以快速进展性痴呆起病八例散发型克雅病的临床特点

Clinical characteristics of eight patients of sporadic Creutzfeldt-Jakob disease with rapidly progressive dementia

目的分析8例临床诊断为散发性克雅病(sCJD)患者的临床特点,以提高对克雅病的认识。方法回顾性分析2012年3月至2019年5月首都医科大学附属北京地坛医院神经内科收治的7例及感染中心收治的1例sCJD患者的临床资料及辅助检查结果。结果8例患者均为亚急性起病,主要临床特点包括快速进展性痴呆、锥体外系及小脑症状、锥体束征以及相关精神症状。磁共振弥散加权像序列(DWI)显示以对称或不对称的沿皮层走行的花边样高信号为特征性磁共振改变。脑电图以周期性三相波为主。8例患者中6例行脑脊液14-3-3蛋白检查,均呈阳性。所有患者均于发病后2年内死亡。结论sCJD多为亚急性起病,常以快速进展性痴呆为突出的临床表现,磁共振DWI序列可见皮层花边征、脑电图周期性三相波等有助于临床医生早期识别和诊断本病。

Objective To investigate the clinical characteristics of 8 patients with sporadic Creutzfeldt-Jakob disease(sCJD),and to improve the understanding of the disease.Methods The clinical data and results of auxiliary examinations of 8 cases with sCJD were analyzed,retrospectively,among whom 7 cases were from the Department of Neurology and 1 case from the Infection Center of Beijing Ditan Hospital,Capital Medical University from March 2012 to May 2019.Results All the 8 patients had subacute onset.The main clinical characteristics included rapid progressive dementia,extrapyramidal and cerebellar symptoms,pyramidal tract signs,and psychiatric symptoms.Diffusion-weighted imaging(DWI)of magnetic resonance(MR)demonstrates symmetrical or asymmetrical lace-like high signal traveling along the cortex.The electroencephalogram(EEG)was dominated by periodic three-phase waves.Six cases among the 8 patients underwent cerebrospinal fluid 14-3-3 protein examination,all of them were positive.All patients died within 2 years after onset.Conclusions Most sCJD were subacute onset,characterized by rapid progressive dementia.The cortical lace sign of magnetic resonance DWI and periodic three-phase wave of electroencephalogram were helpful for clinicians to identify and diagnose the disease in early stage.