title: Structural and functional neuroimaging in human prion diseases.
abstract: BACKGROUND
  Prion diseases are neurodegenerative disorders resulting from the accumulation of a misfolded isoform of the cellular prion protein (PrPc). They can occur as acquired, sporadic, or hereditary forms. Although prion diseases show a wide range of phenotypic variations, pathological features and clinical evolution, they are all characterised by a common unfavourable course and a fatal outcome.
  
  
  RESULTS
  Some variants, such as kuru, have practically disappeared, while others, for example the variant Creutzfeldt-Jakob (vCJD) or those attributable to iatrogenic causes, are still in force and pose a challenge to current medicine. There are no definitive pre-mortem diagnostic tests, except for vCJD, where a tonsil biopsy detects 100% of the cases. For this reason, diagnostic criteria dependent on statistical probability have had to be created. These require complementary examinations, such as an electroencephalogram (EEG) or the detection of 14-3-3 protein in cerebrospinal fluid (CSF). Only the pulvinar sign in magnetic resonance imaging (MRI) has been included as a vCJD diagnostic criterion. The present review discusses neuroimaging findings for each type of prion disease in patients with a definitive histopathological diagnosis.
  
  
  CONCLUSIONS
  The aim is to define the usefulness of these complementary examinations as a tool for the diagnosis of this family of neurodegenerative diseases.
authors.affiliation: Departamento de Neurología, Clínica Universidad de Navarra, Pamplona, Navarra, Spain. sortegac@unav.es
authors.forename: S
authors.initials: S
authors.lastname: Ortega-Cubero
authors.forename: M R
authors.initials: MR
authors.lastname: Luquín
authors.forename: I
authors.initials: I
authors.lastname: Domínguez
authors.forename: J
authors.initials: J
authors.lastname: Arbizu
authors.forename: I
authors.initials: I
authors.lastname: Pagola
authors.forename: M M
authors.initials: MM
authors.lastname: Carmona-Abellán
authors.forename: M
authors.initials: M
authors.lastname: Riverol
mesh_terms: D048948:14-3-3 Proteins / Q000134:cerebrospinal fluid; D001921:Brain / Q000473:pathology*; D007562:Creutzfeldt-Jakob Syndrome / Q000175:diagnosis / Q000473:pathology; D004569:Electroencephalography; D016098:Gerstmann-Straussler-Scheinker Disease / Q000175:diagnosis / Q000473:pathology; D006801:Humans; D034062:Insomnia, Fatal Familial / Q000175:diagnosis / Q000473:pathology; D007729:Kuru / Q000175:diagnosis / Q000473:pathology; D008279:Magnetic Resonance Imaging; D059906:Neuroimaging; D018621:PrPC Proteins / Q000134:cerebrospinal fluid / Q000378:metabolism; D017096:Prion Diseases / Q000175:diagnosis / Q000473:pathology*
chemical_list: D048948:14-3-3 Proteins; D018621:PrPC Proteins