摘要
目的总结分析爆发型亚急性硬化性全脑炎（SSPE）的临床和脑电图特征,以提高临床对该病的认识。方法分析2010年2月至2013年11月北京大学第一医院儿科住院的7例确诊或高度疑似SSPE患儿的社会学资料、临床表现、脑电图和实验室检查资料、治疗反应及临床转归。结果 7例患儿均以周期性失张力和（或）周期性痉挛为主要表现,无其他癫痫发作类型;脑电图持续存在长间隔（5~20 s）的广泛性周期性复合波;对各种抗癫痫药物治疗无效,类固醇皮质激素常使病情明显加重。所有患儿临床症状均呈进行性恶化,快速进入昏迷和去皮质强直状态,并在2~6.5个月内死亡。其中2例血清和脑脊液麻疹病毒特异性Ig G抗体滴度增高,1例脑活检提取到麻疹病毒RNA,确诊为爆发型SSPE;另外4例因未能获取到麻疹病毒抗体的检测结果,在排除其他病因后诊断为高度疑似爆发型SSPE。结论 SSPE可表现为爆发型病程,脑电图的广泛性周期性复合波对SSPE可提供重要的诊断线索。确诊依靠血清和脑脊液发现麻疹病毒Ig G抗体。类固醇皮质激素治疗可使病情更加恶化。
Objective To summarize and analyze the clinical and EEG characteristics of fluminant subacute scleros- ing panencephalitis （SSPE） with the purpose of increasing cognition of the disease. Methods Seven children diagnosed or suspected with SSPE were included, their sociology data, clinical and EEG manifestations, laboratory examination da- ta, response to therapy as well as clinical outcome were analyzed. Results All seven children presented as periodic atonic or spasm movement, with no other epileptic seizure type. EEGs were characteristiced by sustained long-interval periodic complexes discharges （5 ~ 20 s）. All kinds of antiepileptic drugs were ineffective, and cortieosteroids often ex- acerbated the state of the illness. The clinical condition showed progressive aggravation in all patients, who rapidly de- veloped to coma and decortex rigidity and died in 2 ~ 6.5 months. Two children had increased titers of measles virus anti- body in serum and CSF, and measles virus RNA was extracted in brain biopsy tissue in one case, which was definitely diagnosed as fluminant SSPE. Other 4 cases were highly suspected as fluminant SSPE because the measles virus anti- body testing result was not obtained but other any possible pathogenesis was excluded. Conclusion SSPE could present a fluminant course. Generalized periodic complexes discharges on EEG could provide very important diagnostic clues to SSPE. A definite diagnosis depends on finding measles virus antibody in serum and CSF. Corticosteroids should not he used for fear of deteriorating disorders.
出处
《中国实用儿科杂志》
CSCD
北大核心
2015年第7期521-526,共6页
Chinese Journal of Practical Pediatrics
关键词
亚急性硬化性全脑炎
麻疹病毒
脑电图
周期性复合波
subacute sclerosis panencephalitis
measles virus
electroencephalography （EEG）
periodic complexes discharges
