摘要目的 分析43例临床可能或很可能克雅病患者的脑电图特征及其与磁共振弥散加权像、临床表现的关系. 方法 收集2013年1月至2015年12月首都医科大学宣武医院以可疑克雅病住院的患者,对其一般资料及脑电图、MRI及临床表现进行回顾性分析.根据脑电图是否有典型周期性尖锐复合波将患者分为脑电图典型改变组、脑电图不典型改变组和脑电图暂时无改变组.比较各组年龄、病程、临床表现及MRI特点,采用Spearman法分析克雅病患者脑电图改变情况与病程、MRI及临床表现的相关性.结果 出院时诊断为可能或很可能克雅病患者43例,男26例,女17例,中位病程为4个月.发病年龄为31~80岁,平均(58.0±9.8)岁,51岁及以上者占86.0%.临床特征按比例大小排序依次是认知障碍35例(81.4%)、精神及行为异常29例(67.4%)、锥体束损害28例(65.1%)、小脑症状24例(55.8%)、锥体外系症状23例(53.5%)、肌阵挛17例(39.5%)、睡眠障碍13例(30.2%)、视力障碍13例(30.2%),无动性缄默2例(4.7%).39.5%(17/43)患者有典型周期性尖锐复合波(脑电图典型改变组),51.2%(22/43)有节律失调和不同形式慢波等非典型改变(脑电图非典型改变组),仅9.3%(4/43)暂时未见异常改变(脑电图无改变组).脑电图典型改变组MRI飘带征的出现率为82.4%(14/17),脑电图非典型改变组为77.3%(17/22),脑电图无改变组无一例出现MRI飘带征(0/4),脑电图典型改变组和非典型改变组均显著高于无改变组,且差异有统计学意义(均P<0.05).Spearman相关分析结果显示,克雅病患者脑电图改变情况与病程(r=0.351,P=0.021)及视力障碍(r=-0.377,P=0.013)相关,与年龄、肌阵挛等临床表现及MRI不相关(均P>0.05).结论 在疾病的不同阶段脑电图呈现出与病程相关的特征性改变.脑电图与MRI是评价克雅病患者不同方面状态的两个不同手段,把二者结合起来可更好地评价患者的疾病状态.

abstractsObjective To analyze the electroencephalograph (EEG) features of 43 patients with clinically possible or probable Creutzfeldt-Jakob disease (CJD) and its correlation with megnetic resonance imaging (MRI) imaging and clinical manifestations.Methods All patients diagnosed with "suspected CJD" who were hospitalized in Xuanwu Hospital from January 2013 to December 2015 were collected.The clinical data, EEG and MRI imaging features were analyzed retrospectively.Based on the periodic sharp wave complexes (PSWC) appearance in EEG results, the patients were divided into typically changed group (TCG), atypically changed group (ACG) and unchanged group (UCG).Age, disease duration, clinical manifestations and MRI features among three groups were analyzed and the correlations between patient′s EEG features and age, disease duration, clinical manifestations or MRI features were explored using spearman method.Results Among the 43 patients with possible or probable CJD disease, 26 were male and 17 were female with an average disease duration of 4 months.The age of onset ranged from 31 to 80 with an average of (58.0±9.8) years old, and 86.0% of patients were 51 years old or above.Clinical characteristics of CJD patients according to occurrence rate were as follows: 35 cases (81.4%) with cognitive impairment, 29 cases (67.4%) with mental and behavior disorder, 28 cases (65.1%) with pyramidal tract damage, 24 cases (55.8%) with cerebellar symptoms, 23 cases (53.5%) with extrapyramidal symptoms,17 cases (39.5%) with myoclonic, 13 cases (30.2%) with dyssomnia, 13 cases (30.2%) with visual disorder and 2 cases (4.7%) with akinetic mutism.Regarding EEG features, 39.53% (17/43) of patients showed typical periodic sharp wave complexes (PSWC) (TCG group), 51.2% (22/43) had irregular rhythm and different forms of slow wave (ACG group) and only 9.3% (4/43) had no EEG change (UCG group).The occurrence rate of ribbon sign in MRI was 82.4% (14/17) in TCG group, 77.3% (17/22) in ACG group and none in UCG group.The rates were significantly higher in TCG and ACG group than that in UCG group (both P<0.05).Spearman correlation analysis revealed that EEG features was correlated with disease duration (r=0.351, P=0.021) and visual impairment (r=-0.377, P=0.013) for all CJD patients.There was no correlation between EEG and MRI or other clinical manifestations such as myoclonic, age and so on (all P>0.05).Conclusions EEG showed typical changes associated with disease duration in different stages of disease.EEG and MRI are two different means to evaluate different aspects of patients with CJD disease, and combination of two means could achieve better evaluation results.