CAJ | 학술논문

目的：总结亚急性硬化性全脑炎（SSPE）的临床表现、影像学特征、脑脊液和脑电图改变以及预后，以提高对该病的认识。方法对福建省立医院收治的2例 SSPE 患儿的临床资料进行回顾性分析，并复习文献。结果收集文献40篇，病例数加本文2例共计116例。SSPE 好发于儿童、青少年时期（5~20岁），男：女=3.7：1，部分患者幼儿时期有麻疹病史，典型病例隐袭起病，慢性进展。病理特征是脑内广泛炎性反应和核内包涵体。颅脑 CT 或 MRI可见颅内多发脱髓鞘病灶。脑脊液和血清麻疹病毒抗体滴度和比值升高。特征性脑电图为广泛性周期复合慢波。目前尚无特效治疗方法，预后差。结论 SSPE 早期诊断困难，典型病例依据临床症状、影像学检查、脑电图和脑脊液改变可临床诊断。
목적：총결아급성경화성전뇌염（SSPE）적림상표현、영상학특정、뇌척액화뇌전도개변이급예후，이제고대해병적인식。방법대복건성립의원수치적2례 SSPE 환인적림상자료진행회고성분석，병복습문헌。결과수집문헌40편，병례수가본문2례공계116례。SSPE 호발우인동、청소년시기（5~20세），남：녀=3.7：1，부분환자유인시기유마진병사，전형병례은습기병，만성진전。병리특정시뇌내엄범염성반응화핵내포함체。로뇌 CT 혹 MRI가견로내다발탈수초병조。뇌척액화혈청마진병독항체적도화비치승고。특정성뇌전도위엄범성주기복합만파。목전상무특효치료방법，예후차。결론 SSPE 조기진단곤난，전형병례의거림상증상、영상학검사、뇌전도화뇌척액개변가림상진단。
Objectjve To analyze the clinical and imaging characteristics,changes of cerebrospinal fluid and electroencephalogram,as well as prognosis of subacute sclerosing panencephalitis(SSPE)in order to improve the understanding of the disease. Methods The clinical data of two SSPE patients admitted to Fujian Provincial Hospital were retrospectively analyzed,and the literatures were reviewed. Results A total of 40 literatures and 116 cases(including 2 cases of this study) were collected. SSPE was prone to occur among children and adolescence(5 to 20 years old),and the proportion of male and female was 3. 7: 1. Some of the patients had history of measles in early childhood,and SSPE typically had insidious onset and chronic progress. The pathological features of the disease were extensive inflammation inside the brain and intranuclear inclusions. Brain CT or MRI showed multiple intracranial demyelination lesions. The measles antibody titer and ratio in cerebrospinal fluid and serum were increased. The characteristics of electroencephalographic( EEG)were widely periodic slow wave complexes. There was no effective treatment to completely cure SSPE at present,and the prognosis was poor. Conclusjon The diagnosis of SSPE is often difficult in the early stages. In a typical case,diagnosis is based on clinical symptoms,brain imaging,EEG and CSF findings.