摘要
目的探讨儿童晚发型痫性痉挛的病因、临床-电生理特点、疗效及预后。方法对湖南省儿童医院神经内科住院部2015年1月至2019年12月期间临床诊断为晚发型痫性痉挛的102例患儿进行回顾性研究,收集其临床资料、实验室检查结果、治疗方案,选取其中84例有门诊随访资料者,收集其治疗转归及预后情况等。结果102例患儿中,男性68例,女性34例,起病年龄1岁至11岁零3个月(中位数25个月)。颅内感染、围生期脑损伤、脑发育异常为已知主要病因,部分病因不明。根据有无明确病因分为症状组及隐源组,两组在年龄、性别、发作形式、有无高度失律等分布上比较差异无显著意义。隐源组以成串性痉挛发作居多,症状组多表现为孤立性痉挛发作。两组4周短期疗效中总体有效率为59.8%,其中隐源组有效率(73.8%)高于症状组(50%)。促肾上腺皮质激素(ACTH)总体有效率为80.6%,隐源组ACTH治疗组的有效率为88.9%,显著高于抗癫痫药物组及甲泼尼龙组。隐源组单纯痉挛发作的患儿治疗4周有效率显著高于合并其余发作形式组。结论晚发型痫性痉挛的发生主要与后天获得性脑损伤、颅内结构异常、中枢神经系统感染有关,痉挛成串或孤立发作,发作间期脑电图多无典型高度失律,隐源性晚发型痫性痉挛对ACTH治疗敏感,建议动态评估影像学检测了解有无颅内结构异常及完善基因检测。
Objective To investigate the etiology,clinical and electrophysiological characteristics,curative effect and prognosis of children with late-onset epileptic spasms(LOS).Methods A retrospective study was conducted on 102 children with LOS clinically diagnosed in the Department of Neurology of Hunan Children's Hospital from January 2015 to December 2019.The clinical data,laboratory examination results and treatment plan were collected.84 cases of them with outpatient follow-up data were selected and their treatment outcomes and prognosis were collected.Results 102 children with LOS were enrolled,including 68 males and 34 females,with the onset age ranging from 1 year to 11 years and 3 months(median age:25 months).The main causes were known as intracranial infection,perinatal brain injury and cerebral dysplasia,and the causes of 42 cases was unknown.The patients were divided into the symptom group and the cryptogenic group according to whether the etiology was obvious.The distribution of age,sex,onset form and absence of peak loss was not significantly different between the two groups.Cluster spasmodic seizures were most common in the cryptogenic group,while isolated spasmodic seizures were most common in the symptom group.The overall effective rate of 4-week short-term therapy was 59.8%in the two groups,and the effective rate of the cryptogenic group group(73.8%)was higher than that of the symptom group(50%).The overall effective rate of ACTH was 80.6%,and the effective rate of ACTH treatment group was 88.9%,which was significantly higher than that of AEDs group and methylprednisolone group.The 4-week effective rate in the cryptogenic group with spasm alone was significantly higher than that in the patients with other onset forms.Conclusion LOS is mainly related to acquired brain injury,intracranial structural abnormality and central nervous system infection.The spasm manifested as cluster or single seizures.The hypsarrhythmia of background was rare in EEG.Cryptic LOS is sensitive to corticotropin therapy.It is recommended to evaluate the imaging findings dynamically so as to find out intracranial structural abnormalities and to improve gene detection.
作者
廖彩时
康庆云
杨理明
LIAO Caishi;KANG Qingyun;YANG Limin(Department of Neurology,Hunan Children's Hospital,Changsha(410007),Hunan China)
出处
《癫痫与神经电生理学杂志》
2021年第4期231-237,共7页
Journal of Epileptology and Electroneurophysiology(China)
基金
湖南省卫生计生委科研课题(NO:B20180528)。
