Neuropsychological, behavioral, and anatomical evolution in right temporal variant frontotemporal dementia: A longitudinal and post-mortem single case analysis

Abstract

We describe a patient with semantic variant of frontotemporal dementia who received longitudinal clinical evaluations and structural MRI scans and subsequently came to autopsy. She presented with early behavior changes and semantic loss for foods and people and ultimately developed a pervasive semantic impairment affecting social-emotional as well as linguistic domains. Imaging revealed predominant atrophy of the right temporal lobe, with later involvement of the left, and pathology confirmed bilateral temporal involvement. Findings support the view that left and right anterior temporal lobes serve as semantic hubs that may be affected differentially in semantic variant by early, relatively unilateral damage.

Keywords:

• Semantic dementia
• Frontotemporal dementia
• Right temporal variant of FTD
• Temporal lobe
• TDP-43

This work was supported by NIH (NIDCD F32DC010945, NIA P50 AG023501, NIA P01 AG019724, NIA R01 AG033017, NINDS R01 NS050915), Alzheimer’s Disease Research Center of California (03-75271 DHS/ADP/ARCC); Larry L. Hillblom Foundation; John Douglas French Alzheimer’s Foundation; Koret Family Foundation; McBean Family Foundation; and the Consortium for Frontotemporal Dementia Research.