Article
A novel phenotype of sporadic Creutzfeldt-Jakob disease.
Journal of neurology, neurosurgery, and psychiatry - 1 Dec 2007
Giaccone G, Di Fede G, Mangieri M, Limido L, Capobianco R, Suardi S, Grisoli M, Binelli S, Fociani P, Bugiani O, Tagliavini F
Abstract excerpt
An atypical case of sporadic Creutzfeldt-Jakob disease (CJD) is described in a 78-year-old woman homozygous for methionine at codon 129 of the prion protein (PrP) gene. The neuropathological signature was the presence of PrP immunoreactive plaque-like deposits in the cerebral cortex, striatum and thalamus. Western blot analysis showed a profile of the pathological form of PrP (PrP(Sc)) previously unrecognised in...
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