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Corticobasal Degeneration

Abstract

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NORD is very grateful to Suzee Lee, MD, Assistant Professor, UCSF Memory and Aging Center, for assistance in the preparation of this report.

Synonyms of Corticobasal Degeneration

  • CBD
  • CBGD
  • cortical basal ganglionic degeneration

Disorder Subdivisions

  • No subdivisions found.

General Discussion

Summary
Corticobasal degeneration (CBD) is a rare progressive neurological disorder characterized by cell loss and deterioration of specific areas of the brain. Affected individuals often initially experience motor abnormalities in one limb that eventually spreads to affect all the arms and legs. Such motor abnormalities include muscle rigidity and the inability to perform purposeful or voluntary movements (apraxia). Affected individuals may have sufficient muscle power for manual tasks, but often have difficulty directing their movements appropriately. Although CBD was historically described as a motor disease, it is now recognized that cognitive and behavioral symptoms also herald CBD and not uncommonly predate motor symptoms. Initial symptoms typically appear in people during the sixth decade, and may include poor coordination or difficulty accomplishing goal-directed tasks (e.g., buttoning a shirt). The exact cause of corticobasal degeneration is unknown.

Introduction
Because signs and symptoms associated with corticobasal degeneration are frequently caused by other neurodegenerative disorders, researchers use the term "corticobasal syndrome" to indicate the clinical diagnosis based on signs and symptoms. The term "corticobasal degeneration" refers to those meeting the neuropathological criteria for the disorder at autopsy. This is an important distinction because clinicopathological series indicate that about less than half of patients diagnosed with corticobasal syndrome during life actually has corticobasal degeneration at autopsy.

Organizations related to Corticobasal Degeneration

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