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Clinical features of sporadic Creutzfeldt-Jakob disease in China and its auxiliary examinations: A report of 55 cases

Author(s): Bo YAN | Jia-tang ZHANG | Wei ZHAO | Lin MA | Da-yi YIN | Wei-quan JIA | Xu-sheng HUANG | Sen-yang LANG | Chuan-qiang PU | Sheng-yuan YU

Journal: Medical Journal of Chinese People's Liberation Army
ISSN 0577-7402

Volume: 38;
Issue: 10;
Start page: 855;
Date: 2013;
Original page

Keywords: Creutzfeldt-Jakob disease | electroencephalography | diffusion magnetic resonance imaging | positron-emission tomography

Objectives To analyze the clinical features of sporadic Creutzfeldt-Jakob disease (sCJD) in China, and the sensitivity and specificity of auxiliary examinations to improve the early diagnostic rate of sCJD. Methods Clinical manifestations, 14-3-3 protein test, electroencephalography (EEG), neuroimaging and neuropathology of 55 patients with sCJD were retrospectively analyzed. Results Among 55 patients with sCJD, 10 were neuropathologically confirmed, 38 clinically probable and 7 clinically possible. The average age was 55.8 years old (ranging from 36-75 years) and the interval between onset of symptoms and death ranged 5 to 22 months (average 11.4 months). The majority of patients showed subacute onset and the most frequent initial symptom was progressive dementia. Nine patients received 14-3-3 protein detection and 3 out of them had positive results (33.3%). Fifty-three patients underwent EEG examinations, 40 out of them showed typical periodic sharp wave complexes (PSWC, 75.5%). Abnormal hyperintense lesions on diffusion-weighted images (DWI) were observed in 47/50 (94%) patients. Of 16 patients who underwent 18F-fluorodeoxyglucose positron emission tomography/CT (18F-FDG PET/CT) scan, 15 (93.8%) showed hypometabolism, especially 13 patients showed abnormal high-intensity zone on PET/CT in addition to the hyper-intense areas observed in DWI. Conclusions Chinese sCJD patients have a much earlier onset age and longer disease duration than those reported in the foreign literatures. The majority of patients have subacute onset, and the most frequent initial symptom is progressive dementia. For patients suspected of sCJD, typical EEG and brain DWI changes could provide important reference for clinical diagnosis of sCJD. PET/CT is of great value in early diagnosis and differential diagnosis of sCJD. DOI: 10.11855/j.issn.0577-7402.2013.10.016
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