Behavioral Variant Frontotemporal Dementia: Diagnosis and Treatment Interventions

Purpose of Review The diagnosis and treatment of behavioral variant frontotemporal dementia is challenging and often delayed because of overlapping symptoms with more common dementia syndromes or primary psychiatric illnesses. The purpose of this paper is to explore the relevant presentation, diagnostic workup, pathophysiology, and both pharmacologic and non-pharmacologic management. Recent Findings Behavioral variant frontotemporal dementia is a highly heritable disorder. The gradual accumulation of diseased protein culminates in the destruction of those brain circuits responsible for much of one's emotional and social functioning. Behavioral variant frontotemporal dementia is a progressive neurodegenerative disorder with a far-reaching impact on patients and caregivers. Patients often present with emotional blunting, lack of empathy, apathy, and behavioral disinhibition. Non-pharmacologic interventions and caregiver support are the cornerstone of treatment. The use of cholinesterase inhibitors and memantine is not supported by the evidence. While current pharmacologic therapies target only certain symptoms, there are disease modifying agents currently in or nearing the clinical research stage.