Codistribution of Amyloid β Plaques and Spongiform Degeneration in Familial Creutzfeldt-Jakob Disease With the E200K-129M Haplotype

Validation of the Erlangen Score Algorithm for Differential Dementia Diagnosis in Autopsy-Confirmed Subjects

“…On the other hand, we observed that 23% (13 out of 57) of the definite non-AD patients, which were categorized as neurochemically probable AD (ES = 4). This, in turn, is in line with the presence of concomitant AD pathology in non-AD dementia patients, as reported previously [28][29][30]. Indeed, many of the non-AD cases in this study that had an ES suggestive for AD pathological findings (n = 7), presented with AD-related neuropathological changes that may have had a higher impact than expected.…”

Validation of the Erlangen Score Algorithm for Differential Dementia Diagnosis in Autopsy-Confirmed Subjects

“…On the other hand, we observed that 23% (13 out of 57) of the definite non-AD patients, which were categorized as neurochemically probable AD (ES = 4). This, in turn, is in line with the presence of concomitant AD pathology in non-AD dementia patients, as reported previously [28][29][30]. Indeed, many of the non-AD cases in this study that had an ES suggestive for AD pathological findings (n = 7), presented with AD-related neuropathological changes that may have had a higher impact than expected.…”

Specific amyloid-β42 deposition in the brain of a Gerstmann-Sträussler-Scheinker disease patient with a P105L mutation on the prion protein gene

“…Colocalization of PrP and Aβ in the same kuru plaques in patients with GSS was previously described [9], including various type of GSS, such as GSS-P105L [8], GSS-A117V [10] or GSS-P102L [11], and fCJD with E200K mutations [12]. Aβ deposits were primarily observed to be surrounding PrP deposits, but PrP deposits were never located around the Aβ deposits.…”

The characterization of AD/PART co-pathology in CJD suggests independent pathogenic mechanisms and no cross-seeding between misfolded Aβ and prion proteins

“…This is the largest study to date on the characterization of AD/PART co-pathology in a CJD cohort representative of both sporadic and genetic forms and all major disease subtypes. Several previous studies provided evidence of a significant association between CJD and AD in both humans and animal models [25, 36, 79]. These data, combined with others indicating that PrP C acts as a receptor mediating the internalization of Aβ aggregates, facilitating their neurotoxicity, are often taken as evidence of shared pathogenic pathways between the two disorders [35, 65].…”

“…Previous findings of an excess of AD-related pathological changes in gCJD compared to sCJD [25, 36, 45] led to the suggestion that PRNP mutations may favor AD pathology possibly through a cross-seeding mechanism. To address this issue, we compared the relative level of AD pathology between genetic and sporadic CJD.…”

Validation of the Erlangen Score Algorithm for Differential Dementia Diagnosis in Autopsy-Confirmed Subjects

“…On the other hand, we observed that 23% (13 out of 57) of the definite non-AD patients, which were categorized as neurochemically probable AD (ES = 4). This, in turn, is in line with the presence of concomitant AD pathology in non-AD dementia patients, as reported previously [28][29][30]. Indeed, many of the non-AD cases in this study that had an ES suggestive for AD pathological findings (n = 7), presented with AD-related neuropathological changes that may have had a higher impact than expected.…”

Specific amyloid-β42 deposition in the brain of a Gerstmann-Sträussler-Scheinker disease patient with a P105L mutation on the prion protein gene

“…Colocalization of PrP and Aβ in the same kuru plaques in patients with GSS was previously described [9], including various type of GSS, such as GSS-P105L [8], GSS-A117V [10] or GSS-P102L [11], and fCJD with E200K mutations [12]. Aβ deposits were primarily observed to be surrounding PrP deposits, but PrP deposits were never located around the Aβ deposits.…”

The characterization of AD/PART co-pathology in CJD suggests independent pathogenic mechanisms and no cross-seeding between misfolded Aβ and prion proteins

“…This is the largest study to date on the characterization of AD/PART co-pathology in a CJD cohort representative of both sporadic and genetic forms and all major disease subtypes. Several previous studies provided evidence of a significant association between CJD and AD in both humans and animal models [25, 36, 79]. These data, combined with others indicating that PrP C acts as a receptor mediating the internalization of Aβ aggregates, facilitating their neurotoxicity, are often taken as evidence of shared pathogenic pathways between the two disorders [35, 65].…”

“…Previous findings of an excess of AD-related pathological changes in gCJD compared to sCJD [25, 36, 45] led to the suggestion that PRNP mutations may favor AD pathology possibly through a cross-seeding mechanism. To address this issue, we compared the relative level of AD pathology between genetic and sporadic CJD.…”

Validation of the Erlangen Score Algorithm for Differential Dementia Diagnosis in Autopsy-Confirmed Subjects

“…On the other hand, we observed that 23% (13 out of 57) of the definite non-AD patients, which were categorized as neurochemically probable AD (ES = 4). This, in turn, is in line with the presence of concomitant AD pathology in non-AD dementia patients, as reported previously [28][29][30]. Indeed, many of the non-AD cases in this study that had an ES suggestive for AD pathological findings (n = 7), presented with AD-related neuropathological changes that may have had a higher impact than expected.…”

Specific amyloid-β42 deposition in the brain of a Gerstmann-Sträussler-Scheinker disease patient with a P105L mutation on the prion protein gene

“…Colocalization of PrP and Aβ in the same kuru plaques in patients with GSS was previously described [9], including various type of GSS, such as GSS-P105L [8], GSS-A117V [10] or GSS-P102L [11], and fCJD with E200K mutations [12]. Aβ deposits were primarily observed to be surrounding PrP deposits, but PrP deposits were never located around the Aβ deposits.…”

The characterization of AD/PART co-pathology in CJD suggests independent pathogenic mechanisms and no cross-seeding between misfolded Aβ and prion proteins

“…This is the largest study to date on the characterization of AD/PART co-pathology in a CJD cohort representative of both sporadic and genetic forms and all major disease subtypes. Several previous studies provided evidence of a significant association between CJD and AD in both humans and animal models [25, 36, 79]. These data, combined with others indicating that PrP C acts as a receptor mediating the internalization of Aβ aggregates, facilitating their neurotoxicity, are often taken as evidence of shared pathogenic pathways between the two disorders [35, 65].…”

“…Previous findings of an excess of AD-related pathological changes in gCJD compared to sCJD [25, 36, 45] led to the suggestion that PRNP mutations may favor AD pathology possibly through a cross-seeding mechanism. To address this issue, we compared the relative level of AD pathology between genetic and sporadic CJD.…”