Characteristics of patients with Creutzfeldt-Jakob disease in China, 2019

Xiao Kang; Zhou Wei; Wang Yuan; Dong Xiaoping; Shi Qi

doi:10.3784/jbjc.202104250217

Volume 37 Issue 2

Mar. 2022

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Article Navigation > Disease Surveillance > 2022 > 37(2): 185-190

Xiao Kang, Zhou Wei, Wang Yuan, et al. Characteristics of patients with Creutzfeldt-Jakob disease in China, 2019[J]. Dis Surveill, 2022, 37(2): 185-190. doi: 10.3784/jbjc.202104250217

Citation:

Xiao Kang, Zhou Wei, Wang Yuan, et al. Characteristics of patients with Creutzfeldt-Jakob disease in China, 2019[J]. Dis Surveill, 2022, 37(2): 185-190. doi: 10.3784/jbjc.202104250217

Xiao Kang, Zhou Wei, Wang Yuan, et al. Characteristics of patients with Creutzfeldt-Jakob disease in China, 2019[J]. Dis Surveill, 2022, 37(2): 185-190. doi: 10.3784/jbjc.202104250217

Citation:

Xiao Kang, Zhou Wei, Wang Yuan, et al. Characteristics of patients with Creutzfeldt-Jakob disease in China, 2019[J]. Dis Surveill, 2022, 37(2): 185-190. doi: 10.3784/jbjc.202104250217

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Characteristics of patients with Creutzfeldt-Jakob disease in China, 2019

doi: 10.3784/jbjc.202104250217

Institute for Viral Disease Prevention and Control, State Key Laboratory for Communicable Disease Prevention and Control, Chinese Center for Disease Control and Prevention, Beijing 102206, China

Funds: This study was supported by the Grant from the State Key Laboratory for Infectious Disease Prevention and Control (No.2021SKLID506, No.2021SKLID101, No.2019SKLID307) and National Natural Science Foundation of China (No.81630062)

More Information

Corresponding author: Dong Xiaoping, Email: dongxp238@sina.com; Shi Qi, Email: shiqi76@126.com
Received Date: 2021-04-25
Accepted Date: 2022-01-19

Available Online: 2021-10-25

Publish Date: 2022-03-23

Abstract

Abstract

Objective To describe epidemiological and clinical characteristics of the patients with Creutzfeldt-Jakob disease (CJD) in 12 provinces in China. Methods The clinical and epidemiological information of CJD patients obtained from China CJD surveillance network were analyzed. Blood and cerebral spinal fluid (CSF) specimens were collected from these patients. Western blot assay was conducted for detecting 14-3-3 protein in CSF, and Polymerase Chain Reaction (PCR) and sequencing were performed by using DNA extracted from whole blood genome for the analyses on polymorphism of 129 and 219 amino acid and mutation of PRNP gene. Results In 2019, a total of 520 CJD cases were detected in the surveillance, including 190 (36.54%) probable CJD cases, 8 (1.54%) possible sporadic CJD cases, 25 (4.81%) genetic CJD cases, 5 (0.96%) fatal familial insomnia (FFI) cases and 1 (0.19%) cases of Gerstmann-Straussler-Scheinker (GSS) syndrome. The cases occurred sporadically without clustering in time, place and population. The median age of diagnosed and probable CJD cases was 62 years (40–84 years old), and the male to female ratio of the cases was 0.96∶1. The median age of possible CJD cases was 64.5 years (45–76 years old), and the male to female ratio of the cases was 1∶1. Rapidly progressive dementia was the major initial symptom. Among 508 cases, 491 were M/M in allele 129, and 497 were E/E in allele 219. Conclusion CJD occurred sporadically in China in 2019. The time, place and population distributions, gender ratio and average onset age of the CJD cases were consistent with the general characteristics of sporadic CJD cases in the world.
- Creutzfeldt-Jakob disease,
- Surveillance,
- 14-3-3 protein,
- Prionproteingene,
- Magneticresonanceimaging

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References(28)

References

[1]	Prusiner SB. Prions[J]. Proc Natl Acad Sci USA, 1998, 95(23): 13363–13383. DOI: 10.1073/pnas.95.23.13363.
[2]	Collinge J. Prion diseases of humans and animals: their causes and molecular basis[J]. Annu Rev Neurosci, 2001, 24: 519–550. DOI: 10.1146/annurev.neuro.24.1.519.
[3]	Liberski PP. Historical overview of prion diseases: a view from afar[J]. Folia Neuropathol, 2012, 50(1): 1–12.
[4]	Kulczycki J. Creutzfeldt-Jakob disease－the past or the future[J]. Przegl Epidemiol, 2006, 60 Suppl 1: 63–67.
[5]	Will RG, Ironside JW, Zeidler M, et al. A new variant of Creutzfeldt-Jakob disease in the UK[J]. Lancet, 1996, 347(9006): 921–925. DOI: 10.1016/s0140−6736(96)91412−9.
[6]	Collinge J. Variant Creutzfeldt-Jakob disease[J]. Lancet, 1999, 354(9175): 317–323. DOI: 10.1016/S0140−6736(99)05128−4.
[7]	Puoti G, Bizzi A, Forloni G, et al. Sporadic human prion diseases: molecular insights and diagnosis[J]. Lancet Neurol, 2012, 11(7): 618–628. DOI: 10.1016/S1474−4422(12)70063−7.
[8]	Brandel JP, Peckeu L, Haïk S. The French surveillance network of Creutzfeldt-Jakob disease. Epidemiological data in France and worldwide[J]. Transfus Clin Biol, 2013, 20(4): 395–397. DOI: 10.1016/j.tracli.2013.02.029.
[9]	Noguchi-Shinohara M, Hamaguchi T, Yamada M. Epidemiology and surveillance system of prion disease in Japan[J]. Nihon Rinsho, 2007, 65(8): 1379–1383.
[10]	Klug GM, Boyd A, Lewis V, et al. Creutzfeldt-Jakob disease: Australian surveillance update to 31 December 2004[J]. Commun Dis Intell Q Rep, 2005, 29(3): 269–271.
[11]	Krasnianski A, Heinemann U, Ponto C, et al. Clinical findings and diagnosis in genetic prion diseases in Germany[J]. Eur J Epidemiol, 2016, 31(2): 187–196. DOI: 10.1007/s10654−015−0049−y.
[12]	王晶, 周伟, 肖康, 等. 2017年中国克-雅病监测网络病例特征分析[J]. 疾病监测,2019,34(3):226–231. DOI:10.3784/j.issn.1003−9961.2019.03.010. Wang J, Zhou W, Xiao K, et al. Characteristics of patients with Creutzfeldt-Jakob disease in China, 2017[J]. Dis Surveill, 2019, 34(3): 226–231. DOI: 10.3784/j.issn.1003−9961.2019.03.010.
[13]	肖康, 周伟, 王园, 等. 2018年中国克–雅病监测网络病例特征分析[J]. 疾病监测,2021,36(2):131–136. DOI: 10.3784/jbjc.202006290222. Xiao K, Zhou W, Wang Y, et al. Characteristics of patients of Creutzfeldt-Jakob disease detected in surveillance in China, 2018[J]. Dis Surveill, 2021, 36(2): 131–136. DOI: 10.3784/jbjc.202006290222.
[14]	Bratosiewicz-Wąsik J, Smoleń-Dzirba J, Watała C, et al. Association of the PRNP regulatory region polymorphisms with the occurrence of sporadic Creutzfeldt-Jakob disease[J]. Folia Neuropathol, 2012, 50(1): 68–73.
[15]	中华人民共和国国家卫生和计划生育委员会. WS/T 562－2017 克–雅病诊断［S］. 北京: 中国标准出版社, 2018 National Health and Family Planning Commission of the People′s Republic of China. WS/T 562－2017　Diagnosis of Creutzfeldt-jakob disease［S］. Beijing: China Standards Press, 2018.
[16]	侯星生, 高晨, 张宝云, 等. 中国不同民族人群中PrP蛋白基因第129位氨基酸多态性分析[J]. 中华实验和临床病毒学杂志,2002,16(2):105–108. DOI:10.3760/cma.j.issn.1003−9279.2002.02.001. Hou XS, Gao C, Zhang BY, et al. Characteristics of polymorphism of 129^th amino acid in PRNP among Han and Uighur Chinese[J]. Chin J Exp Clin Virol, 2002, 16(2): 105–108. DOI: 10.3760/cma.j.issn.1003−9279.2002.02.001.
[17]	Chen C, Wang JC, Shi Q, et al. Analyses of the survival time and the influencing factors of Chinese patients with prion diseases based on the surveillance data from 2008−2011[J]. PLoS One, 2013, 8(5): e62553. DOI: 10.1371/journal.pone.0062553.
[18]	Shibuya S, Higuchi J, Shin RW, et al. Protective prion protein polymorphisms against sporadic Creutzfeldt-Jakob disease[J]. Lancet, 1998, 351(9100): 419. DOI: 10.1016/S0140−6736(05)78358−6.
[19]	Kobayashi A, Teruya K, Matsuura Y, et al. The influence of PRNP polymorphisms on human prion disease susceptibility: an update[J]. Acta Neuropathol, 2015, 130(2): 159–170. DOI: 10.1007/s00401−015−1447−7.
[20]	Nozaki I, Hamaguchi T, Sanjo N, et al. Prospective 10-year surveillance of human prion diseases in Japan[J]. Brain, 2010, 133(10): 3043–3057. DOI: 10.1093/brain/awq216.
[21]	Poser S, Mollenhauer B, Krauβ A, et al. How to improve the clinical diagnosis of Creutzfeldt-Jakob disease［J］. Brain, 1999, 122(Pt 12): 2345–2351. DOI: 10.1093/brain/122.12.2345.
[22]	Hamlin C, Puoti G, Berri S, et al. A comparison of tau and 14-3-3 protein in the diagnosis of Creutzfeldt-Jakob disease[J]. Neurology, 2012, 79(6): 547–552. DOI: 10.1212/WNL.0b013e318263565f.
[23]	Peckeu L, Delasnerie-Lauprètre N, Brandel JP, et al. Accuracy of diagnosis criteria in patients with suspected diagnosis of sporadic Creutzfeldt-Jakob disease and detection of 14-3-3 protein, France, 1992 to 2009[J]. Euro Surveill, 2017, 22(41): 16–00715. DOI: 10.2807/1560−7917.ES.2017.22.41.16−00715.
[24]	Riva-Amarante E, Jiménez-Huete A, Toledano R, et al. Usefulness of high b-value diffusion-weighted MRI in the diagnosis of Creutzfeldt-Jakob disease[J]. Neurología, 2011, 26(6): 331–336. DOI: 10.1016/j.nrl.2010.12.003.
[25]	Cambier DM, Kantarci K, Worrell GA, et al. Lateralized and focal clinical, EEG, and FLAIR MRI abnormalities in Creutzfeldt-Jakob disease[J]. Clin Neurophysiol, 2003, 114(9): 1724–1728. DOI: 10.1016/s1388−2457(03)00109−3.
[26]	Vitali P, Maccagnano E, Caverzasi E, et al. Diffusion-weighted MRI hyperintensity patterns differentiate CJD from other rapid dementias[J]. Neurology, 2011, 76(20): 1711–1719. DOI: 10.1212/WNL.0b013e31821a4439.
[27]	Wilham JM, Orrú CD, Bessen RA, et al. Rapid end-point quantitation of prion seeding activity with sensitivity comparable to bioassays[J]. PLoS Pathog, 2010, 6(12): e1001217. DOI: 10.1371/journal.ppat.1001217.
[28]	Orrú CD, Groveman BR, Hughson AG, et al. Rapid and sensitive RT-QuIC detection of human Creutzfeldt-Jakob disease using cerebrospinal fluid[J]. mBio, 2015, 6(1): e02451–14. DOI: 10.1128/mBio.02451−14.