良性婴儿家族性惊厥12例分析

Analysis of 12 cases of Benign Infantile Familial Seizures

关键词:  婴儿  家族性  惊厥

Key words:  infant  infantile  convulsion

摘要:

对于良性婴儿家族性惊厥(BIFS)的认识不多，引导我们去发现其临床及脑电图特点。自1996～2004年间，作者陆续发现12例BIFS患儿，具有如下的临床表现：首发年龄3月～2岁，无异常个人史，临床、生化及放射线检查正常，生长发育正常良好。临床及脑电图特征相似：平均发生年龄6月，短暂部分性发作(经常继发全面性发作)及全面性发作，丛集性发作(平均每天发作2～10次)，平均持续2天，间歇期脑电图正常，遗传方式显示为常染色体显性遗传。家族史、临床及脑电图特征是认识该病的关键，且预后良好，临床酌情用药。

Abstract:

Benign infantile familial seizures are not well known,leading us to study their clinical and EEG characteristics. Clinical. Between 1996 and 2004, authors found 12 patients with following manifestations:convulsions occurrred between 3 month and 2 year of age, normal personal history, no abnormality on clinical, biological and radiological investigations, normal developmental outcome with at least 1 year follow-up. The clinical and EEG characteristics were the same: at the mean age of 6 months, brief partial seizures (often secondarily or apparently generalized) occuring in a cluster of two to 10 episodes a day for a mean duration of 2 days, with ictal EEG showing normal. The clinical and EEG characteristics are important in order to recognize this type of infantile convulsions, which have a good prognosis and need no aggressive treatment.