Clinical characteristics of six patients with sporadic Creutzfeldt-Jakob disease

Objective To summarize the clinical characteristics of 6 patients with sporadic Creutzfeldt-Jakob disease (CJD). Methods The clinical characteristics of 6 patients with sporadic CJD diagnosed and treated in Beijing Ditan Hosipital, Capital Medical University from January 2011 to October 2017 were analyzed, retrospectively, while the related literatures were reviewed. Results Among the 6 patients with CJD, there were 3 males and 3 females, aging 54-78 years old. The first symptoms of the patients were various, mainly with rapid progressive dementia. Five patients had cognitive impairment. One patient had no clinical symptoms, but during physical examination, high signal intensity of brain lobes was found on DWI, and walking instability occurred 7 months after physical examination. High signal intensity in lobes and/or basal ganglia could be seen on DWI images among all the 6 patients. Cerebrospinal fluid 14-3-3 protein were positive in 6 patients. Electroencephalography (EEG) of 4 patients showed periodic sharp wave complexes. All the 6 patients died of pulmonary infection and respiratory failure. Conclusions The possibility of CJD should be considered even if the clinical symptoms are atypical, and the brain magnetic resonance imaging, electroencephalogram (EEG) and cerebrospinal fluid (cerebrospinal fluid) should be reviewed regularly to avoid missed diagnosis. Key words: Creutzfeldt-Jakob disease; Clinical characteristics; Magnetic resonance imaging