title: Clinical and laboratory features of 14 young Chinese probable sCJD patients.
abstract: Sporadic Creutzfeldt-Jakob disease (sCJD) occurs frequently in the relatively older population, mainly in the groups of 60-69 and 70-79 year-old. Since 2006 when China performed national CJD surveillance, 14 young probable sCJD patients below 40 year-old were identified, counting for 1.93% of all probable sCJD cases. The clinical features of young probable sCJD cases, including the onset feature, the presence of sCJD-associated signs and the clinical duration, are indistinguishable from those of older patients. Special sCJD-associated abnormalities on EEG and MRI were noticed in 7 and 10 cases. CSF 14-3-3 was positive in 7 cases. CSF RT-QuIC showed positive reactive curves in 9 cases, with short lag phases. PRNP sequencing did not find any mutation. Due to low rate of brain autopsy in China, performances of other CJD-associated examinations as much as possible are extremely important for the distinguish diagnosis of young probable sCJD patients.
authors.affiliation: a State Key Laboratory for Infectious Disease Prevention and Control , Collaborative Innovation Center for Diagnosis and Treatment of Infectious Diseases (Zhejiang University), National Institute for Viral Disease Control and Prevention, Chinese Center for Disease Control and Prevention , Beijing , China.
authors.forename: Qi
authors.initials: Q
authors.lastname: Shi
authors.affiliation: a State Key Laboratory for Infectious Disease Prevention and Control , Collaborative Innovation Center for Diagnosis and Treatment of Infectious Diseases (Zhejiang University), National Institute for Viral Disease Control and Prevention, Chinese Center for Disease Control and Prevention , Beijing , China.
authors.forename: Kang
authors.initials: K
authors.lastname: Xiao
authors.affiliation: a State Key Laboratory for Infectious Disease Prevention and Control , Collaborative Innovation Center for Diagnosis and Treatment of Infectious Diseases (Zhejiang University), National Institute for Viral Disease Control and Prevention, Chinese Center for Disease Control and Prevention , Beijing , China.
authors.forename: Cao
authors.initials: C
authors.lastname: Chen
authors.affiliation: a State Key Laboratory for Infectious Disease Prevention and Control , Collaborative Innovation Center for Diagnosis and Treatment of Infectious Diseases (Zhejiang University), National Institute for Viral Disease Control and Prevention, Chinese Center for Disease Control and Prevention , Beijing , China.
authors.forename: Wei
authors.initials: W
authors.lastname: Zhou
authors.affiliation: a State Key Laboratory for Infectious Disease Prevention and Control , Collaborative Innovation Center for Diagnosis and Treatment of Infectious Diseases (Zhejiang University), National Institute for Viral Disease Control and Prevention, Chinese Center for Disease Control and Prevention , Beijing , China.
authors.forename: Chen
authors.initials: C
authors.lastname: Gao
authors.affiliation: a State Key Laboratory for Infectious Disease Prevention and Control , Collaborative Innovation Center for Diagnosis and Treatment of Infectious Diseases (Zhejiang University), National Institute for Viral Disease Control and Prevention, Chinese Center for Disease Control and Prevention , Beijing , China.
authors.forename: Jing
authors.initials: J
authors.lastname: Wang
authors.affiliation: a State Key Laboratory for Infectious Disease Prevention and Control , Collaborative Innovation Center for Diagnosis and Treatment of Infectious Diseases (Zhejiang University), National Institute for Viral Disease Control and Prevention, Chinese Center for Disease Control and Prevention , Beijing , China.
authors.forename: Bao-Yun
authors.initials: BY
authors.lastname: Zhang
authors.affiliation: a State Key Laboratory for Infectious Disease Prevention and Control , Collaborative Innovation Center for Diagnosis and Treatment of Infectious Diseases (Zhejiang University), National Institute for Viral Disease Control and Prevention, Chinese Center for Disease Control and Prevention , Beijing , China.
authors.forename: Yuan
authors.initials: Y
authors.lastname: Wang
authors.affiliation: a State Key Laboratory for Infectious Disease Prevention and Control , Collaborative Innovation Center for Diagnosis and Treatment of Infectious Diseases (Zhejiang University), National Institute for Viral Disease Control and Prevention, Chinese Center for Disease Control and Prevention , Beijing , China.
authors.forename: Xiao-Ping
authors.initials: XP
authors.lastname: Dong
mesh_terms: D048948:14-3-3 Proteins / Q000134:cerebrospinal fluid; D000328:Adult; D001921:Brain / Q000473:pathology*; D002681:China / Q000453:epidemiology; D007562:Creutzfeldt-Jakob Syndrome / Q000134:cerebrospinal fluid / Q000175:diagnosis* / Q000453:epidemiology / Q000235:genetics; D004569:Electroencephalography; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D009154:Mutation; D000072002:Prion Proteins / Q000235:genetics; D055815:Young Adult
chemical_list: D048948:14-3-3 Proteins; C512656:PRNP protein, human; D000072002:Prion Proteins
keywords: Creutzfeldt-Jakob disease; prion disease; sporadic; surveillance; young patient