ArticlePloS one2012
Human prion diseases in the Netherlands (1998-2009): clinical, genetic and molecular aspects.
Article in PloS one, 2012. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 36 papers, 2 of them syntheses that pooled it.
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Who cites it
36 citing papers in PubMed, 2 syntheses or guidelines pooled it, 50 citations in OpenAlex.
- The First Meta-Analysis of the M129V Single-Nucleotide Polymorphism (SNP) of the Prion Protein Gene (Cells · 2021Pooled it
- Interventions to reduce the risk of surgically transmitted Creutzfeldt-Jakob disease: a cost-effective modelling review.Health technology assessment (Winchester, England) · 2020Pooled it
- A two-step processing method is recommended to inactivate human sporadic M1 and V2 prions.The Journal of general virology · 2026Article
- Pedigree analysis and genetic inheritance of fatal familial insomnia (FFI) in a Portuguese multigenerational family.Journal of neurology · 2025Article
- Defining the phenotypic spectrum of sporadic Creutzfeldt-Jakob disease MV2K: the kuru plaque type.Brain : a journal of neurology · 2023Article
- Article
- Creutzfeldt-Jakob Disease: A Case Report and Literature Review for Understanding the Big Picture.Cureus · 2022Article
- Prion strains associated with iatrogenic CJD in French and UK human growth hormone recipients.Acta neuropathologica communications · 2021Article
- Further Characterization of Glycoform-Selective Prions of Variably Protease-Sensitive Prionopathy.Pathogens (Basel, Switzerland) · 2021Article
- Genetic Risk Factors of Creutzfeldt-Jakob Disease in the Population of Newborns in Slovakia.Pathogens (Basel, Switzerland) · 2021Article
- First participant diagnosed with Creutzfeldt-Jakob disease in the population-based Rotterdam Study was classified with mild cognitive impairment.BMJ case reports · 2021Article
- Human Prion Disease Surveillance in Washington State, 2006-2017.JAMA network open · 2020Article
- CSF biomarkers of neuroinflammation in distinct forms and subtypes of neurodegenerative dementia.Alzheimer's research & therapy · 2019Article
- Diagnostic value of surrogate CSF biomarkers for Creutzfeldt-Jakob disease in the era of RT-QuIC.Journal of neurology · 2019Article
- The characterization of AD/PART co-pathology in CJD suggests independent pathogenic mechanisms and no cross-seeding between misfolded Aβ and prion proteins.Acta neuropathologica communications · 2019Article
- Variably protease-sensitive prionopathy mimicking frontotemporal dementia.Neuropathology : official journal of the Japanese Society of Neuropathology · 2019Article
- Genetic PrP Prion Diseases.Cold Spring Harbor perspectives in biology · 2018Review
- Prion disease.Handbook of clinical neurology · 2018Review
- Atypical Creutzfeldt-Jakob disease with PrP-amyloid plaques in white matter: molecular characterization and transmission to bank voles show the M1 strain signature.Acta neuropathologica communications · 2017Article
- Accuracy of diagnosis criteria in patients with suspected diagnosis of sporadic Creutzfeldt-Jakob disease and detection of 14-3-3 protein, France, 1992 to 2009.Euro surveillance : bulletin Europeen sur les maladies transmissibles = European communicable disease bulletin · 2017Article
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Authors and funding
16 authors at 9 institutions in 4 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Prion diseases are rare and fatal neurodegenerative disorders that can be sporadic, inherited or acquired by infection. Based on a national surveillance program in the Netherlands we describe here the clinical, neuropathological, genetic and molecular characteristics of 162 patients with neuropathologically confirmed prion disease over a 12-year period (1998-2009). Since 1998, there has been a relatively stable mortality of Creutzfeldt-Jakob disease (CJD) in the Netherlands, ranging from 0.63 to 1.53 per million inhabitants per annum. Genetic analysis of the codon 129 methionine/valine (M/V) polymorphism in all patients with sporadic CJD (sCJD) showed a trend for under-representation of VV cases (7.0%), compared with sCJD cohorts in other Western countries, whereas the MV genotype was relatively over-represented (22,4%). Combined PrP(Sc) and histopathological typing identified all sCJD subtypes known to date, except for the VV1 subtype. In particular, a "pure" phenotype was demonstrated in 60.1% of patients, whereas a mixed phenotype was detected in 39.9% of all sCJD cases. The relative excess of MV cases was largely accounted for by a relatively high incidence of the MV 2K subtype. Genetic analysis of the prion protein gene (PRNP) was performed in 161 patients and showed a mutation in 9 of them (5.6%), including one FFI and four GSS cases. Iatrogenic CJD was a rare phenomenon (3.1%), mainly associated with dura mater grafts. Three patients were diagnosed with new variant CJD (1.9%) and one with variably protease-sensitive prionopathy (VPSPr). Post-mortem examination revealed an alternative diagnosis in 156 patients, most commonly Alzheimer's disease (21.2%) or vascular causes of dementia (19.9%). The mortality rates of sCJD in the Netherlands are similar to those in other European countries, whereas iatrogenic and genetic cases are relatively rare. The unusual incidence of the VV2 sCJD subtype compared to that reported to date in other Western countries deserves further investigation.
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