Semantic Dementia: a specific network-opathy

Phillip D. Fletcher, Jason D. Warren
2011 Journal of Molecular Neuroscience  
Semantic dementia (SD) is a unique syndrome in the frontotemporal lobar degeneration spectrum. Typically presenting as a progressive, fluent anomic aphasia, SD is the paradigmatic disorder of semantic memory with a characteristic anatomical profile of asymmetric, selective antero-inferior temporal lobe atrophy. Histopathologically, most cases show a specific pattern of abnormal deposition of protein TDP-43. This relatively close clinical, anatomical and pathological correspondence suggests SD
more » ... a promising target for future therapeutic trials. Here, we discuss outstanding nosological and neurobiological challenges posed by the syndrome and propose a pathophysiological model of SD based on sequential, regionally determined disintegration of a vulnerable neural network.
doi:10.1007/s12031-011-9586-3 pmid:21710360 pmcid:PMC3207124 fatcat:m5zcovadfzb6dh7opuag5mbuie