Caraballo R, Cersósimo R, Galicchio S, Fejerman N
Servicio de Neurologia, Hospital de Pediatría, Garrahan, Buenos Aires, Argentina.
Rev Neurol. 1997 May;25(141):682-4.
Benign Infantile Familial Convulsions (HBIFC), are characterized by brief partials seizures, occasionally with secondary generalization, with onset in the first year of life, family history of similar electroclinical seizures and same age of appearance.
We presented 16 patients, 10 girls and 6 boys, evaluated in our Service between 1990-1996. We analyzed, age of onset of the seizures, sex, family history of epilepsy, neurologic exam, semiology, distribution, frequency and duration of the seizures, EEG, neuroradiologic studies and evolution.
The patients had partial seizures, which occurred mainly in clusters, with onset ranged from 3 to 8 months, with normal neurological exam and psychomotor development. The interictal EEG was normal and the course was benign. Treatment response to antiepileptic drugs was good.
Our presentation confirmed that BIFC are a new partial idiopathic epileptic syndrome, with a genetic predisposition, probably with an autosomal dominant inheritance, which would be recognize in the next international classification of epilepsy and epileptic syndromes.
良性婴儿家族性惊厥（BIFC）的特征为短暂的局灶性发作，偶尔继发全面性发作，发作始于生命的第一年，有类似电临床发作的家族史且发病年龄相同。
我们报告了1990年至1996年间在我们科室评估的16例患者，其中10例女孩，6例男孩。我们分析了发作的起始年龄、性别、癫痫家族史、神经系统检查、发作症状学、发作分布、频率和持续时间、脑电图、神经放射学检查及病情演变。
患者出现局灶性发作，主要成簇发作，发作起始年龄为3至8个月，神经系统检查及精神运动发育正常。发作间期脑电图正常，病程呈良性。对抗癫痫药物治疗反应良好。
我们的报告证实BIFC是一种新的局灶性特发性癫痫综合征，具有遗传易感性，可能为常染色体显性遗传，这将在下一版国际癫痫及癫痫综合征分类中得到认可。
