儿童失神癫240例治疗及预后随访报告:应用改良的1989年儿童失神癫诊断标准

目的总结儿童失神癫(CAE)的治疗和预后,为CAE的合理用药和评价远期预后提供依据。方法对1999年10月至2005年12月北京市3家医院为研究CAE易感基因收集的CAE患儿的治疗用药、疗效及预后进行随访。CAE诊断标准参考1989年国际抗癫联盟(ILAE)提出的癫及癫综合征分类诊断标准,并制定了统一的CAE纳入标准和排除标准。根据CAE的选药原则进行治疗,评估CAE患儿的远期预后。结果3家医院共收集符合ILAECAE诊断标准的患儿339例,其中296例符合本研究制定的CAE纳入标准。296例患儿中有56例患儿因失访未得到远期预后随访结果,有随访结果者240例(81.1%),其中男94例(39.2%),女146例(60.8%)。失神发作起病年龄为3岁3个月至12岁,其中4～8岁174例(72.5%)。39例(16.2%)有热性惊厥史,18例(7.5%)有热性惊厥家族史和(或)癫家族史,5例(2.1%)有失神癫家族史。失神癫发作频率为每日5～50次,其中每日发作10～30次占80%。出现失神持续状态1例。伴全面强直-阵挛发作12例(5.0%)。所有患儿发作期EEG均表现为双侧对称同步的3Hz棘慢波爆发,头颅影像学检查均未见异常。治疗首选丙戊酸234例,其中217例(92.7%)于服药后3d至6个月发作完全控制,15例加用另一种药物(其中氯硝西泮7例、硝西泮4例及拉莫三嗪4例)后发作控制,余2例单用丙戊酸2年后仍有发作,但发作次数明显减少;首选拉莫三嗪4例,其中3例发作控制,1例服药1年发作未控制,改用丙戊酸1周后发作控制。2例首选托吡酯治疗,其中1例发作控制,1例服药5个月效果不明显,改用丙戊酸2个月后发作控制。240例患儿随访时间为2～7年,158例(65.8%)已停用抗癫药物,其中停药1年以上者96例,停药后均无复发;82例尚未停用抗癫药物患儿中,80例发作完全控制2年以上,仅有2例仍有失神发作。随访的240例患儿在校学习成绩为中等及以上者有171例(71.3%)。结论丙戊酸是治疗CAE的首选药物,对绝大多数患儿疗效好。少数用丙戊酸发作未控制者可选用拉莫三嗪或苯二氮类药物。典型CAE患儿远期预后良好。

Treatment and prognosis of childhood absence epilepsy in 240 patients:Application of modified diagnostic criteria of childhood absence epilepsy 1989

Objective Childhood absence epilepsy(CAE)is one of the idiopathic generalized epilepsy syndrome,characterized by multiple typical absence seizures per day.The aim of this study was to investigate the effectiveness of medical treatment and prognosis of CAE patients from three hospitals in Beijing.Methods All patients were collected from the three hospitals between October 1999 and December 2005 as part of a research project to identify susceptibility genes of CAE with the same inclusion and exclusion criteria.The diagnostic criteria of CAE were proposed by the International League Against Epilepsy(ILAE) in 1989.Patients were treated in the three different hospitals.Doctors who collected the patients made a decision of choice of antiepileptic drugs.The effectiveness of medical treatment on seizures and the prognosis of the CAE patients were followed up.Results In a cohort of 339 patients fulfilled the diagnostic criteria of CAE proposed by the ILAE in 1989,296 of 339 patients met the inclusion criteria of this study,outcome was available in 240 of 296 patients.Among 240 patients,94 were males(39.2%) and 146 were females(60.8%).The onset age of absence seizures ranged from 3 years and 3 months to 12 years,with a peak around 4 to 8 years(72.5%) of age.Thirty nine patients(16.2%) had a history of febrile seizures.Eighteen patients(7.5%) had a family history of febrile seizures or epilepsy.Twelve patients(5%) subsequently had several generalized tonic-clonic seizures.One patient had a history of absence status epilepticus.All patients had electroencephalogram pattern with a bilateral symmetric and synchronous discharge of regular 3Hz spike-wave discharges after spontaneity or hyperventilation.Valproic acid(VPA) was the first choice of drug therapy in 234 patients.Seizures were controlled in 217 out of 234 patients by VPA monotherapy.Fifteen of 234 patients received clonazepam(7),nazepam(4),or lamotrigine(4) as an add-on therapy.All of their seizures were controlled.Lamotrigine was the first choice in 4 cases,3 of them were completely controlled.Topiramate was the first choice in 2 cases,one was also completely controlled.All patients were followed up for 2 to 7 years.One hundred fifty eight patients(65.8%) had withdrawn antiepileptic drugs,complete remission maintained for more than 1 year after withdrawal of medication in 96 patients.No seizure relapsed in patients after medication withdrawal.Complete remission maintained for more than 2 years on medication in 80 patients.Only 2 patients still had seizures on medication.Learning score in the school was beyond the middle level in 171 patients(71.3%).Conclusions VPA is the first-line drug for CAE.It is effective in the majority of patients.Lamotrigine and benzodiazepines appear to be effective in resistant absence seizures in combination with VPA.Typical CAE is an epileptic syndrome with an excellent prognosis.