Creutzfeldt‐Jakob disease with Alzheimer‐type Aβ‐reactive amyloid plaques

Abstract

Creutzfeldt‐Jakob disease and Gerstmann‐Sträussler‐Scheinker syndrome are classified as transmissible cerebral amyloidoses, in contrast to the non‐transmissible amyloidoses of Alzheimer's disease type. While the aetiologies of Creutzfeldt‐Jakob disease and Alzheimer's disease and the molecular composition of their amyloids are different, similar basic pathogenetic mechanisms operate in both diseases through synthesis and processing of amyloid precursor proteins, to produce an accumulation of amyloid deposits. We report here a case of Creutzfeldt‐Jakob disease exhibiting numerous diffuse Aβ immunoreactive plaques, thus presenting features of both Creutzfeldt‐Jakob disease and Alzheimer's disease. The existence of such cases underlines the existence of a ‘grey’ area between the two types of amyloidoses.

On the ‘classification’ of neurodegenerative disorders: discrete entities, overlap or continuum?

“…Aβ pla ques were present but with diffuse PrP sc immunoreactivity in the grey matter of the cerebral cortex and cerebellum. Similar cases were described by Barcikowska et al [23] in which CJD occurred together with diffuse Aβ plaques.…”

Alzheimer-type neuropathology in a 28 year old patient with iatrogenic Creutzfeldt-Jakob disease after dural grafting

“…29 33 There are several previous reports about Alzheimer-type changes in elderly sporadic CJD patients. 1 3 12 13 20 22 25 27 We report here the presence of plaque-type and vascular BA deposits in a 28 year old patient with iCJD who had undergone a post-traumatic dura transplantation 23 years before death. This case represents (a) the iCJD case with the longest incubation time after dural grafting reported so far, (b) the youngest documented patient with concomitant CJD and Alzheimer-type neuropathology to date, (c) the first description of Alzheimer-type changes in iCJD, and (d) the second case of iCJD in Austria.…”

Involvement of Dab1 in APP processing and β-amyloid deposition in sporadic Creutzfeldt–Jakob patients

“…Deposition of both PrP sc and Ain the central nervous system (CNS) results in neurodegeneration. Moreover, the coexistence of Aand PrP sc amyloid deposits in affected brains, although controversial, has been widely reported (Barcikowska et al, 1995;Debatin et al, 2008;Ferrer et al, 2001;Hainfellner et al, 1998;Leuba et al, 2000;Tsuchiya et al, 2004). In addition, a recent study indicates that PrP c may participate in the removal of A oligomers, which would implicate PrP c in AD (Charveriat et al, 2009).…”