title: Plaque-type deposition of prion protein in the damaged white matter of sporadic Creutzfeldt-Jakob disease MM1 patients.
abstract: Plaque-type deposition of prion protein (PrP) in the brain has been extremely rare in sporadic Creutzfeldt-Jakob disease patients with methionine homozygosity at polymorphic codon 129 of the PrP gene and type 1 abnormal isoform of PrP (sCJD-MM1). Here we report three sCJD-MM1 patients who showed prominent PrP-positive amyloid plaques in the cerebral and cerebellar white matter. All three patients showed clinical courses of long duration (2 years < or =), particularly at the end-stage. The white matter of these patients was severely damaged because of the prolonged disease duration. Furthermore, Alzheimer's amyloid precursor protein, which accumulates within the axonal swellings under pathological conditions, co-accumulated with the PrP-amyloid plaques. These findings suggest that the axonal damage reflecting the prolonged disease duration causes the deposition of PrP-amyloid plaques in the white matter. The present study shows that PrP-amyloid plaques can occur in the white matter of sCJD-MM1 cases.
authors.affiliation: Division of CJD Science and Technology, Department of Prion Research, Tohoku University Graduate School of Medicine, 2-1 Seiryo-machi, Aoba-ku, Sendai, 980-8575, Japan.
authors.forename: Atsushi
authors.initials: A
authors.lastname: Kobayashi
authors.forename: Kunimasa
authors.initials: K
authors.lastname: Arima
authors.forename: Masafumi
authors.initials: M
authors.lastname: Ogawa
authors.forename: Miho
authors.initials: M
authors.lastname: Murata
authors.forename: Takahiro
authors.initials: T
authors.lastname: Fukuda
authors.forename: Tetsuyuki
authors.initials: T
authors.lastname: Kitamoto