儿童急性脑病伴双相发作及后期弥散降低的临床与影像学特点  

Clinical and imaging features of acute encephalopathy with biphasic seizures and late reduced diffusion in children

作　　者：张美姣 林丽 王文徽[3] 李文辉 魏翠洁[1] 谢涵[1] 章清萍[1] 吴晔[1] 熊晖[1] 周水珍 杨斌 包新华[1] Zhang Meijiao;Lin Li;Wang Wenhui;Li Wenhui;Wei Cujie;Xie Han;Zhang Qingping;Wu Ye;Xiong Hui;Zhou Shuizhen;Yang Bin;Bao Xinhua(Department of Pediatrics,Peking University First Hospital,Beijing 100034,China;Department of Neurology,Provincial Children′s Hospital Affiliated to Anhui Medical University,Hefei 230051,China;Department of Neurology,Shanxi Children′s Hospital,Taiyuan 030013,China;Department of Neurology,Children′s Hospital of Fudan University,National Children′s Medical Center,Shanghai 201102,China)

机构地区：[1]北京大学第一医院儿科,北京100034 [2]安徽省儿童医院儿童神经科,合肥230051 [3]山西省儿童医院神经内科,太原030013 [4]国家儿童医学中心、复旦大学附属儿科医院神经科,上海201102

出　　处：《中华儿科杂志》2023年第11期989-994,共6页Chinese Journal of Pediatrics

摘　　要：目的探讨儿童急性脑病伴双相发作及后期弥散降低(AESD)的临床与影像学特点。方法病例系列研究,选择2021年10月至2023年7月北京大学第一医院、安徽省儿童医院、复旦大学附属儿科医院和山西省儿童医院就诊的21例AESD患儿作为研究对象。总结其临床资料包括影像学、实验室检查以及治疗情况和预后特点。运用描述性统计分析。结果21例AESD患儿中男11例、女10例,起病年龄为2岁6月龄(1岁7月龄,3岁6月龄)。具有双相发作的典型病例18例,均为发热24 h内出现早期发作,其中16例为全面性发作、2例为局灶性发作,7例达惊厥持续状态。仅有双相中的晚期发作的非典型病例3例。21例患儿的晚期发作出现在病程的第3~9天,其中12例为局灶性发作、6例为全面性发作、3例有局灶和全面性两种发作形式。21例患儿在病程第3~11天头颅磁共振弥散加权成像(DWI)上均可见皮层下白质弥散受限,即“亮树征”。19例患儿在病程第12天至3个月存在额、顶、枕叶为主的弥漫性脑萎缩。20例患儿曾被误诊为自身免疫性脑炎、中枢神经系统感染、热性惊厥、可逆性后头部脑病综合征、急性播散性脑脊髓炎和偏侧惊厥-偏瘫-癫痫综合征。21例患儿均予糖皮质激素冲击及大剂量丙种球蛋白治疗,效果欠佳。至2023年7月,在访18例患儿中17例遗留不同程度的神经系统后遗症,其中11例存在脑病后癫痫;1例恢复正常。结论AESD以双相发作及影像学“亮树征”为特点,以对症、支持治疗为主,免疫治疗无肯定疗效。神经系统后遗症发生率高,病死率较低。Objective To explore the clinical and imaging features of acute encephalopathy with biphasic seizures and late reduced diffusion(AESD)in children.Methods For the case series study,21 children with AESD from Peking University First Hospital,Provincial Children′s Hospital Affiliated to Anhui Medical University,Children′s Hospital of Fudan University,and Shanxi Children′s Hospital who were diagnosed and treated from October 2021 to July 2023 were selected.Clinical data were collected to summarize their clinical information,imaging,and laboratory tests,as well as treatment and prognostic characteristics.Descriptive statistical analysis was applicated.Results Of the 21 cases with AESD,11 were males and 10 were females,with the age of onset of 2 years and 6 months(1 year and 7 months,3 years and 6 months).Of the 21 cases,18 were typical cases with biphasic seizures.All typical cases had early seizures within 24 hours before or after fever onset.Among them,16 cases had generalized seizures,2 cases had focal seizures,and 7 cases reached the status epilepticus.Of the 21 cases,3 atypical cases had late seizures in biphasic only.The late seizures in the 21 cases occurred on days 3 to 9.The types of late seizures included focal seizures in 12 cases,generalized seizures in 6 cases,and both focal and generalized seizures in 3 cases.Diffusion-weighted imaging(DWI)test on days 3 to 11 showed reduced diffusion of subcortical white matter which was named"bright tree sign"in all cases.The diffuse cerebral atrophy predominantly presented in the front-parietal-temporal lobes was found in 19 cases between day 12 and 3 months after the onset of the disease.Among 21 cases,20 had been misdiagnosed as autoimmune encephalitis,central nervous system infection,febrile convulsions,posterior reversible encephalopathy syndrome,acute disseminated encephalomyelitis,and hemiconvulsion-hemiplegia-epilepsy syndrome.All the cases received high-dose gammaglobulin and methylprednisolone pulse therapy with poor therapeutic effect.By July 2023,18 c

关 键 词：儿童 急性脑病 急性脑病伴双相发作及后期弥散降低 亮树征 

分 类 号：R742[医药卫生—神经病学与精神病学]