The diagnosis of human prion diseases.
Prion diseases present a wide spectrum of clinical and neuropathological features. In this review, clinical and neuropathological findings are summarized along with criteria for the diagnosis of the molecular-phenotypic subtypes of sporadic Creutzfeldt-Jakob disease (CJD), familial CJD, new variant CJD, iatrogenic CJD, Gerstmann-Straussler-Scheinker disease, fatal familial insomnia and kuru.
Kordek
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Department of Pathology, Chair of Oncology, Medical University, Lódź, Poland. radekkordek@interia.pl
D000328:Adult; D017668:Age of Onset; D000368:Aged; D000682:Amyloid / Q000032:analysis / Q000235:genetics; D001284:Atrophy; D001921:Brain / Q000473:pathology; D002556:Cerebrospinal Fluid Proteins / Q000032:analysis; D002648:Child; D016000:Cluster Analysis; D003062:Codon / Q000235:genetics; D007562:Creutzfeldt-Jakob Syndrome / Q000175:diagnosis / Q000453:epidemiology / Q000235:genetics / Q000473:pathology / Q000635:transmission; D004196:Disease Outbreaks; D004569:Electroencephalography; D005006:Ethnicity; D005060:Europe / Q000453:epidemiology; D005260:Female; D016098:Gerstmann-Straussler-Scheinker Disease / Q000175:diagnosis; D006801:Humans; D007049:Iatrogenic Disease; D007729:Kuru / Q000175:diagnosis; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009512:New Guinea / Q000453:epidemiology; D017096:Prion Diseases / Q000145:classification / Q000175:diagnosis* / Q000453:epidemiology / Q000235:genetics / Q000473:pathology; D000072002:Prion Proteins; D011328:Prions / Q000145:classification / Q000235:genetics / Q000302:isolation & purification; D011498:Protein Precursors / Q000032:analysis / Q000235:genetics; D014481:United States / Q000453:epidemiology
D000682:Amyloid; D002556:Cerebrospinal Fluid Proteins; D003062:Codon; C512656:PRNP protein, human; D000072002:Prion Proteins; D011328:Prions; D011498:Protein Precursors