Corticobasal degeneration

Corticobasal degeneration is an uncommon neurodegenerative disease and is one of the subset of so-called taupathies.

The vast majority of cases are sporadic, although a number of familial cases have been described 2. Patients are usually elderly, with the youngest reported case being 40 years of age 3.

Patients present with slowly progressive symptoms and signs tyically including 1-2:

  • apraxia
  • dystonia
  • postural instability
  • akinetic-rigid syndrome
  • myoclonic jerks

In addition a degree of cognitive impariment is common, often with pronounced frontal lobe sign 2.

Alien limb phenomenon is also frequently observed, and is believed to be due to supplementary motor area involvement 1-2,4.

Unlike Parkinson disease, these symptoms are not ameliorated by levodopa 1

The characteristic histopathological findings are neuronal loss and numerous swollen achromatic neurons 1. Although these features are seen throughout the brain, certain regions are more severely affected. They include:

  • frontoparietal cortex
  • subcortical structures
    • striatum
    • substantia nigra

MRI is the modality of choice for assessing corticobasal degeneration, although similar findings can, only to a certain degree, be seen on CT.

MRI

Typical findings include 1-2:

  • asymmetric cortical atrophy
  • bilateral atrophy of the basal ganglia
  • atrophy of the corpus callosum 2
  • T2 hyperintensity
    • subcortical white matter of affected gyri
    • posterolateral putamen

SPECT and PET studies tend to demonstrate hypometabolism in the superior parietal and superior frontal areas, as well as (but less frequently) in the basal ganglia 2.

Unfortunately prognosis is poor, with patients demonstrating gradual neurological decline. Death occurs typically 5 to 10 years after the diagnosis is first made 3.

Clinically there is overlap with 1:


Neurodegenerative diseases

Neurodegenerative diseases are legion and their classification just as protean. A useful approach is to divide them according to underlying pathological process, although even using this schema, there is much overlap and thus resulting confusion.

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Article Information

rID: 12988
Tag: cases
Synonyms or Alternate Spellings:
  • Cortical-basal ganglionic degeneration
  • Corticonigral degeneration with neuronal achromasia
  • Cortical-basal ganglionic degeneration (CBGD)
  • CBGD
  • Corticobasal degeneration (CBD)

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