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Progress in Clinical Neurosciences: Frontotemporal Dementia-Pick's Disease

Published online by Cambridge University Press:  02 December 2014

Andrew Kertesz
Andrew Kertesz*
Affiliation:
Department of Clinical Neurological Sciences, St. Joseph's Hospital, University of Western Ontario, London, Ontario, Canada
*
Department of Clinical Neurological Sciences, St. Joseph’s Hospital, University of Western Ontario, London, Ontario, N6A 4V2, Canada.
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Abstract:

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Frontotemporal dementia (clinical Pick's disease) is a relatively common, but underdiagnosed degenerative disease in the presenium. Estimated prevalence ranges from 6-12% of dementias. The behavioural, aphasic and extrapyramidal presentations are labeled FTD-behavioural variant, Primary Progressive Aphasia (PPA) and Corticobasal Degeneration/Progressive Supranuclear Palsy (CBD/PSP). The diagnostic features and course of each are described and their overlap in the evolution of the illness is emphasized. The neuropathology ranges from the most common tau negative ubiquitin positive amyotrophic lateral sclerosis (ALS) type inclusions to the tau positive classical Pick bodies and more or less distinct changes of PSP and CBD. The genetics of the relatively frequent tau mutations and the yet unsolved problem of tau negative families are discussed. The tau negative cases tend to be associated with the behavioural presentation and semantic dementia and the tau positive ones with PPA and the CBD/PSP syndrome. However the overlap is too great to split the disease. A glossary to navigate the proliferating terminology is included.

Résumé:

RÉSUMÉ:

La démence fronto-temporale (DFT - maladie Clinique de Pick) estunemaladierelativementfréquente mais sous-diagnostiquée chez les patients préséniles Sa prévalence serait de 6 à 12% chez les patients atteints de démence. Les manifestations comportementales, aphasiques et extrapyramidalessontconsidérées comme des variantescomportementales de la DFT, de l’aphasie progressive primaire (APP) et de la dégénérescence cortico-basale (DCB)/paralysiesupranucléaire progressive (PSP). Nous décrivons les manifestations diagnostiques et l’évolution de chacune et nous soulignonsleurchevauchement au cour de l’évolution de la maladie. La neuropathologievarie de la forme la plus fréquente qui est la présence d’inclusions de type DLA ubiquitine positives tau négatives, aux corps de Pick classiques tau positifs et aux changements plus oumoinsdistincts de la PSP et de la DCB. Nous discutons des aspects génétiques des mutations relativementfréquentes de la protéine tau et du problème non résolu des familles qui ne sont pas porteuses de mutations de cetteprotéine. Les cas tau négatifs sont en général associés à des manifestations comportementales et à une démence sémantique et les cas tau positifsà l’APP et au syndrome DCB/PSP. Cependant, le chevauchementest trop considérable pour en faire différentes entités. Nous ajoutons un glossaireafin de définir uneterminologie enexpansion.

Type
Review Article
Information
Canadian Journal of Neurological Sciences , Volume 33 , Issue 2 , May 2006 , pp. 141 - 148
Copyright
Copyright © The Canadian Journal of Neurological 2006

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