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[小儿失神癫痫综合征与青少年失神癫痫综合征差异的前瞻性研究]

[Prospective study of the differences between the syndromes of infantile absence epilepsy and syndromes of juvenile absence epilepsy].

作者信息

Oller L

机构信息

Centro Antiepilèptico de Barcelona, Escuelas Pias.

出版信息

Rev Neurol. 1996 Aug;24(132):930-6.

PMID:8755355
Abstract

In this prospective study of the differences between the epileptic syndrome of absence attacks in the child (EAN) and the syndrome of juvenile absence attacks (EAJ), the author considers the characteristics of these syndromes in order to differentiate their various aspects, namely clinical features, complementary tests, course and prognosis. These cases are from a series of 6,299 epileptic patients. One of the objectives of a prospective study begun in 1970 was to evaluate the clinical course of all cases in which valproic acid was used. Valproic acid is known to be most effective in the basic crises of these two syndromes, namely the typical absence attacks. It is therefore an analysis of the clinical features and their subsequent course and prognosis when thus treated, since a retrospective study including a much larger number of patients would not be considered to be satisfactory in view of the 'bias' due to only some patients having access to treatment with this drug. The author analyzes 138 cases of EAN and 42 cases of EAJ seen between 1970 and the end of 1995. These syndromes, particularly EAN, have always been considered to have an excellent prognosis. The author tries to show from the point of view of long term follow-up, that absence attacks may not only persist into adult life but also be associated with other types of crises which worsen the prognosis. Thus, for instance, in EAN 50% of the cases show generalized tonic-clonic seizures on long-term follow-up. In EAJ the corresponding figure is 76.1%. This data modifies previous ideas as to the benign course of these syndromes when they are followed-up for a long period. The basic criteria were: a first visit after 1970 and an age of onset between 3 and 10 years for EAN and over 10 years for EAJ. In all cases there was the following conditions, onset by absence attacks, lack of permanent neurological or psychological disorders, and a video-EEG recording of a typical generalized EEG during an attack.

摘要

在这项关于儿童失神发作癫痫综合征(EAN)和青少年失神发作综合征(EAJ)差异的前瞻性研究中,作者考量了这些综合征的特征,以便区分其各个方面,即临床特征、辅助检查、病程和预后。这些病例来自6299例癫痫患者。1970年开始的一项前瞻性研究的目标之一是评估所有使用丙戊酸的病例的临床病程。已知丙戊酸对这两种综合征的基本发作,即典型失神发作最为有效。因此,这是对接受该治疗时的临床特征及其后续病程和预后的分析,因为鉴于仅部分患者能够使用该药物治疗所导致的“偏差”,一项纳入更多患者的回顾性研究将被认为是不令人满意的。作者分析了1970年至1995年底期间所见的138例EAN病例和42例EAJ病例。这些综合征,尤其是EAN,一直被认为预后良好。作者试图从长期随访的角度表明,失神发作不仅可能持续至成年期,还可能与其他类型的发作相关,从而使预后恶化。例如,在EAN中,50%的病例在长期随访中出现全身性强直阵挛发作。在EAJ中,相应的数字是76.1%。这一数据改变了以往关于这些综合征长期随访时良性病程的观点。基本标准为:1970年后首次就诊,EAN的发病年龄在3至10岁之间,EAJ的发病年龄超过10岁。所有病例均具备以下条件:由失神发作起病,无永久性神经或心理障碍,且发作期间有典型全身性脑电图的视频脑电图记录。

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