Brief ReportsClinical Presentation of Sporadic Creutzfeldt-Jakob Disease in Han-ChineseChen, Shuai MD*; He, Shuang MD*; Xu, Yin-Yan MD†; Teng, Hai-Ying MD‡; Zhang, Jie-Wen MD*Author Information *Department of Neurology, Henan Provincial People’s Hospital, People’s Hospital of Zhengzhou University †Department of Neurology, The Second Affiliated Hospital of Zhengzhou University, Zhengzhou ‡Department of Neurology, Hongxing Hospital, Hami, China S.C. and S.H. contributed equally. Supported by National Natural Science Fund (81671068). The study was approved by the Ethics Committee of People’s Hospital of Zhengzhou University. The authors declare no conflicts of interest. Reprints: Jie-Wen Zhang, MD, Department of Neurology, Henan Provincial People’s Hospital, People’s Hospital of Zhengzhou University, Zhengzhou 450001, China (e-mail: email@example.com). Alzheimer Disease & Associated Disorders: April–June 2020 - Volume 34 - Issue 2 - p 188-190 doi: 10.1097/WAD.0000000000000350 Buy Metrics Abstract The clinical presentation in Chinese patients with sporadic Creutzfeldt-Jakob disease (sCJD) may be unique due to the big difference in the codon 129 polymorphism of the prion protein gene (PRNP). This study retrospectively reviewed 26 cases of sCJD diagnosed in a single center in recent years. All 26 sCJD patients received brain magnetic resonance imaging scan, cerebrospinal fluid 14-3-3 protein detection, electroencephalogram, and PRNP gene screening. The codon 129 polymorphism were all homozygous MM in 26 sCJD patients. The main onset symptoms of sCJD patients were rapidly progressive dementia, visual impairment, and cerebellar ataxia. At the time of diagnosis, the incidence of myoclonus and akinetic mutism were relatively low (<50%). For auxiliary examinations, the positive rate of the typical magnetic resonance imaging (MRI) abnormalities, cerebrospinal fluid 14-3-3 protein, and electroencephalogram-periodic sharp wave complex was 96%, 64%, and 50%, respectively. As MM genotype is dominant and brain MRI is sensitive, brain MRI seems to play a major role in diagnosis of sCJD in Chinese. Copyright © 2019 Wolters Kluwer Health, Inc. All rights reserved.