Cover

Dementia in Clinical Practice

Editor(s): Giannakopoulos P. (Geneva) 
Hof P.R. (New York, N.Y.) 
Table of Contents
Vol. 24, No. , 2009
Section title: Frontotemporal Dementia
Giannakopoulos P, Hof PR (eds): Dementia in Clinical Practice. Front Neurol Neurosci. Basel, Karger, 2009, vol 24, pp 140–148
(DOI:10.1159/000197893)

Clinical Features and Diagnosis of Frontotemporal Dementia

Kertesz A.
St. Joseph’s Hospital, University of Western Ontario, London, Ont., Canada

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Article / Publication Details

First-Page Preview
Abstract of Frontotemporal Dementia

Published online: 1/26/2009
Cover Date: 2009

Number of Print Pages: 9
Number of Figures: 0
Number of Tables: 0

ISBN: 978-3-8055-9015-0 (Print)
eISBN: 978-3-8055-9016-7 (Online)

Abstract

Frontotemporal degeneration (FTD), formerly known as Pick’s disease has become recognized as adistinct and relatively common entity encompassing behavioural (bvFTD language (PPA) andextrapyramidal (CBD/PSP) presentations. Further clinical subdivisions such as semantic dementia(SD), and pathological subtypes such as mesial temporal sclerosis increase the complexity of diagnosis.The relatively younger age of onset, the typical presentations of syndromes and focal asymmetricalfrontotemporal atrophy on imaging allows experienced clinicians to make the diagnosisconfidently as long as the overlap between the syndromes is recognized. There is also an overlapwith ALS pathologically and clinically. The underlying histology in FTD/Pick complex is ubiquitinpositive tau and synuclein negative neuronal inclusions (FTLD-U) in more than half of autopsies andtau positive CBD/PSP/ Pick bodies (FTLD-T) in the rest. The clinical syndromes of bvFTD and SD arelikely associated with FTLD-U and PPA/CBDS/PSP with FTLD-T, but there is too much overlap to predictthe pathology from the clinical syndromes reliably. The ubiquitin-tau pathological dichotomy isbest considered under the Pick complex umbrella to allow for the significant overlap. So far trazodonein behavior and galantamine in aphasia had symptomatic benefit in small trials and SSRI-sand antipsychotics in uncontrolled reports were used as symptomatic therapies. Recent discoveriesof tau and progranulin (in the ubiquitin-positive cases) mutations on chromosome 17 and othermutations on chromosome 3 and 9 in the high incidence of autosomal dominant families and acommon protein abnormality, the TDP-43 in FTLD-U and ALS are likely to be important in findingtherapeutic targets.


Article / Publication Details

First-Page Preview
Abstract of Frontotemporal Dementia

Published online: 1/26/2009
Cover Date: 2009

Number of Print Pages: 9
Number of Figures: 0
Number of Tables: 0

ISBN: 978-3-8055-9015-0 (Print)
eISBN: 978-3-8055-9016-7 (Online)


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Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in goverment regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.
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