Retrospective Analysis Of236Patients With Sporadic Creutzfeldt-takob Disease In Chinese

BackgroundCreutzfeldt-Jakob disease also known as subacute spongiformencephalopathy and corticostriatal-spinal degeneration, is the mostcommon human prion disease, reported by the Creutzfeldt in1920firstly.The disease has a worldwide distribution, incidence of1/1000000. Mostpatients died within a few months, the course is generally not more than1year. Since1980, firstly reported by Zhongshan Medical University withthe autopsy confirmed two cases of CJD, has been more than200casesreported nationwide over the country. The disease is contagious andabsolutely lethal, and the Ministry of Health began to monitor the diseasein2003nationwide, by the World Health Organization requirements,making it the only non-statutory implementation of the monitoring ofinfectious diseases.ObjectiveTo study the clinical characteristics of Sporadic Creutzfeldt-Jakob disease in Chinese.MethodsPatient records in the first affiliated hospital of Chongqing MedicalUniversity and China Biological Medicine Database (CBM) and ChinaNational Knowledge Infrastructure (CNKI) were searched. Demographicdata, clinical manifestations, laboratory findings of patients with sCJDwere analyzed.ResultsOf the total236patients,93had integral data,127cases were male,and109were female. Mean age was57.5years old. The cardinal symptomsin turn were dementia181(93.3%), myoclonus150(76.1%),extrapyramidal symptoms139(70.6%) etc.195patients with headmagnetic resonance imaging(MRI), diffusion-weighted imaging (DWI) of92cases showed abnormal hyperintensities in cerebral cortex or basalganglia of93cases with DWI.190patients to check upelectroencephalogram (EEG) examination,171showed periodic sharp wavecomplexes (PSWC).41patients’14-3-3protein was positive incerebrospinal fluid (CSF), and11was negative.83patients’ brain tissuesby pathological examination are all in line with pathological changes ofsCJD. ConclusionsThe first symptoms of sCJD are various. Dementia, myoclonus andextrapyramidal performance are the main symptoms of sCJD. The latestage of sCJD and more no mobility silence appears, the average durationof7.4months, the head-sensitive magnetic resonance DWI early, the vastmajority as early as in the brain electroretinogram change. When a patientsee a doctor with rapidly progressive dementia, and his DWI and EEG havethese changes, we should take into account the probable of sCJD.