Accurate assessment of behavioral variant frontotemporal dementia

Abstract

Behavioral variant Frontotemporal dementia (bvFTD) is a neurodegenerative syndrome characterized by profound changes in personality and behavior, including social disinhibition, loss of empathy, apathy and compulsive behaviors. While cognitive decline does occur (typically beginning with executive dysfunction), these issues tend to emerge mid-disease course, rather than early on. Onset is insidious, typically beginning between ages 45-65 and prevalence is equal to Alzheimer's disease (AD) in individuals under the age of 65. Despite significant advancements in our understanding of bvFTD over the past 12 years, misdiagnosis remains common. For example, a significant subset of individuals with bvFTD initially receive a diagnosis of early-onset AD, or late life psychiatric disturbance. Given their expertise in the assessment of cognition, behavior and emotion, neuropsychologists can play an important role in the differential diagnosis and management of this disease. This chapter begins with an up-to-date discussion of the clinical, neuropathological and genetic features of the disease, and then moves into a review of the neuropsychological literature. A structured discussion of key aspects to cover in a neuropsychological assessment is provided, and a case example of a 'typical' bvFTD patient is presented.