<abstract xmlns="http://www.w3.org/1999/xhtml"><sec id="j_sjph-2018-0028_s_006_w2aab3b7b7b1b6b1aab1c18b1Aa"><h3>Introduction</h3><p>The objective was to present the results of surveillance of prion diseases in Slovenia that was established in 1996 and then to assess the interdisciplinary approach according to the algorithm of case management and reporting data to the National Register at the National Institute of Public Health.</p></sec><sec id="j_sjph-2018-0028_s_007_w2aab3b7b7b1b6b1aab1c18b2Aa"><h3>Methods</h3><p>A descriptive study of Creutzfeldt-Jakob disease (CJD) recorded in the period from 1996 to 2017 was carried out.</p></sec><sec id="j_sjph-2018-0028_s_008_w2aab3b7b7b1b6b1aab1c18b3Aa"><h3>Results</h3><p>A total of 123 cases of prion disease were notified between 1996 and 2017. Out of these, 68 were recorded and confirmed by autopsy as sporadic CJD with an average incidence rate of 1,5 cases per million population per year. In one case a gene analysis showed mutation E200K in prion protein gene, PRNP. Two cases of the Gerstman-Sträussler Scheinker syndrome and one clinical case of fatal insomnia with new PRNP mutation, N181S, were notified. Diagnostic value of protein 14-3-3 analysis in the liquor reached 82% sensitivity and 71% specificity. 25 cases of notified clinically possible/probable CJD were disproved after autopsy. In eleven notified possible CJD cases the autopsy had not been performed. Variant CJD has not yet been proven in Slovenia.</p></sec><sec id="j_sjph-2018-0028_s_009_w2aab3b7b7b1b6b1aab1c18b4Aa"><h3>Conclusion</h3><p>Incidence rates were comparable with other European countries. Completeness of reporting and proper management of CJD cases according to the algorithm of reporting, management and case confirmation would need some improvement. A well-functioning surveillance system, including timely notifications, would enable an appropriate epidemiological investigation and an effective response to public health risks, thus the awareness of prion diseases should not decline.</p></sec></abstract>

IntroductionThe objective was to present the results of surveillance of prion diseases in Slovenia that was established in 1996 and then to assess the interdisciplinary approach according to the algorithm of case management and reporting data to the National Register at the National Institute of Public Health.MethodsA descriptive study of Creutzfeldt-Jakob disease (CJD) recorded in the period from 1996 to 2017 was carried out.ResultsA total of 123 cases of prion disease were notified between 1996 and 2017. Out of these, 68 were recorded and confirmed by autopsy as sporadic CJD with an average incidence rate of 1,5 cases per million population per year. In one case a gene analysis showed mutation E200K in prion protein gene, PRNP. Two cases of the Gerstman-Sträussler Scheinker syndrome and one clinical case of fatal insomnia with new PRNP mutation, N181S, were notified. Diagnostic value of protein 14-3-3 analysis in the liquor reached 82% sensitivity and 71% specificity. 25 cases of notified clinically possible/probable CJD were disproved after autopsy. In eleven notified possible CJD cases the autopsy had not been performed. Variant CJD has not yet been proven in Slovenia.ConclusionIncidence rates were comparable with other European countries. Completeness of reporting and proper management of CJD cases according to the algorithm of reporting, management and case confirmation would need some improvement. A well-functioning surveillance system, including timely notifications, would enable an appropriate epidemiological investigation and an effective response to public health risks, thus the awareness of prion diseases should not decline.

Analysis of 22 years of surveillance for prion diseases in Slovenia, 1996 to 2017

Slovenian Journal of Public Health

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 3.0 License.

Analiza 22-Letnega epidemiološkega spremljanja prionskih bolezni v Sloveniji, 1996–2017

IntroductionThe objective was to present the results of surveillance of prion diseases in Slovenia that was established in 1996 and then to assess the...

Analysis of 22 years of surveillance for prion diseases in Slovenia, 1996 to 2017

Article Category: Original scientific article

Published Online: Oct 01, 2018

Page range: 227 - 233

Received: Mar 22, 2018

Accepted: Sep 05, 2018

DOI: https://doi.org/10.2478/sjph-2018-0028

Keywords
Creutzfeldt-Jakob disease, prion diseases, surveillance, epidemiology

© 2018 National Institute of Public Health, Slovenia, published by Sciendo

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 3.0 License.

Analysis of 22 years of surveillance for prion diseases in Slovenia, 1996 to 2017

Article Category: Original scientific article

Published Online: Oct 01, 2018

Page range: 227 - 233

Received: Mar 22, 2018

Accepted: Sep 05, 2018

DOI: https://doi.org/10.2478/sjph-2018-0028

KeywordsCreutzfeldt-Jakob disease, prion diseases, surveillance, epidemiology

© 2018 National Institute of Public Health, Slovenia, published by Sciendo

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 3.0 License.

Keywords
Creutzfeldt-Jakob disease, prion diseases, surveillance, epidemiology