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

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 histopatho...

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Published in:Dementia and geriatric cognitive disorders 2002-01, Vol.13 (2), p.80-90
Main Authors: 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.
Format: Article
Language:English
Subjects:
Basal Ganglia Diseases - diagnosis
Basal Ganglia Diseases - genetics
Basal Ganglia Diseases - pathology
Biological and medical sciences
Brain - diagnostic imaging
Brain - pathology
Cerebral Cortex
Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases
Dementia
Dementia - diagnosis
Dementia - genetics
Dementia - pathology
Electroencephalography
Humans
Magnetic Resonance Imaging
Male
Medical sciences
Middle Aged
Neurodegenerative Diseases - diagnosis
Neurodegenerative Diseases - genetics
Neurodegenerative Diseases - pathology
Neurology
Neuropsychological Tests
Original Research Article
Pedigree
Tomography, Emission-Computed, Single-Photon
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container_title Dementia and geriatric cognitive disorders
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creator 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.
description 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.
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subjects Basal Ganglia Diseases - diagnosis
Basal Ganglia Diseases - genetics
Basal Ganglia Diseases - pathology
Biological and medical sciences
Brain - diagnostic imaging
Brain - pathology
Cerebral Cortex
Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases
Dementia
Dementia - diagnosis
Dementia - genetics
Dementia - pathology
Electroencephalography
Humans
Magnetic Resonance Imaging
Male
Medical sciences
Middle Aged
Neurodegenerative Diseases - diagnosis
Neurodegenerative Diseases - genetics
Neurodegenerative Diseases - pathology
Neurology
Neuropsychological Tests
Original Research Article
Pedigree
Tomography, Emission-Computed, Single-Photon
title Corticobasal Degeneration and Frontotemporal Dementia Presentations in a Kindred with Nonspecific Histopathology
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