Creutzfeldt-Jakob病肌阵挛及相关影响因素分析  

Myoclonus and it＇s associated factors in Creutzfeldt-Jakob disease

作　　者：刘静[1] 王红星 李莉萍[1] 洪霞[4] 刘爱华[1] 叶静[1] 宋雷[1] 张进[1] 李京[1] 户宁宁 孙志超[1] 李思然[1] 杨延辉 董会卿[1] 王玉平 

机构地区：[1]首都医科大学宣武医院神经内科,北京100053 [2]北京脑重大疾病研究院 [3]北京脑重大疾病研究院影像科 [4]中国中医科学院西苑医院脑病科

出　　处：《中华医学杂志》2016年第45期3656-3661,共6页National Medical Journal of China

基　　金：国家自然科学基金（81301138）;国家863计划项目（2015AA020514）;中国博士后科学基金（200902119）;新世纪百千万人才工程北京市级人选（2010-005）;北京市卫生系统高层次卫生技术人才培养项目（2009-3-53）

摘　　要：目的 分析47例临床可能或很可能克－雅病（CJD）患者的肌阵挛特征及相关影响因素。方法 收集2013年1月至2015年12月以＂可疑CJD＂诊断在首都医科大学宣武医院神经内科住院的患者，对其肌阵挛及其他临床表现、脑电图（EEG）、磁共振成像（MRI）、脑脊液（CSF）14－3－3蛋白等资料进行回顾性分析。比较肌阵挛阳性组与阴性组年龄、病程、临床表现及EEG、MRI、脑脊液14－3－3蛋白特点，并采用Spearman法分析CJD患者肌阵挛出现情况与年龄、病程、临床表现及EEG的相关性。结果 （1）肌阵挛阳性组CJD患者锥体外系症状、视力障碍及睡眠障碍出现率均较阴性组高，差异有统计学意义（P值分别为0.028、0.025、0.004）；Spearman相关性分析结果显示CJD患者肌阵挛的出现与锥体外系症状（P＝0.024）、视力障碍（P＝0.030）、睡眠障碍（P＝0.001）相关。（2）肌阵挛阳性及阴性组EEG改变差异无统计学意义（P〉0.05）；进一步将17例肌阵挛阳性的患者分为EEG典型改变组9例（9/17），EEG不典型改变组4例（4/17）和EEG暂时无改变组4例（4/17），三组间的肌阵挛及其他临床表现差异均无统计学意义（均P〉0.05）；相关分析也未发现肌阵挛与EEG间差异有统计学意义（P＝0.201）。结论 CJD患者的肌阵挛多出现于运动系统障碍（包括锥体束、锥体外系症状和小脑症状）之后，其出现与锥体外系症状、视力障碍、睡眠障碍相关，与脑电图周期性尖慢复合波之间未见明确相关性。Objective To analyze features and the related factors of myoclonus of 47 patients with probable or possible Creutzfeldt-Jakob disease （CJD）. Methods All patients diagnosed with＂suspected CJD＂ and hospitalized in Xuanwu Hospital from January 2013 to November 2015 were included, and their clinical information and myoclonus features were analyzed. Age, clinical, course and manifestation, EEG, MRI, CSF14-3-3 features between myoclonus positive group and negative group were compared, and the correlation between myoclonus features and these factors was analyzed using Spearman correlation analyses.Results （1） Occurrence rate of extrapyramidal symptoms （P=0.028）, visual impairment （P=0.025） and dyssomnia （P=0.004） were higher in myoclonus positive group, the differences were significant. Spearman correlation analysis showed that myoclonus was related to extrapyramidal symptoms （P=0.024）, visual impairment （P=0.030） and dyssomnia （P=0.001）. （2） EEG features showed no significant difference between myoclonus positive and negative group. The 17 myoclonus positive patients were divided into three subgroups, typical EEG change group 52.94%（9/17）, atypical EEG change group 23.53%（4/17） and no EEG change group 23.53%（4/17）. Difference of myoclonus and other clinical manifestations were not significant among the three subgroups（P〉0.05）; correlation analysis also found no statistically significant correlation between myoclonus and EEG （P=0.201）.Conclusions Myoclonus often occurs after the damage of locomotor system （including pyramidal tract, extracorticospinal tract and cerebellum） among CJD patients, and it is related to extrapyramidal symptoms , visual impairment and dyssomnia . There is undefined correlation between myoclonus and periodic sharp wave complexes （PSWC） in EEG.

关 键 词：克-雅病 肌阵挛 脑电图 周期性尖慢复合波 

分 类 号：R742[医药卫生—神经病学与精神病学]