亚急性硬化性全脑炎的临床特点分析  

Analysis of clinical characteristics of subacute sclerosing panencephalitis

作　　者：张凡 李久伟[2] 石秀玉[1] 王红梅[2] 胡琳燕[1] 邹丽萍[1] Zhang Fan;Li Jiuwei;Shi Xiuyu;Wang Hongmei;Hu Linyan;Zou Liping(Department of Pediatrics,First Medical Center of PLA General Hospital,Beijing 100853,China)

机构地区：[1]解放军总医院第一医学中心儿内科,北京100853 [2]首都医科大学附属北京儿童医院神经内科,北京100853

出　　处：《北京医学》2020年第11期1066-1069,1074,共5页Beijing Medical Journal

摘　　要：目的总结亚急性硬化性全脑炎(subacute sclerosing panencephalitis,SSPE)患儿的临床病程特点,发现早期诊断线索,增加早期干预机会。方法选取2016年1月1日至2018年12月31日解放军总医院第一医学中心小儿内科和首都医科大学附属北京儿童医院神经内科确诊为SSPE并住院治疗的患儿6例,均随访至2019年12月31日或疾病终点。结果3例学龄期患儿以智力减退或夜间遗尿为Ⅰ期首发症状,其他3例学龄前期患儿无明显Ⅰ期症状。不同治疗方案均未能阻止疾病进展,3例在随访阶段进入了Ⅲ期,其中1例在Ⅲ期因重症肺炎死亡,没有患儿在随访阶段进入Ⅳ期。结论SSPE患儿临床表现多样,治疗困难。当学龄期患儿出现智力减退或遗尿时应注意麻疹感染史。通过对麻疹感染家庭针对性的医学教育可以提高SSPE的早期确诊率,增加早期干预的机会。Objective To summarize the clinical characteristics of children with subacute sclerosing panencephalitis(SSPE),to find clues to early diagnosis to increase the chance of early intervention.Methods A total of six children who were diagnosed with SSPE and admitted to the First Medical Center of PLA General Hospital and Beijing Children’s Hospital,Capital Medical University in the period from January 1st,2016 to December 31st,2018.They were all followed up until December 31st,2019 or the end of the disease.Results Three school-age children showed obvious symptoms in stageⅠwith mental retardation or nocturnal enuresis,while other preschool children behaved normally without evident symptom in stageⅠ.Different treatment regimens had failed to stop the disease from progressing.Three children entered stageⅢduring follow-up and one of them died of severe pneumonia.No child had entered stageⅣ.Conclusions SSPE has various clinical manifestations and is difficult to treat.The history of measles infection should be worthy of special attention in school-age children with mental retardation or nocturnal enuresis.Early diagnosis rate of SSPE can be improved and opportunities for early intervention can be increased through specific medical education for targeted families in which measles infection once happened.

关 键 词：亚急性硬化性全脑炎 临床特征 早期诊断 治疗 

分 类 号：R742.9[医药卫生—神经病学与精神病学]