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September 2016

Behavioral Variant Frontotemporal Dementia: One Syndrome, Several Circuits, Many Mechanisms

Author Affiliations
  • 1Department of Neurology, College of Medicine, Mayo Clinic, Rochester, Minnesota

Copyright 2016 American Medical Association. All Rights Reserved. Applicable FARS/DFARS Restrictions Apply to Government Use.

JAMA Neurol. 2016;73(9):1051-1052. doi:10.1001/jamaneurol.2016.1793

During the past 20 years, the clinical syndrome of behavioral variant frontotemporal dementia (bvFTD) has become better recognized, but it still represents a challenging diagnosis for many neurologists and psychiatrists, not to mention primary care physicians. Simultaneously, dramatic advances in our understanding of the neurobiological underpinnings of the frontotemporal degenerations have occurred, of which bvFTD is the most common cognitive clinical phenotype.

Behavioral variant FTD is characterized by a constellation of behavioral aberrations that include alterations in personality and dysfunctional changes in the conduct of interpersonal interactions.1 The analysis of the distinctive abnormal behaviors seen in bvFTD has helped to advance our understanding of how the brain constructs appropriate behavior in the normal situations. However, the study of bvFTD has had consequences that go far beyond the psychology of abnormal behavior. Imaging and neuropathologic studies of bvFTD have had an immense effect on understanding how cortical and subcortical structures are involved in the control of behavior. The publication of consensus criteria for the diagnosis of bvFTD by an international consortium1 highlighted the degree of agreement about the prototypical clinical features.