摘要
目的总结急性脑病伴双相发作及后期弥散减低(AESD)的临床、影像特征及预后。方法回顾性分析华中科技大学同济医学院附属武汉儿童医院神经内科及康复医学科自2020年1月至2022年4月收治的8例AESD患儿的临床、影像学资料及随访情况。结果8例患儿的前驱感染症状以消化系统、呼吸系统感染为主,均在发热后24 h内出现惊厥发作;病程呈双相,其中第一相病程中3例出现惊厥持续状态,第二相病程中均表现为丛集性发作伴意识障碍加重,4例伴不自主动作。7例患儿被收入重症监护室,4例行气管插管,1例合并脓毒性休克。8例患儿第二相病程中颅脑MRI均示"亮树征",包括双侧对称性受累和双侧非对称性受累,受累部位分为弥漫性和局灶性。8例患儿均予糖皮质激素和(或)丙种球蛋白治疗,2例予亚低温脑保护。出院后随访,患儿恢复期(3个月以上)颅脑MRI复查示脑萎缩、硬膜下积液、脑软化等改变,6个月时8例患儿均存活,但均遗留不同程度神经系统后遗症,扩展格拉斯哥预后量表评分4分2例、5分4例、7分2例。结论AESD是一种特殊的临床影像综合征,双相惊厥发作、头颅MRI示"亮树征"为其特征性的临床及影像表现。该病患儿存活率高,但易遗留不同程度的神经系统后遗症。
Objective To summarize the clinical-imaging features and prognoses of acute encephalopathy combined with biphasic seizures and late reduced diffusion(AESD).Methods A retrospective analysis was performed.The clinical and imaging data and follow-up results of 8 children with AESD,admitted to Departments of Neurology and Rehabilitation Medicine,Wuhan Children's Hospital Affiliated to Tongji Medical College,Huazhong University of Science and Technology from January 2020 to April 2022 were collected.Results The antecedent infections in 8 children were predominantly gastrointestinal and respiratory tract infections and convulsive seizures occured within 24 h of fever.The clinical presentation was biphasic:3 had sustained convulsive state in the first phase of the disease,and all showed cluster seizures with worsening impairment of consciousness in the second phase of the disease,including 4 with involuntary movements during recovery from the disease.Seven children required admission to Intensive Care Unit,4 required tracheal intubation for assisted ventilation,and 1 was combined with septic shock.Brain MRI findings of 8 children were"bright tree"in the second phase of the disease,including bilateral symmetrical and bilateral asymmetrical involvements,with diffuse and focal involvements.All 8 children were treated with glucocorticoids and/or gammaglobulin,and 2 were given mild hypothermia brain protection.Follow-up was performed for more than 3 months,and brain MRI indicated cerebral atrophy,subdural effusion and cerebral malacia.All the 8 children survived with different degrees of sequelae.Two children had Extended Glasgow Outcome Scale scores of 4,4 had scores of 5,and 2 had scores of 7.Conclusion AESD is a special clinical-maging syndrome,characterized by biphasic seizure and"bright tree"on head MRI;the survival is high,but neurological sequelae of different degrees are easily left behind.
作者
黄山
何雨洋
朱红敏
Huang Shan;He Yuyang;Zhu Hongmin(chool of Medicine,Wuhan University of Science and Technology,Wuhan 430016,China;Department of Rehabilitation Medicine,Wuhan Children's Hospital Affiliated to Tongji Medical College,Huazhong University of Science and Technology,Wuhan 430016,China)
出处
《中华神经医学杂志》
CSCD
北大核心
2023年第11期1136-1143,共8页
Chinese Journal of Neuromedicine
基金
湖北省科技计划立项项目 (2022DCC020)。
关键词
急性脑病
急性脑病伴双相发作及后期弥散减低
儿童
Acute encephalopathy
Acute encephalopathy combined with biphasic seizures and late reduced diffusion
Child
