In vivo staging of frontotemporal lobar degeneration TDP-43 type C pathology

Abstract

Background: TDP-43 type C is one of the pathological forms of frontotemporal lobar degeneration (FTLD) and mainly associated clinically with the semantic variant of primary progressive aphasia (svPPA). We aimed to define in vivo the sequential pattern of neuroanatomical involvement in a cohort of patients with FTLD-TDP type C pathology. Methods: We extracted the volumes of a set of cortical and subcortical regions from MRI scans of 19 patients with post mortem confirmed TDP-43 type C pathology (all with left hemisphere-predominant atrophy at baseline). In the initial development phase, we used w-scores computed from 81 cognitively normal controls to define a set of sequential stages of neuroanatomical involvement within the FTLD-TDP type C cohort where a w-score of