Clinical data were collected for all enrolled patients. Electroencephalography (EEG), magnetic resonance imaging (MRI), and lumbar puncture were performed during hospitalization. All participants were administered the Barthel Index, a measure of functional severity commonly used to evaluate prion diseases (18 (link), 19 (link)). MRI scans were performed on a 3.0 Tesla MRI system (Siemens Magnetom Trio Tim MRI system, Germany) using standard coil. T1-weighted, T2-weighted, fluid-attenuated inversion recovery (FLAIR), diffusion-weighted image (DWI), apparent diffusion coefficient (ADC) data were acquired. EEG monitoring was performed using a 32-channel digital EEG system (DAVINCI-SAM, Micromed, Mogliano Veneto, Italy). Cerebrospinal fluid (CSF) and blood samples were collected by the medical staff in our hospital and transferred to the Chinese Center for Disease Control and Prevention, where CSF 14-3-3 protein was detected by western blot and the prion protein gene (PRNP) was analyzed (20 (link)). Typical MRI imaging for CJD diagnosis was defined as the high signal of DWI or FLAIR in caudate/putamen or at least two cortical regions (temporal, parietal, occipital). Typical EEG pattern was defined as periodic sharp wave complexes (PSWCs) (17 (link)).
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