Abstract
Although typical cases of Creutzfeldt-Jakob disease are readily recognized pathologically and clinically, variant forms often pose a diagnostic challenge. From the 1920's, when this disease was first characterized, until quite recently diagnosis relied heavily on morphologic changes. New advances in immunoassays and PrP gene analysis now provide important adjuncts in recognizing the spectrum of disorders of PrP metabolism associated with these transmissible encephalopathies.
Publication types
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Historical Article
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Review
MeSH terms
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Brain / metabolism
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Brain / pathology*
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Creutzfeldt-Jakob Syndrome* / classification*
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Creutzfeldt-Jakob Syndrome* / genetics
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Creutzfeldt-Jakob Syndrome* / history
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Creutzfeldt-Jakob Syndrome* / pathology
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DNA Mutational Analysis
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Female
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Gerstmann-Straussler-Scheinker Disease / classification
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Gerstmann-Straussler-Scheinker Disease / genetics
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Gerstmann-Straussler-Scheinker Disease / pathology
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History, 20th Century
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Humans
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Male
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PrPSc Proteins / genetics*
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PrPSc Proteins / metabolism
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Prion Diseases / classification*
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Prion Diseases / genetics
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Prion Diseases / history
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Prion Diseases / pathology