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。

abstractsObjective 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.