Prosopagnosia as unusual presentation of semantic dementia: a case study

Abstract

Object: Semantic dementia is one of the main clinical variants of frontotemporal dementia and it is characterized by severe loss of conceptual knowledge with relative preservation of other cortical abilities.

Methods: We described a case of a patient affected by right temporal variant of frontotemporal lobar degeneration, characterized by prosopagnosia as an unusual disease onset.

Results: The neuropsychological evaluation showed a progressive deficit in face recognition and a slow decline in language in the absence of behavioral alterations. Magnetic resonance imaging showed diffuse vascular ischemic lesions, cortical atrophy in bilateral temporal lobes, and a marked bilateral decrease hippocampal volume, mainly involving the right side.

Conclusions: Our findings suggest that prosopagnosia could be a predictive marker of atypical semantic dementia onset.

Keywords:

• Frontotemporal lobar degeneration
• Hippocampus atrophy
• Prosopagnosia
• Semantic dementia