新型冠状病毒病合并散发性克-雅病的临床特征(附1例报告)  

Clinical features of coronavirus disease 2019 complicated with probable sporadic Creutzfeldt-Jakob disease(report of one case)

作　　者：韩丽娟[1] 徐运[1] 张馨[1] HAN Lijuan;XU Yun;ZHANG Xin(Department of Neurology,the Affiliated Drum Tower Hospital of Nanjing University Medical School,Nanjing 210008,China)

机构地区：[1]南京大学医学院附属鼓楼医院神经内科,210008

出　　处：《临床神经病学杂志》2025年第1期52-57,共6页Journal of Clinical Neurology

基　　金：科技创新2030-“脑科学与类脑研究”重大项目(2022ZD0211800)。

摘　　要：目的探讨新型冠状病毒病(COVID-19)合并散发性克-雅病(sCJD)的临床特点。方法回顾性分析1例COVID-19合并很可能sCJD患者的临床资料,并进行文献复习。结果患者66岁男性,以视觉障碍及共济失调起病,逐渐出现快速进展性认知功能障碍、精神行为异常、锥体束征等表现,血清抗髓鞘少突胶质细胞糖蛋白免疫球蛋白G抗体阳性(1∶32),CSF Tau蛋白明显升高,14-3-3蛋白阳性,头颅MRI有2个以上皮质受累,诊断为很可能sCJD。患者在病程1个月左右感染了COVID-19,且病情逐渐加重。文献检索出11个合并COVID-19的sCJD病例。结合本例,12例患者发病中位年龄70岁,男性占66.67%。75%的sCJD患者CSF总Tau水平明显升高,66.67%患者使用了实时震荡诱导转化法检测到了阳性致病性朊蛋白。83.33%的患者早期接受了糖皮质激素或静脉注射丙种球蛋白治疗,无明显疗效。72.7%的患者在发病2~5个月内死亡,18.2%的患者在发病1~2个月内发展为浅昏迷状态。结论COVID-19有加速sCJD病情发生发展可能。CSF总Tau蛋白水平显著增高可作为COVID-19合并sCJD与自身免疫性脑炎鉴别诊断的依据。Objective To analyze the clinical features of coronavirus disease 2019(COVID-19)complicated with probable sporadic Creutzfeldt-Jakob disease(sCJD).Methods The clinical data of one patient with COVID-19 complicated with probable sCJD were retrospectively analyzed,and the relevant literatures were reviewed.Results The patient was a 66-year-old male,onset with visual impairment and ataxia,and gradually developed rapid progressive cognitive impairment,mental and behavioral abnormalities,pyramidal tract signs and other manifestations.Serum anti-myelin oligodendrocyte glycoprotein immunoglobulin G antibody was positive(1∶32),CSF Tau protein was significantly increased,14-3-3 protein was positive,and cranial MRI had more than 2 cortical involvement,which was diagnosed as likely sCJD.The patient was infected with COVID-19 about 1 month after the onset of the disease,and the condition gradually worsened.Eleven cases of sCJD complicated with COVID-19 were reviewed form the literature.Combined with this case,the median age of 12 patients was 70 years old,and 66.67%were male.The total Tau level in CSF was significantly increased in 75%of sCJD patients,and positive pathogenic prion protein was detected by real-time quaking-induced conversion method in 66.67%of patients.83.33%of patients received early glucocorticoid or intravenous gamma globulin treatment,with no significant effect.72.7%of the patients died within 2-5 months of onset,and 18.2%of the patients developed shallow coma within 1-2 months of onset.Conclusions COVID-19 has the potential to accelerate the development of sCJD.Elevated level of protein Tau is a promising target for differentiating sCJD contaminant with COVID-19 and autoimmue encephalitis.

关 键 词：散发性克-雅病 新型冠状病毒病 视觉障碍 快速进展性痴呆 共济失调 

分 类 号：R742[医药卫生—神经病学与精神病学]