Physical Address

304 North Cardinal St.
Dorchester Center, MA 02124

Corticobasal Degeneration


KEY FACTS

Terminology

  • Corticobasal degeneration

    • Progressive neurodegenerative disease

    • Presents with cognitive dysfunction, “asymmetrical” parkinsonism

Imaging

  • Severe focal asymmetric cortical atrophy

    • Perirolandic (posterior frontal, parietal cortex)

    • Relative sparing of temporal, occipital regions

  • ↑ signal intensity in frontal &/or parietal subcortical white matter

  • Marked T2 hypointensity

    • Putamen, globi pallidi

  • FDG-18 PET decreased uptake in cortical and subcortical regions (frontal, temporal, sensorimotor, and parietal association cortices), caudate, lentiform nucleus, and thalamus

  • SPECT: Asymmetric hypoperfusion in frontoparietal lobes, basal ganglia (putamen), thalamus, and cerebellar hemispheres

Top Differential Diagnoses

  • Progressive supranuclear palsy

  • Frontotemporal lobar dementia/degeneration

  • Alzheimer disease

  • Dementia with Lewy bodies

  • Amyotrophic lateral sclerosis

Pathology

  • Hyperphosphorylated tau/abnormal filamentous inclusions accumulate in neurons/glia

Clinical Issues

  • Unilateral or asymmetrical parkinsonism

    • Dystonia, tremor

    • Ideomotor apraxia, “alien limb” phenomenon

  • Cognitive decline

Parasagittal T1WI MR through the right hemisphere in a 63-year-old man with corticobasal degeneration shows prominent volume loss in the posterior frontal and parietal cortex
. The anterior frontal lobe cortex is normal in appearance.

Coronal FLAIR MR in the same patient shows asymmetric parietal atrophy, worse on the right
, with subcortical white matter hyperintensity
.

(Courtesy A. Erbetta, MD.)

Axial T2 MR in a 71-year-old man with corticobasal degeneration demonstrates asymmetric atrophy and thin cortex in the left perirolandic region
.

Axial T2WI in a 72-year-old woman with corticobasal degeneration shows the hyperintense putaminal rim
suggestive of the disease.

TERMINOLOGY

Abbreviations

  • Corticobasal degeneration (CBD)

Synonyms

  • Corticobasal ganglionic degeneration (CBGD)

  • Corticodentatonigral degeneration with neuronal achromasia

Definitions

  • Progressive neurodegenerative disease

    • Presents with cognitive dysfunction, “asymmetrical” parkinsonism

    • Characterized pathologically by cortical and striatal tau protein accumulation

IMAGING

You're Reading a Preview

Become a Clinical Tree membership for Full access and enjoy Unlimited articles

Become membership

If you are a member. Log in here

Related Posts