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Neurology. 2003 Feb 11;60(3):514-7.

Sporadic Creutzfeldt-Jakob disease with MM1-type prion protein and plaques.

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Department of Neurology and Neurobiology of Aging, Kanazawa University Graduate School of Medical Science, Ishikawa 920-8640, Japan.


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.

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