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目的探讨癫伴慢波睡眠期持续棘慢波(CSWS)患儿的临床表现、EEG特征、治疗反应及预后。方法对16例CSWS患儿的临床及EEG资料进行分析,并随访治疗效果及预后。结果本组16例患儿起病年龄3岁8个月～8岁4个月。16例均有癫发作,患儿均有睡眠中局限运动性发作;清醒期发作形式包括不典型失神发作(8/16例)、局限运动性发作(8/16例)、失张力发作(4/16例)、肌阵挛发作(4/16例)及全面强直-阵挛发作(1/16例)。16例均有神经心理损伤及运动倒退。5例为早产,2例有癫家族史,1例诊断为异型苯丙酮尿症。16例患儿EEG均存在睡眠期电持续状态(ESES)现象。8例患儿MRI存在异常,包括大脑皮质发育异常、脑萎缩各2例,先天性胼胝体发育不良、双侧壳核后部异常信号各1例。抗癫药物(AEDs)可减少部分患儿(4/16例)的发作,但对神经心理损伤及运动倒退无效。16例均静脉应用甲泼尼龙冲击治疗,13例癫发作控制,12例神经心理损伤及运动倒退情况明显好转。复查EEG,多数患儿(14/16例)ESES明显改善。随访所有患儿,其中3例4～6岁患儿仍有临床发作,余均无临床发作。8例ESES有复发,并出现认知及运动倒退,其中6例再次予甲泼尼龙冲击治疗,4例临床及EEG均明显好转。至随访时患儿智力均较健康同龄儿低下。结论 CSWS是一种少见的年龄依赖性的儿童癫,临床均有癫发作、神经心理损伤及运动倒退,EEG存在ESES现象。AEDs可减少临床发作,但对神经心理损伤及运动倒退改善不明显。甲泼尼龙冲击治疗可明显改善神经心理损伤、运动倒退情况及EEG的ESES现象。EEG及癫发作转归良好,但常遗留神经心理损伤及运动倒退。
Objective To investigate the clinical manifestations, EEG features, treatment response and prognosis in children with epilepsy accompanied by slow spikes and slow wave waves (CSWS). Methods The clinical and EEG data of 16 children with CSWS were analyzed, and the treatment effect and prognosis were followed up. Results The group of 16 patients with onset age of 3 years and 8 months to 8 years and 4 months. 16 cases had epileptic seizures, children with locomotor locomotor activity were seizures. During the awake period, seizures included atypical deafness episodes (8/16 cases), localized motor attacks (8/16 cases), delayed onset seizures 4/16 cases), myoclonic seizures (4/16 cases) and full tonic-clonic seizures (1/16 cases). All 16 patients had neuropsychological impairment and motor regression. Five were premature, two had a family history of epilepsy, and one had idiopathic phenylketonuria. EEG in all 16 children had persistent sleep state (ESES) phenomenon. There were abnormalities of MRI in 8 cases, including 2 cases of cerebral cortex dysplasia, 2 cases of cerebral atrophy, 1 case of congenital corpus callosum dysplasia and 1 case of abnormal signal of bilateral posterior putamen. Antiepileptic drugs (AEDs) can reduce the number of children (4/16 cases) attack, but neuropsychological damage and exercise regress ineffective. 16 cases were treated with intravenous methylprednisolone pulse therapy, 13 cases of epileptic seizures control, 12 cases of neuropsychological damage and reversal of exercise was significantly improved. EEG review, the majority of children (14/16 cases) ESES significantly improved. All children were followed up, of which 3 cases of children aged 4 to 6 years still have clinical seizures, no more than no clinical attack. 8 cases of ESES recurrence, and cognitive and motor regression, of which 6 cases were treated with methylprednisolone again, 4 cases of clinical and EEG were significantly improved. At follow-up, children’s intelligence was lower than that of healthy peers. Conclusion CSWS is a rare age-dependent childhood epilepsy. Epileptic seizures, neuropsychological impairment and motor regression are all found in CSWS. ESES exists in EEG. AEDs can reduce the clinical attack, but the improvement of neuropsychological injury and exercise is not obvious. Methylprednisolone treatment can significantly improve the neuropsychological injury, exercise reversal and EES ESES phenomenon. EEG and epileptic seizures well, but often left neuropsychological damage and exercise regress.