Sporadic Creutzfeldt-Jakob disease with MM1-type prion protein and plaques.
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.
Ishida
C
C
Department of Neurology and Neurobiology of Aging, Kanazawa University Graduate School of Medical Science, Ishikawa 920-8640, Japan. cishida@noto-hospital.jp
Kakishima
A
A
Okino
S
S
Furukawa
Y
Y
Kano
M
M
Oda
Y
Y
Nakanishi
I
I
Makifuchi
T
T
Kitamoto
T
T
Yamada
M
M
D000368:Aged; D015153:Blotting, Western; D001921:Brain / Q000473:pathology; D001923:Brain Chemistry; D002556:Cerebrospinal Fluid Proteins / Q000032:analysis; D007562:Creutzfeldt-Jakob Syndrome / Q000150:complications / Q000175:diagnosis* / Q000473:pathology; D004569:Electroencephalography; D005260:Female; D005911:Gliosis / Q000209:etiology / Q000473:pathology; D006801:Humans; D007150:Immunohistochemistry; D007177:Inappropriate ADH Syndrome / Q000150:complications / Q000175:diagnosis; D008279:Magnetic Resonance Imaging; D058225:Plaque, Amyloid / Q000473:pathology*; D011328:Prions / Q000032:analysis* / Q000235:genetics
D002556:Cerebrospinal Fluid Proteins; D011328:Prions