Cortical-basal ganglionic degeneration

Neurology. 1990 Aug;40(8):1203-12. doi: 10.1212/wnl.40.8.1203.

Abstract

We report our experience with 15 patients believed to have cortical-basal ganglionic degeneration. The clinical picture is distinctive, comprising features referable to both cortical and basal ganglionic dysfunction. Characteristic manifestations include cortical sensory loss, focal reflex myoclonus, "alien limb" phenomena, apraxia, rigidity and akinesia, a postural-action tremor, limb dystonia, hyperreflexia, and postural instability. The asymmetry of symptoms and signs is often striking. Brain imaging may demonstrate greater abnormalities contralateral to the more affected side. Postmortem studies in 2 patients revealed the characteristic pathologic features of swollen, poorly staining (achromatic) neurons and degeneration of cerebral cortex and substantia nigra. Biochemical analysis of 1 brain showed a severe, diffuse loss of dopamine in the striatum. This condition is more frequent than previously believed, and the diagnosis can be predicted during life on the basis of clinical findings. However, as with other "degenerative" diseases of the nervous system, a definitive diagnosis of cortical-basal ganglionic degeneration requires confirmation by autopsy.

Publication types

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

MeSH terms

  • Aged
  • Autopsy
  • Basal Ganglia / diagnostic imaging
  • Basal Ganglia / pathology
  • Basal Ganglia Diseases / diagnostic imaging
  • Basal Ganglia Diseases / pathology
  • Basal Ganglia Diseases / physiopathology*
  • Brain Chemistry
  • Brain Diseases / diagnostic imaging
  • Brain Diseases / pathology*
  • Cerebral Cortex / diagnostic imaging
  • Cerebral Cortex / pathology*
  • Dopamine / analysis
  • Female
  • Homovanillic Acid / analysis
  • Humans
  • Male
  • Middle Aged
  • Neurons / pathology
  • Tomography, X-Ray Computed

Substances

  • Dopamine
  • Homovanillic Acid