Corticobasal Degeneration and Frontotemporal Dementia Presentations in a Kindred with Nonspecific HistopathologyBoeve 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
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.
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 email@example.com
Received: Accepted: August 27, 2001
Number of Print Pages : 11
Number of Figures : 7, Number of Tables : 2, Number of References : 43
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