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Cognitive NeurologyA clinical textbook$
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Stefano Cappa, Jubin Abutalebi, Jean-Francois Demonet, Paul Fletcher, and Peter Garrard

Print publication date: 2008

Print ISBN-13: 9780198569275

Published to Oxford Scholarship Online: March 2012

DOI: 10.1093/acprof:oso/9780198569275.001.0001

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Frontotemporal lobar degeneration

Frontotemporal lobar degeneration

(p.229) Chapter 12 Frontotemporal lobar degeneration
Cognitive Neurology

Jared Narvid

Maria Luisa Gorno-Tempini

Oxford University Press

Frontotemporal lobar degeneration (FTLD) is a syndrome of focal, often asymmetric, neurodegeneration presenting clinically as a progressive dementia associated with focal atrophy of the frontal and/or temporal lobes. The disease is capable of producing striking changes in personality, behaviour, and language, with a diverse range of clinical presentations. Two major clinical presentations of FTLD are recognized. In the frontal variant — usually designated frontotemporal dementia (FTD) — the emergence of disinhibition, mental rigidity, stereotyped and perseverative behaviour, hyperorality, and loss of insight give rise to marked changes in personality. An aphasic variant — designated primary progressive aphasia (PPA) — is also recognized. The fluent variant appear to have difficulty expressing and understanding meaning, a syndrome that has been given the name semantic dementia (SD). In contrast, the nonfluent variant — progressive nonfluent aphasia (PNFA). Because FTLD can be associated with degeneration of cortical and bulbar motor neurons and anterior horn cells of the spinal cord, some patients with FTD, SD, or PNFA also meet clinical criteria for amyotrophic lateral sclerosis (ALS).

Keywords:   frontotemporal lobar degeneration, asymmetric neurodegeneration, progressive dementia, frontotemporal dementia, primary progressive aphasia, progressive nonfluent aphasia

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