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Related Experiment Videos

Cortical-basal ganglionic degeneration.

D E Riley1, A E Lang, A Lewis

  • 1Department of Neurology, Case Western Reserve University, Cleveland, OH.

Neurology
|August 1, 1990
PubMed
Summary
This summary is machine-generated.

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Cortical-basal ganglionic degeneration (CBGD) presents unique symptoms from both cortical and basal ganglia dysfunction. Early clinical diagnosis is possible, though autopsy confirms this rare neurological disorder.

Area of Science:

  • Neurology
  • Neuroscience
  • Pathology

Background:

  • Cortical-basal ganglionic degeneration (CBGD) is a rare neurodegenerative disorder.
  • It involves both cortical and basal ganglia dysfunction, leading to a distinctive clinical presentation.

Observation:

  • We describe 15 patients with suspected CBGD.
  • Key symptoms include cortical sensory loss, alien limb phenomena, apraxia, rigidity, akinesia, tremor, dystonia, hyperreflexia, and postural instability.
  • Asymmetrical symptoms and contralateral brain imaging abnormalities are often observed.

Findings:

  • Postmortem examination of 2 patients revealed characteristic features: swollen, achromatic neurons and degeneration of the cerebral cortex and substantia nigra.
  • Biochemical analysis showed a severe, diffuse loss of dopamine in the striatum.

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  • The condition appears more frequent than previously thought.

    • Implications:

    • Clinical diagnosis of CBGD is feasible during a patient's lifetime based on characteristic findings.
    • While clinical suspicion is high, definitive diagnosis requires postmortem confirmation.
    • Understanding CBGD's clinical and pathological features aids in recognizing this complex neurological condition.