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

Abstract

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. Key words: Creutzfeldt Jakob disease; Rapid progressive dementia; Magnetic resonance electroencephalogram; 14-3-3 protein