Creutzfeldt-Jakob病脑脊液14-3-3蛋白变化特征及其影响因素分析

目的分析47例临床可能或很可能克雅氏病(CJD)患者的脑脊液(CSF)14-3-3蛋白特征,为CJD早期临床评估及诊断提供参考。方法收集2013年1月至2016年1月以"可疑CJD"诊断在首都医科大学宣武医院住院的患者,对其一般资料及CSF 14-3-3蛋白特征进行分析。比较CSF 14-3-3蛋白阳性组与阴性组年龄、病程、临床表现及脑电图(EEG)、磁共振(MRI)的特点,并采用Spearman法分析CJD患者CSF 14-3-3蛋白与年龄、病程、MRI、EEG及临床表现的相关性。结果 47例患者中有46例患者进行了腰椎穿刺并送检CSF 14-3-3蛋白,其中有5例既往CSF 14-3-3蛋白检查为阴性者在复查后结果转为阳性,由阴性转为阳性时间为1~3个月。最终CSF 14-3-3蛋白阳性的患者占58.70%(27/46),14-3-3蛋白阳性组锥体束(P=0.015)、小脑症状(P=0.012)和视力障碍(P=0.044)均高于阴性组,仅磁共振(MRI)阳性率阳性组低于阴性组(P=0.025),且Spearman法相关性分析显示CJD患者CSF 14-3-3蛋白与锥体束(P=0.029)、视力障碍(P=0.039)及MRI(P=0.006)相关。结论 1 CSF 14-3-3蛋白含量可能与锥体束、小脑症状等运动系统及视力损害有关系,可间接反应CJD患者大脑神经细胞的功能状态;2 CSF 14-3-3蛋白为CJD早期诊断提供参考依据之一,CSF14-3-3蛋白含量随病情进展发生变化,对于首次检查阴性的患者实际工作中要注意复查,以免漏诊。

The role of CSF 14-3-3 protein and associated factors in Creutzfeldt-Jakob disease

Objective To analyze features of 14-3-3 protein in cerebrospinal fluid (CSF) of 47 patients with probable or possible Creutzfeldt-Jakob disease (CJD), provide reference for clinical evaluation and diagnosis of CJD disease in early stage.Method All patients who were diagnosed with “suspected CJD” and hospitalized in Xuanwu Hospital from January 2013 to January 2016 were collected, and their general information and features of 14-3-3 protein in CSF were analyzed retrospectively. We compared patient's age, clinical course, clinical manifestation, electroencephalogram (EEG) and magnetic resonance imaging (MRI) features between 14-3-3 protein positive group and negative group, analyzed correlation between 14-3-3 protein in CSF of CJD patients and their age, clinical course, clinical manifestation, EEG and MRI features using Spearman method.Results I46 in these patients performed lumbar puncture and examined 14-3-3 protein in CSF, 14-3-3 protein result in 5 patients showed negative in past but positive in reexamination, duration of results conversion was 1-3 months. Patients in positive group accounted for 58.70% (27/46). Occurrence rate of pyramidal tract symptom (P=0.015), cerebellar symptom (P=0.012)and visual impairment(P=0.044) in positive group were higher than negative group, only positive rate of MRI was lower (P=0.025),analysis using Spearman method showed that 14-3-3 protein in CSF of CJD patients was related to pyramidal tract symptom (P=0.029), visual impairment (P=0.039), MRI features (P=0.006). Conclusion ①14-3-3 protein in CSF may be associated with pyramidal tract symptom, cerebellar symptom and visual impairment in clinical manifestations, indirect reflects functional state of cerebral neuronal cells of CJD patients.②14-3-3 protein provide a type of evidence for CJD early diagnosis and changes along with development of disease, periodic review should be emphasized in patients with negative result previously.