Selective Neuronal and Network Vulnerability in Frontotemporal Dementia

Abstract

Event Abstract Back to Event Selective Neuronal and Network Vulnerability in Frontotemporal Dementia William Seeley1* 1 University of California, San Francisco, Memory and Aging Center , United States Neurodegenerative diseases target specific neurons within nodes participating in large-scale distributed networks (Seeley, Crawford, Zhou, Miller, & Greicius, 2009). Often, disease spreads along anatomically constrained and predictable interconnected pathways. Frontotemporal dementia (FTD), once conceived as a diffuse frontotemporal disorder, can now be viewed as resulting from damage to a specific anterior cingulate-frontoinsular system (Seeley, 2008). This network incorporates subcortical and brainstem emotional-autonomic regulatory centers and enables normal social behavior in the healthy brain. Within the anterior cingulate and frontoinsular cortex, patients with the behavioral variant of FTD show selective loss of von Economo neurons, which are large bipolar, layer V projection neurons found only within this network and only in humans, apes, elephants, and cetaceans: large-brained mammals with complex social structures. The defining biochemical, genetic, physiological, and connectional properties of these neurons remain to be elucidated and could shed light on their vulnerability in FTD. Understanding this neural system, moreover, could clarify core aspects of human sociality and other disorders defined by social-emotional impairment.