Creutzfeldt-Jakob病43例患者的临床分析

目的分析43例临床可能或很可能克雅氏病(CJD)患者的临床特征,为CJD早期诊断提供一些参考。方法搜集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,对其临床特点及实验室资料进行分析。结果 CJD通常在60岁左右发病,平均病程5.70±5.08个月;首发症状多变,以迅速进展性痴呆为主。典型临床表现有6种:迅速进展的痴呆、运动系统损害(锥体束、锥体外系及小脑症状)、肌阵挛、无动性缄默、睡眠障碍和视力障碍。结论 CJD的早期诊断应重视其临床特征,当一个患者具有典型特征中的两项或以上表现时,即使14-3-3蛋白、脑电图(EEG)、磁共振(MRI)均不典型,也要高度警惕CJD,定期复查,以免漏诊。

The clinical features of 43 patients with Creutzfeldt-Jakob disease

Objective To analyze the clinical features of 43 patients with clinically possible or probable Creutzfeldt-Jakob disease (CJD) to provide references for the early clinical evaluation and diagnosis of CJD. Method All patients who were diagnosed with “suspected CJD” and hospitalized in our hospital between January 2013 to January 2016 were collected, and their clinical features and laboratory data were analyzed retrospectively. Results In general, the onset of CJD occurred at about 60 years old, with a mean course of disease of 5.70±5.08 months(median:four months). The first symptoms are changeable, mainly presenting with rapidly progressive dementia. In addition, typical clinical manifestations included 6 types: rapidly progressivedementia, damage in the locomotor system(fibrae pyramidales, extrapyramidal and cerebellar symptoms), myoclonus, akinetic mutism, sleep and vision disorders.Conclusion The early diagnosis of CJD should pay attention to its clinical features, and highly alarming and periodic review should be given to the patient with 2 typical features or above manifestations to avoid misdiagnosis, although 14-3-3 protein, EEG and MRI show atypical findings.