Clinical Features and Diagnosis of Frontotemporal DementiaKertesz A.
St. Joseph’s Hospital, University of Western Ontario, London, Ont., Canada
Do you have an account?
- Rent for 48h to view
- Buy Cloud Access for unlimited viewing via different devices
- Synchronizing in the ReadCube Cloud
- Printing and saving restrictions apply
Rental: USD 8.50
Cloud: USD 20.00
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.
© 2009 S. Karger AG, Basel
Article / Publication Details
Copyright / Drug Dosage / DisclaimerCopyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher or, in the case of photocopying, direct payment of a specified fee to the Copyright Clearance Center.
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 government 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.
Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.