Clinical and laboratory features of 14 young Chinese probable sCJD patients. Issue 2 (4th March 2017)

• Record Type: Journal Article
• Authors: Shi, Qi; Xiao, Kang; Chen, Cao; Zhou, Wei; Gao, Chen; Wang, Jing; Zhang, Bao-Yun; Wang, Yuan; Dong, Xiao-Ping
• Is Part Of: Prion. Volume 11:Issue 2(2017)
• Page Start: 128
• Page End: 135
• Publication Date: 2017-03-04
• Subjects: Creutzfeldt-Jakob disease -- prion disease -- surveillance -- sporadic -- young patient; Protein folding -- Periodicals; Prions -- Periodicals; Proteins -- Biotechnology -- Periodicals; 572.633
• DOI: 10.1080/19336896.2017.1287656
• Languages: English
• ISSNs: 1933-6896

Abstract

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.