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Abstract
ObjectiveSporadic Creutzfeldt-Jakob disease (sCJD) is a fatal and transmissible neurodegenerative disorder. However, no studies have reported Chinese specific characteristics of sCJD. We aimed to identify differences in sCJD between Chinese patients and patients from other countries.MethodsThe data from 57 Chinese sCJD patients were retrospectively analyzed, including demographic data, clinical manifestations, laboratory examinations, electroencephalograms (EEGs), diffusion-weighted imaging (DWI) scans, positron emission tomography (PET) scans, and pathological results.ResultThe disease was pathologically confirmed in 11 patients. 39 cases were diagnosed as probable sCJD, and 7 were possible. Of the total cases, 33 were male, and 24 were female. The onset age ranged from 36 to 75 years (mean: 55.5, median: 57). Disease onset before the age of 60 occurred in 57.9% of patients. The disease duration from onset to death ranged 5–22 months (mean: 11.6, median: 11), and 51.9% of patients died 7 to 12 months after disease onset. The majority of patients presented with sub-acute onset with progressive dementia. 3 of the 9 patients who took 14-3-3 protein analysis had positive results (33.3%). The sensitivity of EEG was 79.6% (43/54). For DWI and PET examinations, the sensitivities were 94% (47/50) and 94.1% (16/17), respectively. In seven patients who did not show typical hyper-intensities on the first DWI examination, abnormalities of hypo-metabolism in the cerebral cortex were clearly detected by PET. In 13 out of the 17 patients, PET detected extra abnormal regions in addition to the hyper-intense areas observed in DWI.ConclusionThis is the first study to indicate that Chinese sCJD patients have a much earlier onset age and a longer disease duration than other populations, which is most likely related to racial differences. The longer disease duration may also be a probable characteristic of Asian populations. PET had high sensitivity for the diagnosis of sCJD.
Highlights
This is the first study to indicate that Chinese Sporadic Creutzfeldt-Jakob disease (sCJD) patients have a much earlier onset age and a longer disease duration than other populations, which is most likely related to racial differences
The longer disease duration may be a probable characteristic of Asian populations
Sporadic Creutzfeldt-Jakob disease is a fatal prion disease of the nervous system that manifests as progressive dementia, cerebellar ataxia, visual disturbance, and pyramidal and extrapyramidal tract signs, and it is caused by the accumulation of infective prion in the brain
Summary
Sporadic Creutzfeldt-Jakob disease (sCJD) is a fatal prion disease of the nervous system that manifests as progressive dementia, cerebellar ataxia, visual disturbance, and pyramidal and extrapyramidal tract signs, and it is caused by the accumulation of infective prion in the brain. A definitive CJD diagnosis relies on the presence of spongiform degeneration, astrocytes gliosis and nerve cell loss on autopsy or biopsy of brain tissue. As CJD is a globally distributed disease, it has been the focus of many studies. Prior studies have reported that CJD presents differently across races. There have not been any reports regarding differences in sCJD between patients in China and patients in other countries. We retrospectively analyzed patient data and sought to identify specific characteristics of sCJD in Chinese patients. According to the theory that neuronal damage may lead to glucose hypometabolism, we attempted to determine the potential diagnostic value of PET for sCJD based on the 18F-fluorodeoxyglucose positron emission tomography/CT (18F-FDG PET/CT) scans of seventeen patients
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