Unusual clinical presentations of cortical-basal ganglionic degeneration

Ann Neurol. 1996 Dec;40(6):893-900. doi: 10.1002/ana.410400611.


Cortical-basal ganglionic degeneration classically presents predominantly as a motor disorder with a unique constellation of histological alterations characterized by the presence of neuronal loss and gliosis in a selective distribution, swollen achromasic neurons, and extensive deposition of abnormal tau in neurons and glia. We now report 3 patients with this distinctive pathology who presented with cognitive changes and only mild or delayed motor symptoms. In 2 patients with severe dementia, pathological changes were extensive in the anterior frontal lobe, amygdala, and hippocampus. In a third patient who had an isolated speech disturbance for 5 years before developing the more typical motor features of cortical-basal ganglionic degeneration, the most severe changes were observed in the left motor cortex and adjacent Broca's area. It is therefore apparent that the histological changes of cortical-basal ganglionic degeneration result in a variety of clinical presentations depending on the topography of the lesions. On this basis we conclude that cortical-basal ganglionic degeneration should be considered in the differential diagnosis of language disturbances and dementia, particularly when the latter is accompanied by frontal lobe symptomatology, early speech alterations, or parkinsonism.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Aged
  • Basal Ganglia Diseases / pathology*
  • Brain Diseases / metabolism
  • Brain Diseases / pathology*
  • Cerebral Cortex / chemistry
  • Cerebral Cortex / pathology*
  • Gliosis / pathology
  • Humans
  • Male
  • tau Proteins / analysis


  • tau Proteins