Clinicopathologic subtype of Alzheimer's disease presenting as corticobasal syndrome

Nobutaka Sakae, Keith Anthony Josephs, Irene Litvan, Melissa E Murray, Ranjan Duara, Ryan J. Uitti, Zbigniew K Wszolek, Jay van Gerpen, Neill R Graff Radford, Dennis W Dickson

Research output: Contribution to journalArticle

4 Scopus citations

Abstract

Introduction: The corticobasal syndrome (CBS) is associated with several neuropathologic disorders, including corticobasal degeneration and Alzheimer's disease (AD). Method: In this report, we studied 43 AD patients with CBS (AD-CBS) and compared them with 42 AD patients with typical amnestic syndrome (AD-AS), as well as 15 cases of corticobasal degeneration and CBS pathology. Results: Unlike AD-AS, AD-CBS had prominent motor problems, including limb apraxia (90%), myoclonus (81%), and gait disorders (70%). Alien limb phenomenon was reported in 26% and cortical sensory loss in 14%. Language problems were also more frequent in AD-CBS, and memory impairment was less frequent. AD-CBS had more tau pathology in perirolandic cortices but less in superior temporal cortex than AD-AS. In addition, AD-CBS had greater neuronal loss in the substantia nigra. Discussion: AD-CBS is a clinicopathological subtype of AD with an atypical distribution of Alzheimer-type tau pathology. Greater neuronal loss in the substantia nigra may contribute to Parkinsonism which is not a feature of typical AD.

Original languageEnglish (US)
Pages (from-to)1218-1228
Number of pages11
JournalAlzheimer's and Dementia
Volume15
Issue number9
DOIs
StatePublished - Sep 1 2019

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Keywords

  • Alzheimer's disease
  • Corticobasal syndrome
  • Neurodegeneration
  • Neuropathology
  • Tau

ASJC Scopus subject areas

  • Epidemiology
  • Health Policy
  • Developmental Neuroscience
  • Clinical Neurology
  • Geriatrics and Gerontology
  • Cellular and Molecular Neuroscience
  • Psychiatry and Mental health

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