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Assessment of Behavioral Variant Frontotemporal Dementia

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Handbook on the Neuropsychology of Aging and Dementia

Part of the book series: Clinical Handbooks in Neuropsychology ((CHNEURO))

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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 and 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 20 years, misdiagnosis remains common. For example, many 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.

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Notes

  1. 1.

    For a recent review on the genetics of FTD, please read Pottier et al. [56].

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Authors and Affiliations

  1. Department of Psychiatry, University of British Columbia, Vancouver, BC, Canada

    Amanda K. LaMarre

  2. University of California, San Francisco, CA, USA

    Joel H. Kramer

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  2. Joel H. Kramer
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Correspondence to Amanda K. LaMarre .

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Editors and Affiliations

  1. Department of Neurology, Weill Cornell Medicine, New York, NY, USA

    Lisa D. Ravdin

  2. Department of Neurology, University of Miami, Miami, FL, USA

    Heather L. Katzen

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LaMarre, A.K., Kramer, J.H. (2019). Assessment of Behavioral Variant Frontotemporal Dementia. In: Ravdin, L.D., Katzen, H.L. (eds) Handbook on the Neuropsychology of Aging and Dementia. Clinical Handbooks in Neuropsychology. Springer, Cham. https://doi.org/10.1007/978-3-319-93497-6_33

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