Park Jun T, Shahid Asim M, Jammoul Adham
Pediatr Ann. 2015 Feb;44(2):e30-5. doi: 10.3928/00904481-20150203-09.
Benign rolandic epilepsy (BRE), childhood idiopathic occipital epilepsy (CIOE), childhood absence epilepsy (CAE), and juvenile myoclonic epilepsy (JME) are some of the common epilepsy syndromes in the pediatric age group. Among the four, BRE is the most commonly encountered. BRE remits by age 16 years with many children requiring no treatment. Seizures in CAE also remit at the rate of approximately 80%; whereas, JME is considered a lifelong condition even with the use of antiepileptic drugs (AEDs). Neonates and infants may also present with seizures that are self-limited with no associated psychomotor disturbances. Benign familial neonatal convulsions caused by a channelopathy, and inherited in an autosomal dominant manner, have a favorable outcome with spontaneous resolution. Benign idiopathic neonatal seizures, also referred to as "fifth-day fits," are an example of another epilepsy syndrome in infants that carries a good prognosis. BRE, CIOE, benign familial neonatal convulsions, benign idiopathic neonatal seizures, and benign myoclonic epilepsy in infancy are characterized as "benign" idiopathic age-related epilepsies as they have favorable implications, no structural brain abnormality, are sensitive to AEDs, have a high remission rate, and have no associated psychomotor disturbances. However, sometimes selected patients may have associated comorbidities such as cognitive and language delay for which the term "benign" may not be appropriate.
良性中央回癫痫(BRE)、儿童特发性枕叶癫痫(CIOE)、儿童失神癫痫(CAE)和青少年肌阵挛癫痫(JME)是小儿年龄组中一些常见的癫痫综合征。在这四种综合征中,BRE最为常见。BRE在16岁时缓解,许多儿童无需治疗。CAE的癫痫发作缓解率也约为80%;而JME即使使用抗癫痫药物(AEDs)也被认为是一种终身疾病。新生儿和婴儿也可能出现自限性癫痫发作,且无相关的精神运动障碍。由离子通道病引起并以常染色体显性方式遗传的良性家族性新生儿惊厥,预后良好,可自发缓解。良性特发性新生儿惊厥,也称为“第五日惊厥”,是婴儿期另一种癫痫综合征的例子,预后良好。BRE、CIOE、良性家族性新生儿惊厥、良性特发性新生儿惊厥和婴儿良性肌阵挛癫痫被归类为“良性”特发性年龄相关性癫痫,因为它们预后良好、无结构性脑异常、对AEDs敏感、缓解率高且无相关的精神运动障碍。然而,有时部分患者可能伴有合并症,如认知和语言发育迟缓,对于这些患者,“良性”这一术语可能并不合适。