Prion diseases with a focus on Creutzfeldt-Jakob disease, a summary of the incidence of Creutzfeldt-Jakob disease in the Czech Republic over the last 17 years, 2000–2017

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Authors: K. Kolářová ¹; M. Marešová ²; Z. Manďáková ¹; J. Kynčl ^1,3
Authors place of work: Oddělení epidemiologie infekčních nemocí, Centrum epidemiologie a mikrobiologie, Státní zdravotní ústav, Praha ¹; Protiepidemické oddělení Hygienická stanice hlavního města Prahy, pobočka Praha-jih ²; Ústav epidemiologie a biostatistiky, 3. lékařská fakulta, Univerzita Karlova Praha ³
Published in the journal: Epidemiol. Mikrobiol. Imunol. 67, 2018, č. 4, s. 155-160
Category: Původní práce

Summary

Background:

Creutzfeldt-Jakob disease (CJD) is a prion disease. It is a rare, rapidly progressing fatal disorder of the central nervous system, which occurs in four forms: sporadic (sCJD), genetic/familial (gCJD), iatrogenic (iCJD), and variant (vCJD).

Methods:

CJD research in the Czech Republic (CR) is conducted by the National Reference Laboratory for Human Transmissible Spongiform Encephalopathies and Creutzfeldt-Jakob Disease, Department of Pathology and Molecular Medicine, established in 2001 at the Department of Pathology, Thomayer Hospital, Prague. In 2003, this NRL was included in the European network of laboratories monitoring prion diseases. The purpose of the article is to analyse data reported to the EPIDAT system.

Results:

From June 2000 to June 2017, 207 deaths in persons diagnosed with CJN and four suspected deaths due to gCJD were reported to the EPIDAT system (national program of reporting, recording, and analysis of data on transmissible diseases in the CR).

Conclusion:

Reporting CJD cases to the EPIDAT is helpful in meeting the important goals, i.e. monitoring the incidence and trends of the disease. The incidence of gCJD in particular requires improved diagnosis based on a detailed personal and family history, and thorough epidemiological investigation is crucial to detect possible iatrogenic diseases.

Keywords:

Creutzfeldt-Jakob disease – prion – epidemiology – sporadic CDJ – familial CDJ – iatrogenic CDJ – variant CDJ

Zdroje

1. Rohan Z, Parobková E, Johanidesová S, Koukolík F, et al. Lidské prionové nemoci v České republice – 10 let zkušeností s diagnostikou. Cesk Slov Neurol, 2013;76/109(3):300–306.

2. Krombholz MR. Prionové demence. Psychiatr. prax, 2015;16(1):31–34.

3. Imran M, Mahmood S. An overview of human prion diseases. Virology journal, 2011;8(1):559.

4. Rusina R, Matěj R. Prionová onemocnění. Neurologie pro praxi, 2012;13(2):78–82.

5. Zerr I, Kallenberg K, Summers D, Romero C, et al. Updated clinical diagnostic criteria for sporadic Creutzfeldt-Jakob disease. Brain, 2009;132(10):2659–2668.

6. Ladogana A, Puopolo M, Croes E, Budka H, et al. Mortality from Creutzfeldt–Jakob disease and related disorders in Europe, Australia, and Canada. Neurology, 2005;64(9):1586–1591.

7. Litzroth A, Cras P, De Vil B, Quoilin S. Overview and evaluation of 15 years of Creutzfeldt-Jakob disease surveillance in Belgium, 1998–2012. BMC neurology, 2015;15(1):250.

8. Brandel J-P, Peckeu L, Haik S. The French surveillance network of Creutzfeldt–Jakob disease. Epidemiological data in France and worldwide. Transfusion clinique et biologique, 2013;20(4):395–397.

9. Brown P, Brandel J-P, Sato T, Nakamura Y, et al. Iatrogenic Creutzfeldt-Jakob disease, final assessment. Emerging infectious diseases, 2012;18(6):901.

10. Franková MV, Krausová MM. Lidské prionové nemoci. Psychiatrie pro praxi, 2008;9(3):116–119.

11. Hewitt P, Llewelyn C, Mackenzie J, Will R. Creutzfeldt–Jakob disease and blood transfusion: results of the UK Transfusion Medicine Epidemiological Review study. Vox sanguinis, 2006;91(3):221–230.

12. Zerr I, Poser S. Epidemiology and risk factors of transmissible spongiform encephalopathies in man. Prions, 2001;7:93–104.

13. Sikorska B, Knight R, Ironside JW, Liberski PP. Creutzfeldt-Jakob disease. Neurodegenerative Diseases, 2012:76–90.

14. Van Everbroeck B, Michotte A, Sciot R, Godfraind C, et al. Increased incidence of sporadic Creutzfeldt-Jakob disease in the age groups between 70 and 90 years in Belgium. European Journal of Epidemiology, 2006;21(6):443–447.

15. Johnson RT, Gibbs Jr CJ. Creutzfeldt–Jakob disease and related transmissible spongiform encephalopathies. N Engl J Med, 1998;339(27):1994–2004.

16. The National Cjd Research & Surveillance Unit (Ncjdrsu) T. U. O. E. [online]. Diagnostic criteria for human prion disease, January 2017. Dostupný na www: <https://www.cjd.ed.ac.uk/diagnosis-and-testing/diagnostic-criteria>.

17. Český statistický úřad [online]. Počet obyvatel v obcích, [cited 07/03 2017]. Dostupný na www: <https://www.czso.cz/csu/czso/pocet-obyvatel-v-obcich>.

18. Waldemar G, Dubois B, Emre M, Georges J, et al. Recommendations for the diagnosis and management of Alzheimer‘s disease and other disorders associated with dementia: EFNS guideline. European Journal of Neurology, 2007;14(1):e1–e26.

19. Rohan Z, Rusina R, Marešová M, Matěj R. Human prion diseases in the Czech Republic. Česk Epidemiol Mikrobiol Imunol, 2015;64(3):115–120.

20. Gao C, Shi Q, Tian C, Chen C, et al. The epidemiological, clinical, and laboratory features of sporadic Creutzfeldt-Jakob disease patients in China: surveillance data from 2006 to 2010. PLoS One, 2011;6(8):e24231.

21. Gubbels S, Bacci S, Laursen H, Hogenhaven H, et al. Description and analysis of 12 years of surveillance for Creutzfeldt-Jakob disease in Denmark, 1997 to 2008. Euro Surveill, 2012;17:15.