亚急性硬化性全脑炎八例的临床和实验室特点分析  

Clinical features and laboratory characteristics of eight patients with subacute sclerosing panencephalitis

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作  者:王红梅[1] 王晓慧[1] 邓劼[1] 方方[1] 李久伟[1] 丁昌红[1] 陈春红[1] 韩彤立[1] 王旭[1] 杨欣英[1] 任长虹 李岩[1] Wang Hongmei;Wang Xiaohui;Deng Jie;Fang Fang;Li Jiuwei;Ding Changhong;Chen Chunhong;Han Tongli;Wang Xu;Yang Xinying;Ren Changhong;Li Yan(Department of Neurology,Beijing Children's Hospital,Capital Medical University,National Center for Children's Health,Beijing 100045,China)

机构地区:[1]首都医科大学附属北京儿童医院神经内科,国家儿童医学中心,北京100045

出  处:《中华神经科杂志》2020年第10期798-804,共7页Chinese Journal of Neurology

摘  要:目的探讨亚急性硬化性全脑炎(SSPE)的临床和实验室特点。方法对2014年5月至2019年2月在首都医科大学附属北京儿童医院神经内科住院的8例SSPE患儿的临床资料、实验室检查、脑电图资料进行回顾性分析及随访。结果8例患儿中男性4例,女性4例,发病年龄为2岁7个月至13岁5个月,平均发病年龄5岁6个月。8例患儿均以进行性智力、运动倒退起病,分别在病程11 d至11个月时出现周期性肌阵挛样发作;8例患儿均行视频脑电图检查,结果提示8例患儿均持续存在长间隔(3~20 s)的广泛性周期性复合波;全部患儿血及脑脊液麻疹病毒IgG滴度测定明显高于正常。8例患儿血尿筛查结果大致正常。8例患儿初次头颅核磁检查均未见明显异常,5例患儿复查头颅核磁,其中有2例大脑半球脑沟增深,2例脑白质有异常信号。积极予抗癫痫药物及对症治疗,以及丙种球蛋白、激素、抗病毒药物等治疗,所有患儿病情均进行性恶化。随访3个月至2年7个月,4例患儿死亡,其中3例患儿死亡时病程分别为5个月、1年2个月、2年6个月,另1例死亡时间不详。结论SSPE以进行性智体力倒退起病,病程Ⅱ期出现周期性肌阵挛样抽搐发作以及脑电图的广泛性周期性复合波为诊断线索。确诊需要血及脑脊液发现麻疹病毒IgG抗体。该病尚无特效治疗,预后极差。Objective To investigate the clinical and laboratory characteristics of subacute sclerosing panencephalitis (SSPE).Methods The clinical, laboratory and electroencephalogram (EEG) data of eight patients with SSPE who admitted to the Department of Neurology, Beijing Children's Hospital, Capital Medical University, from May 2014 to February 2019 were retrospectively analyzed and followed up.Results Four of the patients were male and four were female, who aged from two years and seven months to 13 years and five months with a median onset age of five years and six months. All of the eight cases had disease onset with progressive mental and physical regression, then developed periodic myoclonic seizures at the course of 11 days to 11 months. Video EEG examinations showed persistent generalized periodic complex waves with long interval (3-20 s). The IgG titers of measles virus in blood and cerebrospinal fluid of all cases were significantly increased. There was no significant abnormality in blood/urine metabolism screening nor head magnetic resonance imaging for the first time. Five cases performed head magnetic resonance imaging again, in which two cases with deepening hemispheric sulcus, two cases with cerebral white matter signal abnormalities. Antiepileptic drugs, gamma globulin, adrenocortical hormone and antiviral drugs were used after diagnosis though all were ineffective. All patients presented progressive deterioration. During the follow-up period of three months to two years and seven months, four patients died, of which three patients died at the time of five months, one year and two months, two years and six months after onset respectively, and the other one was unknown.Conclusions The diagnostic clues of SSPE are progressive mental and physical regression, recurrent myoclonic seizures during period Ⅱ, as well as the extensive periodic complex waves of EEG. It is necessary to detect measles virus IgG antibody in blood and cerebrospinal fluid to make a definite diagnosis. There is no specific treatment for

关 键 词:亚急性硬化性全脑炎 麻疹病毒 神经退行性病变 肌阵挛 脑电图 

分 类 号:R748[医药卫生—神经病学与精神病学]

 

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