Abstract
The authors report a 75-year-old woman with atypical sporadic Creutzfeldt-Jakob disease (CJD) characterized by MM1-type prion protein (PrP) (methionine homozygosity at codon 129 in the PrP gene and type-1 protease-resistant PrP) and PrP plaques. This patient is the first case of sporadic CJD with plaque-forming MM1-type PrP, suggesting either a shared prion strain with the plaque-forming subset of dural graft-associated CJD or shared host genetic factors that are unrelated to the PrP genotype.
Publication types
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Case Reports
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Research Support, Non-U.S. Gov't
MeSH terms
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Aged
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Blotting, Western
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Brain / pathology
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Brain Chemistry
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Cerebrospinal Fluid Proteins / analysis
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Creutzfeldt-Jakob Syndrome / complications
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Creutzfeldt-Jakob Syndrome / diagnosis*
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Creutzfeldt-Jakob Syndrome / pathology
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Electroencephalography
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Female
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Gliosis / etiology
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Gliosis / pathology
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Humans
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Immunohistochemistry
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Inappropriate ADH Syndrome / complications
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Inappropriate ADH Syndrome / diagnosis
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Magnetic Resonance Imaging
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Plaque, Amyloid / pathology*
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Prions / analysis*
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Prions / genetics
Substances
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Cerebrospinal Fluid Proteins
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Prions