Prionziekten in Nederland: Een overzicht van 29 jaar surveillance

Translated title of the contribution: Prion diseases in The Netherlands: twenty-nine years of surveillance

Hata Karamujić-Čomić, Annemieke J.M. Rozemuller, Marcel M. Verbeek, Afina W. Lemstra, M. Arfan Ikram, Cornelia M. van Duijn

Research output: Contribution to journalArticleAcademicpeer-review


Prion diseases are being monitored in The Netherlands since 29 years, the national registry is coordinated by Erasmus Medical Center. Since 2010, yearly on average 31 new patients are diagnosed with prion disease. There is a slight increase in incidence of prion diseases, probably due to better recognition and improved diagnostics. The most recent development in the diagnostic is the real-time quaking-induced conversion (RT-QuIC) test, which can detect prion proteins in cerebrospinal fluid with high sensitivity and specificity. The polymorphism of codon 129 of the prion gene (PRNP) determines the susceptibility for the different subtypes of Creutzfeldt-Jakob disease (CJD) and influences the clinical course. Awareness for atypical presentations of CJD and for CJD mimics is important, such as autoimmune encephalitis, which is often treatable and can resemble CJD.

Translated title of the contributionPrion diseases in The Netherlands: twenty-nine years of surveillance
Original languageDutch
JournalNederlands Tijdschrift voor Geneeskunde
Publication statusPublished - 21 Sep 2022


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