Corticobasal degeneration and frontotemporal dementia presentations in a kindred with nonspecific histopathology

Bradley F. Boeve, D. M. Maraganore, J. E. Parisi, R. J. Ivnik, B. F. Westmoreland, D. W. Dickson, M. Hutton, J. Hardy, R. J. Caselli, R. C. Petersen

Research output: Contribution to journalArticle

21 Scopus citations

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.

Original languageEnglish (US)
Pages (from-to)80-90
Number of pages11
JournalDementia and geriatric cognitive disorders
Volume13
Issue number2
DOIs
StatePublished - Mar 2 2002

Keywords

  • Corticobasal degeneration
  • Dementia lacking distinctive histopathology
  • Frontotemporal dementia
  • Neurogenetics

ASJC Scopus subject areas

  • Geriatrics and Gerontology
  • Cognitive Neuroscience
  • Psychiatry and Mental health

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