[Prion diseases in The Netherlands: twenty-nine years of surveillance].

Abstract

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.

• Authors: Hata Karamujić-Čomić, Annemieke J M Rozemuller, Marcel M Verbeek, Afina W Lemstra, M Arfan Ikram, Cornelia M van Duijn
• Journal: Nederlands tijdschrift voor geneeskunde (Ned Tijdschr Geneeskd) Vol. 166 (09 21 2022) ISSN: 1876-8784 [Electronic] Netherlands
• Vernacular Title: Prionziekten in Nederland.
• PMID: 36300478 (Publication Type: English Abstract, Journal Article)

Chemical References

• Prion Proteins
• Prions

Topics

• Humans
• Prion Proteins (genetics, cerebrospinal fluid)
• Netherlands (epidemiology)
• Creutzfeldt-Jakob Syndrome (diagnosis, epidemiology, genetics)
• Prion Diseases
• Prions (cerebrospinal fluid)