Alzheimer and Frontotemporal Pathology in Subsets of Primary Progressive Aphasia

摘要: Objective To identify predictors of Alzheimer's disease (AD) versus frontotemporal lobar degeneration pathology in primary progressive aphasia (PPA), and determine whether the AD is atypically distributed to fit aphasic phenotype. Methods Neuropsychological neuropathological analyses 23 consecutive PPA autopsies. All had qualitative determination neurofibrillary tangle (NFT) density. Additional quantitation was done four PPA/AD cases with typical amnestic dementia Alzheimer type. Results The sample contained mostly logopenic, agrammatic, mixed forms PPA. six agrammatics (five tauopathy). Seven 11 logopenics AD. In logopenics, lower memory scores increased probability AD, but there were exceptions. The group showed predominance entorhinal NFT type. small subgroup examined quantitatively, neocortical NFTs more numerous left hemisphere PPA/AD. However, asymmetry low inconsistent. Neuritic plaques did not display consistent asymmetry. Apolipoprotein E4, a major risk factor for predict PPA. Interpretation Subtyping helps at level. our results literature also indicate that no clinical predictor completely reliable individual patients. inconsistent concordance distribution asymmetric atrophy nonamnestic phenotype raises possibility markers encountered autopsy may always reflect nature initiating neurodegenerative process. Ann Neurol 2008