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Clinicopathologic subtype of Alzheimer's disease presenting as corticobasal syndrome.

  • Author(s): Sakae, Nobutaka
  • Josephs, Keith A
  • Litvan, Irene
  • Murray, Melissa E
  • Duara, Ranjan
  • Uitti, Ryan J
  • Wszolek, Zbigniew K
  • van Gerpen, Jay
  • Graff-Radford, Neil R
  • Dickson, Dennis W
  • et al.
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.

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