S1-04-05: Neuropathology of frontotemporal dementia

Abstract

The frontotemporal dementias (FTD) or Pick complex constitute a group of diseases characterized by focal atrophy preferentially involving the anterior lobes of the brain in a bilateral asymmetrical fashion. Some patients present with behavioral disturbances, whereas others manifest language impairments, either non–fluent primary progressive aphasia or semantic dementia. To determine the probability of the association of a given syndrome with a histological substrate. Study of our own 60 case series, supported by review of the literature. Diseases with tau cytoplasmic neuronal inclusions–Pick's disease and corticobasal degeneration– represent nearly half of all cases, and are the most common cause of primary progressive aphasia. Cases with ubiquitin only –motor neuron disease type– cytoplasmic inclusions are the most common substrate of FTD overall, and especially in cases with behavioral or semantic dementia presentations. A subgroup with autosomal dominant disease transmission is characterized by additional intranuclear ubiquitin inclusions.