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[良性婴儿癫痫的发病率、症状学及预后]

[Frequency, semiology and prognosis of benign infantile epilepsy].

作者信息

Ramos-Lizana J, Martinez-Espinosa G, Rodriguez-Lucenilla M I, Aguirre-Rodriguez J, Aguilera-Lopez P

机构信息

Hospital Torrecardenas, 04009 Almeria, Espana.

出版信息

Rev Neurol. 2018 Apr 16;66(8):254-260.

Abstract

INTRODUCTION

Benign infantile epilepsy is an epileptic syndrome of infancy. Until now, only a small number of case-series have been published.

AIM

To study the frequency, semiology and prognosis of benign infantile epilepsy.

PATIENTS AND METHODS

The 827 patients with one or more epileptic seizures seen at our hospital between 1 June 1994 and 1 March 2011 were included and prospectively followed. A diagnosis of benign infantile epilepsy was made in patients that fulfilled the following criteria at six month of evolution: one or more focal and/or generalised seizures, onset before 24 months, no neurological deficit and normal neuroimaging and interictal EEG.

RESULTS

77 cases (9%) met the diagnostic criteria. Semiology of the seizures was similar to that of other focal seizures in children under 24 months. 25% of the patients remained as isolated seizures. Among those with two or more seizures, the probability of achieving a 3 year initial remission without antiepileptic treatment was 86%. In the subgroup of patients with focal seizures without family history the probability was 74% and in five cases a global developmental delay/intellectual disability was detected thereafter.

CONCLUSIONS

Benign infantile epilepsy is a frequent epileptic syndrome. Semiology of seizures is not useful to characterize the syndrome. A diagnosis of benign infantile epilepsy at six month of evolution implies a reasonably good prognosis, but possibly not as good as for other self-limited epilepsies of infancy.

摘要

引言

良性婴儿癫痫是一种婴儿期癫痫综合征。迄今为止,仅有少数病例系列报道发表。

目的

研究良性婴儿癫痫的发病率、症状学及预后。

患者与方法

纳入1994年6月1日至2011年3月1日期间在我院就诊的827例有一次或多次癫痫发作的患者,并对其进行前瞻性随访。在病程6个月时,符合以下标准的患者被诊断为良性婴儿癫痫:一次或多次局灶性和/或全身性发作,发病年龄在24个月之前,无神经功能缺损,神经影像学及发作间期脑电图正常。

结果

77例(9%)符合诊断标准。发作症状学与24个月以下儿童的其他局灶性发作相似。25%的患者仅发作一次。在发作两次或更多次的患者中,未接受抗癫痫治疗而达到3年初始缓解的概率为86%。在无家族史的局灶性发作患者亚组中,该概率为74%,其中5例随后被检测出存在全面发育迟缓/智力残疾。

结论

良性婴儿癫痫是一种常见的癫痫综合征。发作症状学对该综合征的特征描述并无帮助。病程6个月时诊断为良性婴儿癫痫意味着预后相对较好,但可能不如其他婴儿期自限性癫痫。

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