Human Prion Diseases in The Netherlands (1998–2009): Clinical, Genetic and Molecular Aspects

  title={Human Prion Diseases in The Netherlands (1998–2009): Clinical, Genetic and Molecular Aspects},
  author={Casper Jansen and Piero Parchi and S Capellari and Carla A. Ibrahim-Verbaas and Maaike Schuur and Rosaria Strammiello and Patrizia Corrado and Matthew T. Bishop and Willem A van Gool and Marcel M Verbeek and Frank Baas and Wesley van Saane and Wim G. M. Spliet and G. H. Jansen and Cornelia M van Duijn and Annemieke J. M. Rozemuller},
  booktitle={PloS one},
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… CONTINUE READING
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