title: Prion diseases: New considerations.
abstract: The transmissible spongiform encephalopathies, which include Creutzfeldt-Jakob disease, are fatal neurodegenerative disorders caused by the pathological accumulation of abnormal prion protein. The diagnosis of Creutzfeldt-Jakob disease is complex. The electroencephalogram, magnetic resonance imaging, lumbar puncture and genetic testing findings can help in the differential diagnosis of rapidly progressive dementia. There has recently been considerable debate as to whether proteins involved in the development of neurodegenerative diseases should be regarded as prions or only share prion-like mechanisms. Two recent reports described the detection of abnormal prion protein in the nasal mucosa and urine of patients with Creutzfeldt-Jakob disease. These findings raise major health concerns regarding the transmissibility of human prion diseases. We set out to address this neurological hot topic and to draw conclusions on the basis of what is known in the literature thus far.
authors.affiliation: Department of Neurology, University of Szeged, Semmelweis u. 6, H-6725 Szeged, Hungary. Electronic address: annus.adam@med.u-szeged.hu.
authors.forename: Ádám
authors.initials: Á
authors.lastname: Annus
authors.affiliation: Department of Neurology, University of Szeged, Semmelweis u. 6, H-6725 Szeged, Hungary. Electronic address: anett.csati@med.u-szeged.hu.
authors.forename: Anett
authors.initials: A
authors.lastname: Csáti
authors.affiliation: Department of Neurology, University of Szeged, Semmelweis u. 6, H-6725 Szeged, Hungary; MTA-SZTE Neuroscience Research Group, Szeged, Hungary. Electronic address: laszlo.vecsei@med.u-szeged.hu.
authors.forename: László
authors.initials: L
authors.lastname: Vécsei
mesh_terms: D006801:Humans; D009297:Nasal Mucosa / Q000737:chemistry*; D017096:Prion Diseases / Q000175:diagnosis* / Q000635:transmission; D000072002:Prion Proteins / Q000378:metabolism* / Q000652:urine; D014556:Urine / Q000737:chemistry*
chemical_list: D000072002:Prion Proteins
keywords: Creutzfeldt-Jakob disease; Diagnosis; Neurodegenerative diseases; Prion protein; Transmissibility