Younkin, Steven G

Publication Detail

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

B 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 (Profiled Authors: Hardy, John; Dickson, Dennis; Hutton, Michael; Petersen, Ronald C)

Department of Neurology, Mayo Clinic, Rochester, MN 55905, USA. bboeve@mayo.edu
Dementia and geriatric cognitive disorders 2002;13(2):80-90.

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.

3 Originating Grant

• 1.

  PETERSEN, RONALD C

  Mayo Alzheimer's Disease Research Center

  1 May 1999 - 30 April 2014

  NATIONAL INSTITUTE ON AGING

  Total Funding: $ 25,743,859

• 2.

  PETERSEN, RONALD C

  Alzheimers Disease Patient Registry

  30 September 1986 - 31 August 2014

  NATIONAL INSTITUTE ON AGING

  Total Funding: $ 17,300,028

• 3.

  Kokmen, Emre

  ALZHEIMERS DISEASE PATIENT REGISTRY

  Total Funding: $ 3,948,430

Scientific Context

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