The Clinical Stages of Sporadic Creutzfeldt-Jakob Disease with Met/Met Genotype in Korean Patients
- Authors
- Park, So Young; Wang, Min Jeong; Jang, Jae-Won; Park, Young Ho; Lim, Jae-Sung; Youn, Young Chul; Kim, Jungeun; Kim, SangYun
- Issue Date
- 2016
- Publisher
- KARGER
- Keywords
- Creutzfeldt-Jakob disease; Stages; Diffusion-weighted MRI; Electroencephalography; 14-3-3 protein
- Citation
- EUROPEAN NEUROLOGY, v.75, no.5-6, pp 213 - 222
- Pages
- 10
- Journal Title
- EUROPEAN NEUROLOGY
- Volume
- 75
- Number
- 5-6
- Start Page
- 213
- End Page
- 222
- URI
- https://scholarworks.bwise.kr/cau/handle/2019.sw.cau/8699
- DOI
- 10.1159/000445768
- ISSN
- 0014-3022
1421-9913
- Abstract
- Background: Clinical diagnosis of sporadic Creutzfeldt-Jakob disease (sCJD) is currently based on changes occurring in the late disease stages, which limits early-stage detection. Therefore, we investigated the disease course from the vague symptomatic to the terminal phase. Methods: We retrospectively reviewed 36 sCJD patient records, classifying the disease progression into 4 stages based on clinical manifestations: vague symptomatic, possible CJD, probable CJD and chronic vegetative state. We analyzed findings from diffusion-weighted imaging (DWI), electroencephalography (EEG) and cerebrospinal fluid (CSF) 14-3-3 protein testing performed at each stage. Results: In stage 1, the most distinctive feature was DWI hyperintensities in the neocortex, even with negative CSF 14-3-3 protein and EEG results. In stage 2, DWI hyperintensities in the limbic cortex were more remarkable. CSF 14-3-3 protein testing yielded positive results in >80% of patients; EEG showed sensitivity in <30% of patients. With progression toward stage 3, DWI hyperintensities in the subcortical nucleus increased, with a sustained higher rate of hyperintensities in the limbic and neocortical regions. With gradual progression to stage 4, the sensitivity of CSF 14-3-3 protein testing and EEG decreased and increased, respectively within limited data. Conclusions: Understanding disease stage-dependent differences in clinical symptoms and laboratory test results will facilitate early and accurate diagnosis. (C) 2016 S. Karger AG, Basel
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