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Primary Degenerative Dementia Without Alzheimer Pathology

Published online by Cambridge University Press:  18 September 2015

Arthur W. Clark ,
Charles L. White III ,
Herbert J. Manz ,
Irma M. Parhad ,
Bernadette Curry ,
Peter J. Whitehouse ,
John Lehmann  and
Joseph T. Coyle
Arthur W. Clark*
Affiliation:
Departments of Pathology and Clinical Neurosciences, University of Calgary School of Medicine, Calgary
Charles L. White III
Affiliation:
The Department of Pathology, Southwestern Medical School, Dallas, Texas
Herbert J. Manz
Affiliation:
The Departments of Pathology and Neurology, Georgetown University School of Medicine, Washington, D.C.
Irma M. Parhad
Affiliation:
Departments of Pathology and Clinical Neurosciences, University of Calgary School of Medicine, Calgary
Bernadette Curry
Affiliation:
Departments of Pathology and Clinical Neurosciences, University of Calgary School of Medicine, Calgary
Peter J. Whitehouse
Affiliation:
Departments of Neurology and Neuroscience, John Hopkins University School of Medicine, Baltimore, Maryland
John Lehmann
Affiliation:
Department of Neurosciences, CIBA-GEIGY Corporation, Summit, New Jersey
Joseph T. Coyle
Affiliation:
Departments of Psychiatry, Neurosciences, Pharmacology and Pediatrics, John Hopkins University School of Medicine, Baltimore, Maryland
*
Department of Pathology, 11th Floor, Foothills Hospital, 1403 -29th Street, N.W., Calgary, Alberta, Canada T2N 2T9
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Abstract:

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To define the pathology in cases of non-Alzheimer primary degenerative dementia (non-AD PDD), we have studied autopsies from four medical centres accessioned in consecutive years since 1976. Neurochemical studies of the basal forebrain-cortical (BF-C) cholinergic system have been conducted in cases from which frozen tissue was available. Twenty-two cases (mean age 70 years, range 47-86) in which the history was consistent with PDD, but which did not meet anatomic criteria for AD, were selected. Approximately 70 cases of PDD, which were accessioned in the same years and met the anatomic criteria for AD, were excluded. The pathologic findings permitted a classification into six groups: Lewy body disease (LBD), 4 cases; Pick's disease, 6 cases; cortical degeneration with motor neuron disease (CDmnd), 2 cases; hippocampal and temporal lobe sclerosis, 3 cases; few or nonspecific abnormalities, 5 cases; other disorders, 2 cases. Our findings suggest that LBD and Pick's disease account for a large proportion of cases of non-AD PDD in the presenile age group, but that a large number of other disorders occasionally present as PDD. Careful examination of the motor systems, as well as cerebral structures relate' to cognitive function, is important in the neuropathologic evaluation. Lesions of the BF-C cholinergic system have been most consistent and severe in LBD, and have not been identified in CDmnd.

Type
Cellular Clues to Pathogenesis
Information
Canadian Journal of Neurological Sciences , Volume 13 , Issue S4 , November 1986 , pp. 462 - 470
Copyright
Copyright © Canadian Neurological Sciences Federation 1986

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