贵州省2010-2015年克-雅病病例监测结果分析

发布时间：2018-04-15 20:11

  本文选题：克-雅病 + 监测　； 参考：《中国人兽共患病学报》2017年05期

【摘要】：目的分析2010-2015年贵州省克-雅病(Creutzfeldt-Jakob disease,CJD)监测病例流行病学、临床特征、以及病例转归情况。方法对贵州省克-雅病监测网络发现的疑似病例的流行病学、临床特征以及随访资料进行分析,同时结合病例脑脊液、血液标本的实验室检测结果。结果 2010-2015年贵州省报告的23例CJD疑似病例中发现CJD病例11例,其中sCJD临床诊断病例8例,sCJD临床疑似病例2例,gCJD确诊诊断病例1例。11例病例中,首发症状以快速进行性痴呆为主要表现,其次是精神症状、锥体外系症状、小脑症状和皮质性失明;临床症状中进行性痴呆为主要症状,其次是视觉或小脑障碍、肌阵挛、锥体系/锥体外系功能异常、无动性缄默;辅助检查以头颅核磁共振(MRI)异常为主(45.45%);实验室检测脑脊液14-3-3蛋白阳性率较高(70%),血液标本中朊蛋白基因(PRNP)检测129位氨基酸多态性均为M/M型,除1例gCJD确诊诊断病例PRNP基因检测发现D178N突变外,均未发现其它位点突变。11例CJD病例无季节、地区聚集性和职业倾向,以男性为主,年龄中位数在65岁,主要为汉族。流行病学史无特殊。对所有CJD病例在报告当年进行随访,失访率27%,大多数病例均在1年内死亡。结论 2010-2015年贵州省CJD病例中以sCJD为主,其流行病学特征与同期全国监测情况相符。首次在贵州省发现1例gCJD病例,其PRNP基因突变与2011-2014年全国CJD监测网络发现的gCJD突变位点均不相同,与河南2011-2013年间报告的2例遗传型朊蛋白病病例在临床表现及PRNP基因突变位点相似。
[Abstract]:Objective to analyze the surveillance of case epidemiology, clinical characteristics and outcome of Creutzfeldt-Jakob disease (CJDD) in Guizhou Province from 2010 to 2015.Methods Epidemiology, clinical features and follow-up data of suspected cases found by KYD surveillance network in Guizhou province were analyzed. The laboratory results of cerebrospinal fluid and blood samples were also analyzed.Results from 2010 to 2015, 11 cases of CJD were found in 23 suspected cases of CJD in Guizhou Province, including 8 cases of clinical diagnosis of sCJD and 2 cases of suspected cases of sCJD.The first symptoms were rapid progressive dementia, followed by mental symptoms, extrapyramidal symptoms, cerebellar symptoms and cortical blindness; progressive dementia was the main symptom in clinical symptoms, followed by visual or cerebellar disorders, myoclonus,The function of the pyramidal / extrapyramidal system was abnormal, and there was no dynamic silence.The main auxiliary examinations were cranial MRI MRI abnormality 45.455.The positive rate of 14-3-3 protein in cerebrospinal fluid (CSF) was higher than 70% in laboratory, and the amino acid polymorphism at position 129 in blood samples was all M / M type, and the positive rate of 14-3-3 protein in cerebrospinal fluid (CSF) was higher than that in control group (P < 0.05).Except for the D178N mutation found in the PRNP gene of one diagnosed case with gCJD, no other locus mutation was found. 11 cases of CJD were not seasonal. The regional clustering and occupational tendency were mainly male, the median age was 65 years old, mainly Han nationality.There is no special history of epidemiology.All cases of CJD were followed up in the reporting year, the missing rate was 27%, and most of the cases died within 1 year.Conclusion sCJD is the main CJD case in Guizhou province from 2010 to 2015, and its epidemiological characteristics are consistent with the national surveillance in the same period.A case of gCJD was first found in Guizhou Province. The mutation of PRNP gene was different from that of gCJD mutation found by the National CJD Surveillance Network in 2011-2014.The clinical manifestations and PRNP gene mutation sites of two cases of hereditary prion disease reported from 2011 to 2013 in Henan Province were similar.
【作者单位】： 贵州省疾病预防控制中心传防所;贵州省医科大学附属医院神经内科;贵州省第二人民医院神经内科;贵州省人民医院神经内科;
【分类号】：R181.8;R511
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本文编号：1755597