Cent Eur J Public Health 2018, 26(Supplement):S37-S41 | DOI: 10.21101/cejph.a5277

Creutzfeldt-Jakob disease surveillance in Eastern Slovakia from 2004 to 2016

Eva Feketeová1, Dominika Jarčušková1, Alžbeta Janáková2, Eva Rozprávková3, Zlatica Cifráková4, Silvia Farkašová-Inaccone5, Kvetoslava Rimárová6, Erik Dorko6, Zuzana Gdovinová1
1 Department of Neurology, Faculty of Medicine, Pavol Jozef Šafárik University in Košice and Louis Pasteur University Hospital, Košice, Slovak Republic
2 Department of Prion Diseases, Slovak Medical University in Bratislava, Bratislava, Slovak Republic
3 1st Private Hospital Košice-Šaca, Košice, Slovak Republic
4 Department of Neurology, Louis Pasteur University Hospital, Košice, Slovak Republic
5 Department of Forensic Medicine, Faculty of Medicine, Pavol Jozef Šafárik University in Košice, Košice, Slovak Republic
6 Department of Public Health and Hygiene, Faculty of Medicine, Pavol Jozef Šafárik University in Košice, Košice, Slovak Republic

Objectives: An extraordinary incidence of genetic Creutzfeldt-Jakob disease (gCJD) appearing in clusters in the Slovak Republic was described in the 1990's. The aim of the study was to analyse data of CJD cases obtained from surveillance in Eastern Slovakia (ES) (2004-2016), the region outside the described geographical clusters.

Methods: The database set in the project was the source for epidemiological and clinical analysis of CJD cases.

Results: The incidence of CJD in ES (2004-2016) was 1.7/million person-years (95% CI 1-2.4); the incidence increase in the last five years (2012-2016) was comparable to the whole country. Twenty seven of 29 reported CJD cases were available for analysis (mean age 59 years, F/M 15/12). The proportion of gCJD (E200K mutation) cases remained dominant (78%), with 9 familiar cases originating in 4 families. Analysis of the clinical features revealed shorter duration of the symptomatic phase in sporadic CJD (sCJD) (3.4 months) versus gCJD (5.15 months). Cognitive/behavioural changes, insomnia, and sensory disturbance were more pronounced in the early symptoms of gCJD. Periodic EEG discharges were more frequent in sCJD (83%) than gCJD (56%), all 19 available MR findings were CJD specific and localisation of abnormalities varied amongst the CJD forms.

Conclusions: The surveillance of CJD in ES (2004-2016) showed an increased incidence of CJD in ES, reaching the incidence rate of the whole country, with a permanent proportion of 70% gCJD cases based on the E200K mutation. Clinical, electrophysiological and MR features of sCJD and gCJD cases were in conformity with already published data. Epidemiological analysis of CJD in ES shows increasing detection of CJD but also suggests that current routine surveillance systems for CJD may underestimate the true burden of disease, especially sporadic cases in Slovakia.

Klíčová slova: Creutzfeldt-Jakob disease, epidemiology, E200K mutation, genetic CJD, sporadic CJD

Vloženo: 6. prosinec 2017; Revidováno: 30. listopad 2018; Přijato: 30. listopad 2018; Zveřejněno: 31. prosinec 2018  Zobrazit citaci

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Feketeová E, Jarčušková D, Janáková A, Rozprávková E, Cifráková Z, Farkašová-Inaccone S, et al.. Creutzfeldt-Jakob disease surveillance in Eastern Slovakia from 2004 to 2016. Cent Eur J Public Health. 2018;26(Supplement):S37-41. doi: 10.21101/cejph.a5277. PubMed PMID: 30817871.
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    Reference

    1. Brown P, Budka H, Cervenakova L, Collie DA, Green A, Ironside JW, et al. WHO manual for surveillance of human transmissible spongiform encephalopathies including variant Creutzfeldt-Jakob disease. Geneva: WHO; 2003.
    2. Masters CL, Harris JO, Gajdusek DC, Gibbs CJ Jr, Bernoulli C, Asher DM. Creutzfeldt-Jakob disease: patterns of worldwide occurrence significance of familial and sporadic clustering. Ann Neurol. 1979;5(2):117-88. Přejít k původnímu zdroji... Přejít na PubMed...
    3. Brown P, Cathala F, Gajdusek DC. Creutzfeldt-Jakob disease in France: III. Epidemiological study of 170 patients dying during the decade 1968-1977. Ann Neurol. 1979;6(5):438-46. Přejít k původnímu zdroji... Přejít na PubMed...
    4. Mitrova E, Belay G. Creutzfeldt-Jakob disease with E200K mutation in Slovakia: characterization and development. Acta Virol. 2002;46(1):31-9.
    5. Kovacs GG, Puopolo M, Ladogana A, Pocchiari M, Budka H, van Duijn C, et al. Genetic prion disease: the EUROCJD experience. Hum Genet. 2005;118(2):166-74. Přejít k původnímu zdroji... Přejít na PubMed...
    6. Mitrová E, Kosorinová D, Gajdoš M, Šebeková K, Tomečková I. A pilot study of a genetic CJD risk factor (E200K) in the general Slovak population. Eur J Epidemiol. 2014;29(8):595-7. Přejít k původnímu zdroji... Přejít na PubMed...
    7. Maďar R, Maslenová D, Ranostajová K, Straka S, Baška T. Analysis of unusual accumulation of Creutzfeldt-Jakob disease cases in Orava and Liptov regions (northern Slovak focus) 1983-2000. Cent Eur J Public Health. 2003;11(1):19-22.
    8. Slivarichová D, Mitrová E, Ursínyová M, Uhnáková I, Koscová S, Wsólová L. Geographic accumulation of Creutzfeldt-Jakob disease in Slovakia-environmental metal imbalance as a possible cofactor. Cent Eur J Public Health. 2011;19(3):158-64. Přejít k původnímu zdroji...
    9. Ladogana A, Puopolo M, Croes EA, Budka H, Jarius C, Collins S, et al. Mortality from Creutzfeldt-Jakob disease and related disorders in Europe, Australia, and Canada. Neurology. 2005;64(9):1586-91. Přejít k původnímu zdroji... Přejít na PubMed...
    10. Kovacs GG, Seguin J, Quadrio I, Höftberger R, Kapás I, Streichenberger N, et al. Genetic Creutzfeldt-Jakob disease associated with the E200K mutation: Characterization of a complex proteinopathy. Acta Neuropathologica. 2011;121(1):39-57. Přejít k původnímu zdroji... Přejít na PubMed...
    11. Bertoni JM, Brown P, Goldfarb LG, Rubenstein R, Gajdusek DC. Familial Creutzfeldt-Jakob disease (codon 200 mutation) with supranuclear palsy. JAMA. 1992;268(17):2413-5. Přejít k původnímu zdroji...
    12. Levy SR, Chiappa KH, Burke CJ, Young RR. Early evolution and incidence of electroencephalographic abnormalities in Creutzfeldt-Jakob disease. J Clin Neurophysiol. 1986;3(1):1-21. Přejít k původnímu zdroji... Přejít na PubMed...
    13. Steinhoff BJ, Zerr I, Glatting M, Schulz-Schaeffer W, Poser S, Kretzschmar HA. Diagnostic value of periodic complexes in Creutzfeldt-Jakob disease. Ann Neurol. 2004;56(5):702-8. Přejít k původnímu zdroji... Přejít na PubMed...
    14. Fulbright RK, Hoffmann C, Lee H, Pozamantir A, Chapman J, Prohovnik I. MR imaging of familial Creutzfeldt-Jakob disease: a blinded and controlled study. Am J Neuroradiol. 2008;29(9):1638-43. Přejít k původnímu zdroji... Přejít na PubMed...
    15. Appel SA, Chapman J, Prohovnik I, Hoffman C, Cohen OS, Blatt I. The EEG in E200K familial CJD: relation to MRI patterns. J Neurol. 2012;259(3):491-6. Přejít k původnímu zdroji... Přejít na PubMed...
    16. Kandiah N, Tan K, Pan AB, Au WL, Venketasubramanian N, Tchoyoson Lim CC, et al. Creutzfeldt-Jakob disease: which diffusion-weighted imaging abnormality is associated with periodic EEG complexes? J Neurol. 2008;255(9):1411-4. Přejít k původnímu zdroji... Přejít na PubMed...
    17. Shiga Y, Miyazawa K, Sato S, Fukushima R, Shibuya S, Sato Y, et al. Diffusion-weighted MRI abnormalities as an early diagnostic marker for Creutzfeldt-Jakob disease. Neurology. 2004;63(3):443-9. Přejít k původnímu zdroji... Přejít na PubMed...

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