摘要
目的探讨特发性枕叶癫伴睡眠期癫性电持续状态(ESES)患儿的临床表现、EEG特征、治疗反应及预后。方法对8例特发性儿童枕叶癫伴ESES患儿的临床及EEG资料进行分析,并随访其治疗效果及预后。结果本组8例患儿起病年龄为5岁8个月～8岁4个月。8例患儿就诊时均有神经心理损伤及运动倒退,均无相关家族癫病史,头颅影像学检查均未见明显异常。7例有癫发作,表现为醒后局限运动性发作;清醒期亦可发作,发作形式包括局限运动性发作(5/7例)、全面强直-阵挛发作(2/7例)。4例患儿经抗癫药物(AEDs)治疗发作减少,但其神经心理损伤及运动倒退无明显改善;3例患儿经单药奥卡西平治疗神经心理损伤及EEG有轻度改善;7例静脉应用甲泼尼龙冲击治疗,其中6例癫发作控制理想,1例神经心理损伤及运动倒退明显好转。随访8例患儿EEG 0.5～2.0 a,2例ESES现象有改善。至随访时患儿智力均较健康同龄儿低下。结论特发性儿童枕叶癫是一种年龄依赖性的儿童良性癫,若其EEG显示ESES现象,AEDs治疗往往难以显效;激素可减少临床发作,但对神经心理损伤及EEG ESES现象改善不明显。对皮质激素反应欠佳的儿童可尝试单药奥卡西平或联合治疗。
Objective To investigate the clinical manifestations, electroencephalogram（EEG） characteristics, treatment response and prognosis of children with idiopathic occipital epilepsy and electrical status epilepticus in sleep （ESES）. Methods Clinical alad EEG data of 8 cases with idiopathic occipital epilepsy and ESES were analyzed together with follow - up for treatment and prognosis. Results The onset age varied from 5 years and 8 months to 8 years and 4 months in the 8 cases. All the 8 cases had neuropsychological injury and motor regres- sion, but had no history of family epilepsy and clear abnormalities by brain imaging examination. Seven patients had seizures, performing exer- cise - induced limited seizures after waking up ; seizures could also attack during awaking period, including exercise - induced limited seizures （5/7 cases） and overall tonic -elonie seizures （2/7 cases）. Antiepileptic drugs （AEDs） reduced attacks in 4 cases, but did not improve the psychological damage to nerve and motor regression obviously. Mild improvement in neuropsychologieal injury and EEG were observed in 3 cases treated with single drug Oxearbazepine （OXC）. Six cases,who were treated with intravenous methylprednisolone shock therapy, had ideal control, and 1 case had marked improvement of nerve injury and psychological setback in movement. Eight patients were followed up by EEG for 0.5 - 2.0 years, and 2 cases improved in ESES phenomenon. Up to follow - up, the 8 cases of patients still had mental retardation compared with healthy children in the same age. Conclusions Idiopathic occipital epilepsy is an age - dependent benign epilepsy in chil- dren ; AEDs are often ineffective when the EEG showes ESES of the patients ; hormones can reduce the clinical attack, but can not improve the psychological nerve damage and phenomenon of EEG ESES. Children with poor response to eortieosteroids can be treated with OXC monothe- rapy or combined therapy.
出处
《实用儿科临床杂志》
CAS
CSCD
北大核心
2012年第24期1874-1876,共3页
Journal of Applied Clinical Pediatrics
