Frontotemporal dementia (FTD) encompasses a group of neurodegenerative disorders characterized by cognitive and behavioral impairments. Heterozygous mutations in progranulin (PGRN) cause familial FTD and result in decreased PGRN expression, while homozygous mutations result in complete loss of PGRN expression and lead to the neurodegenerative lysosomal storage disorder neuronal ceroid lipofuscinosis...
Primary Progressive Aphasia (PPA) is an aphasic dementia syndrome caused by neurodegenerative disease in which linguistic abilities are selectively disrupted in the relative absence of other cognitive deficits in early stages. The studies presented in this thesis were designed to examine the integrity of word and object processing by contrasting...