国内散发性Creutzfeldt-Jakob病236例临床分析

发布时间：2018-06-29 23:06

本文选题：散发性Creutzfeldt-Jakob病 + 磁共振　；参考：《第三军医大学学报》2013年17期

【摘要】：目的分析国内散发性Creutzfeldt-Jakob病(sporadic creutzfeldt-Jakob disease,sCJD)的临床特点,提高临床诊断的准确性。方法借鉴循证医学系统评价方法,对1990-2012年国内文献报道的临床病例资料及我院神经内科2006-2012年临床诊断为sCJD的住院病历资料进行综合分析。结果剔除可能重复的病例后,共收集到236例,其中完整病例资料93例,7例来自我院病历记录,86例来自文献报道。236例资料中,男性127例,女性109例,平均发病年龄57.5岁。主要症状依次有进行性痴呆93.3%,肌阵挛76.1%,锥体外系表现70.6%等。195例行头部MRI检查,DWI检查93例,其中92例表现为皮质或基底节区高信号。190例行脑电图检查,171例出现典型周期波。52例检测脑脊液14-3-3蛋白,41例(+),11例(-)。83例脑组织病理检查,均符合sCJD病理改变。结论 sCJD首发症状多样,主要临床症状以进行性痴呆、肌阵挛及锥体外系表现为主,病程晚期多出现无动性缄默,平均病程7.4个月。头部磁共振DWI早期敏感,绝大多数早于脑电图改变。根据临床症状结合DWI、EEG及14-3-3蛋白等检查,sCJD的临床诊断并不困难。
[Abstract]:Objective to analyze the clinical features of sporadic Creutzfeldt-Jakob disease (sporadic creutzfeldt-Jakob disease) in China and to improve the accuracy of clinical diagnosis. Methods the data of clinical cases reported in domestic literature from 1990 to 2012 and the medical records of inpatients diagnosed as sCJD from 2006 to 2012 in neurology department of our hospital were comprehensively analyzed by using the method of Evidence-Based Medicine (EBM) system evaluation. Results A total of 236 cases were collected, including 93 cases of complete cases, 7 cases from medical records, 86 cases from literature reports, 127 cases of males and 109 cases of females, with an average onset age of 57.5 years. The main symptoms in turn included progressive dementia (93. 3%), myoclonus (76. 1), extrapyramidal system (70. 6%) and DWI (93 cases). Among them, 92 cases showed hyperintense signal in cortex or basal ganglia. 171 cases showed typical periodic wave in 171 cases. 41 cases (-) 83 cases of cerebrospinal fluid 14-3-3 protein were detected by electroencephalogram. All of them were in accordance with sCJD pathological changes. Conclusion the main clinical symptoms of sCJD are progressive dementia, myoclonus and extrapyramidal system. The head was sensitive on DWI, most of which were earlier than electroencephalogram (EEG). It is not difficult to diagnose sCJD according to clinical symptoms combined with EEG and 14-3-3 protein.
【作者单位】：重庆医科大学附属第一医院神经内科;
【分类号】：R742.9

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