Jump to ContentJump to Main Navigation
Cognitive NeurologyA clinical textbook$
Users without a subscription are not able to see the full content.

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

Show Summary Details
Page of

PRINTED FROM OXFORD SCHOLARSHIP ONLINE (www.oxfordscholarship.com). (c) Copyright Oxford University Press, 2020. All Rights Reserved. An individual user may print out a PDF of a single chapter of a monograph in OSO for personal use. date: 29 January 2020

Frontotemporal lobar degeneration

Frontotemporal lobar degeneration

Chapter:
(p.229) Chapter 12 Frontotemporal lobar degeneration
Source:
Cognitive Neurology
Author(s):

Jared Narvid

Maria Luisa Gorno-Tempini

Publisher:
Oxford University Press
DOI:10.1093/acprof:oso/9780198569275.003.0013

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

Oxford Scholarship Online requires a subscription or purchase to access the full text of books within the service. Public users can however freely search the site and view the abstracts and keywords for each book and chapter.

Please, subscribe or login to access full text content.

If you think you should have access to this title, please contact your librarian.

To troubleshoot, please check our FAQs , and if you can't find the answer there, please contact us .