in any case when sizures occur to classify the type of epileptic syndrome in order to make prognosis and to choose correct treatment. But it is not always possible to establish diagnosis at the beginning of the disease. Often prolonged observation of the patient and evolution of clinical and EEG features lead to define the epileptic syndrome. The aim of the work was to define the type and frequency of epileptic syndromes and their long-term observation in patients with absences occurred as the first (or the only) type of seizures.

SUBJECTS AND METHODS

31 patients who experienced absences as the first (or the only) type of seizures were selected from the group of 600 subjects suffered from epilepsy admitted to the Neuropediatric Department of the National Research Institute of Mother and Child between 1987 and 1998. Epileptic Syndromes were classified according to the International Classification.

RESULTS

There were 31 (5.2%) patients (18 girls and 13 boys) with absences accurred as the first (or the only) type of seizures. The age of the onset of seizures was between 2.5 to 13.5 years (mean 9.3). Seven of them had also tonic-clonic seizures in the course of the observation. They were followed-up for 3 to 9 years (mean 9.2) till the age of 7 to 25 years (mean 18.5). Two epileptic syndromes were established according to the International Classification:--childhood absence epilepsy (CAE) in 27 (4.5%) patients;--juvenile absence epilepsy (JAE) in 4 (0.7%) patients. No one patient showed evolution of disease towards neither juvenile myoclonic epilepsy nor epilepsy with generalized grand mal on awakening during follow-up.

CONCLUSIONS

5.2% of all subjects were patients with idiopathic epilepsies who experienced absences as a first (or the only) type of seizures when absences occur one should consider first of all childhood absence epilepsy and juvenile absence epilepsy. Recognition of juvenile myoclonic epilepsy and epilepsy with generalized grand mal on awakening is less probable.

摘要

未标注

《国际癫痫及癫痫综合征分类》将四种伴有典型失神发作的特发性全身性癫痫区分开来:儿童失神癫痫(CAE)、青少年失神癫痫(JAE)、青少年肌阵挛癫痫(JME)以及觉醒时全身性大发作癫痫(EGMA)。无论何时发生癫痫发作,对癫痫综合征类型进行分类对于判断预后和选择正确治疗方法都至关重要。但在疾病初期并非总能确诊。通常对患者进行长期观察,并根据临床和脑电图特征的演变来确定癫痫综合征。这项研究的目的是确定以失神发作为首发(或唯一)发作类型的患者的癫痫综合征类型及频率,并对其进行长期观察。

研究对象与方法

从1987年至1998年期间收治于国家母婴研究所神经儿科的600例癫痫患者中,选取31例以失神发作为首发(或唯一)发作类型的患者。癫痫综合征根据国际分类进行划分。

结果

有31例(5.2%)患者(18名女孩和13名男孩)以失神发作为首发(或唯一)发作类型。癫痫发作起始年龄在2.5至13.5岁之间(平均9.3岁)。其中7例在观察过程中还出现了强直阵挛发作。他们接受了3至9年(平均9.2年)的随访,直至年龄达到7至25岁(平均18.5岁)。根据国际分类确定了两种癫痫综合征:27例(4.5%)为儿童失神癫痫(CAE);4例(0.7%)为青少年失神癫痫(JAE)。在随访期间,没有患者的病情发展为青少年肌阵挛癫痫或觉醒时全身性大发作癫痫。

结论

所有研究对象中有5.2%是特发性癫痫患者,他们以失神发作为首发(或唯一)发作类型。当出现失神发作时,首先应考虑儿童失神癫痫和青少年失神癫痫。而识别青少年肌阵挛癫痫和觉醒时全身性大发作癫痫的可能性较小。

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