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Vol. 13, No. 2, 2002
Issue release date: February 2002
Section title: Original Research Article
Dement Geriatr Cogn Disord 2002;13:80–90
(DOI:10.1159/000048638)

Corticobasal Degeneration and Frontotemporal Dementia Presentations in a Kindred with Nonspecific Histopathology

Boeve B.F. · Maraganore D.M. · Parisi J.E. · Ivnik R.J. · Westmoreland B.F. · Dickson D.W. · Hutton M. · Hardy J. · Caselli R.J. · Petersen R.C.
Departments of aNeurology, bLaboratory Medicine and Pathology and cPsychiatry and Psychology, Mayo Clinic, Rochester, Minn., Laboratories of dNeuropathology and eNeurogenetics, Mayo Clinic, Jacksonville, Fla., fDepartment of Neurology, Mayo Clinic, Scottsdale, Ariz., USA

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Article / Publication Details

First-Page Preview
Abstract of Original Research Article

Published online: 2/15/2002

Number of Print Pages: 11
Number of Figures: 7
Number of Tables: 2

ISSN: 1420-8008 (Print)
eISSN: 1421-9824 (Online)

For additional information: http://www.karger.com/DEM

Abstract

We report the clinical, neuropsychological, electroencephalographic and radiologic findings in a kindred with varying clinical presentations of a neurodegenerative disorder. Postmortem examination of one member with clinically suspected corticobasal degeneration (CBD) revealed nonspecific histopathology maximally involving the frontoparietal cortex with negligible degenerative changes in the basal ganglia and substantia nigra. The findings in this and other kindreds demonstrate that (1) similar findings on ancillary testing can occur in relatives presumably suffering from the same pathophysiologic process despite dissimilar clinical presentations, (2) the ‘CBD syndrome’ is not specific for CBD, (3) extrapyramidal dysfunction can exist in the absence of appreciable basal ganglia and nigral degeneration, (4) nonspecific histopathology can underlie familial focal/asymmetric cortical degeneration syndromes and (5) many of the findings in CBD are comparable to those reported in frontotemporal dementia.


  

Author Contacts

Bradley F. Boeve, MD
Mayo Clinic, Department of Neurology
200 First Street SW
Rochester, MN 55905 (USA)
Tel. +1 507 284 8983, Fax +1 507 266 4752, E-Mail bboeve@mayo.edu

  

Article Information

Received: Accepted: August 27, 2001
Number of Print Pages : 11
Number of Figures : 7, Number of Tables : 2, Number of References : 43

  

Publication Details

Dementia and Geriatric Cognitive Disorders

Vol. 13, No. 2, Year 2002 (Cover Date: Released February 2002)

Journal Editor: V. Chan-Palay, New York, N.Y.
ISSN: 1420–8008 (print), 1421–9824 (Online)

For additional information: http://www.karger.com/journals/dem


Article / Publication Details

First-Page Preview
Abstract of Original Research Article

Published online: 2/15/2002

Number of Print Pages: 11
Number of Figures: 7
Number of Tables: 2

ISSN: 1420-8008 (Print)
eISSN: 1421-9824 (Online)

For additional information: http://www.karger.com/DEM


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