Right anterior temporal lobe atrophy and person-based semantic defect: a detailed case study.

We report a new case of a right temporal pole variant of frontotemporal dementia (Rtv-FTLD), MD, who presented a slowly progressive deterioration of the recognition of familiar and famous people. We thoroughly investigated MD's face processing and semantic abilities, including a neuroimaging investigation. This analysis revealed a cross-modal person-based deficit together with a more general semantic alteration. However, there was no evidence of impairment in face perception, including holistic processing, or of an abnormal pattern of brain activation in face-sensitive cortical areas. We discuss the nature of face processing in the Rtv-FTLD and the context of a person-based semantic defect.