Clinicopathologic subtype of Alzheimer's disease presenting as corticobasal syndrome

Alzheimers Dement. 2019 Sep;15(9):1218-1228. doi: 10.1016/j.jalz.2019.04.011. Epub 2019 Aug 6.

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.

Keywords: Alzheimer's disease; Corticobasal syndrome; Neurodegeneration; Neuropathology; Tau.

Publication types

  • Research Support, N.I.H., Extramural

MeSH terms

  • Aged
  • Alzheimer Disease* / classification
  • Alzheimer Disease* / pathology
  • Cerebral Cortex / pathology*
  • Female
  • Gait Apraxia / etiology
  • Humans
  • Male
  • Middle Aged
  • Neurodegenerative Diseases* / diagnostic imaging
  • Neurodegenerative Diseases* / pathology
  • Parkinsonian Disorders
  • Temporal Lobe / pathology
  • tau Proteins

Substances

  • tau Proteins