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Corticobasal Degeneration

Corticobasal degeneration (CBD) is a rare neurological disease associated with progressive brain degeneration. The disease, also known as corticobasal ganglionic degeneration, leads to the loss of brain tissue in the cortex, or outer layer of the brain, especially the area in the upper, front section of the brain.

The brain tissue of patients with CBD show cell changes that also appear in patients with two other disorders — frontotemporal dementia and progressive supranuclear palsy. These changes involve a brain protein called tau and may provide researchers with some initial clues regarding the causes of corticobasal degeneration. Currently, there are no known causes, such as toxins or infections.

The gradual loss of brain tissue and symptoms typically begin between ages 45 and 70. Initial symptoms include stiffness; shaky, slow or clumsy movements; and difficulty with speech and comprehension.

Other symptoms include:

  • Balance — Difficulty walking and balancing
  • Memory — Short-term memory problems, such as repeating questions or misplacing objects
  • Muscle Control — Difficulty controlling muscles of the face and mouth
  • Speech — Progressive difficulty speaking and difficulty comprehending language

Symptoms related to muscle control usually begin on one side of the body and spread gradually to the other.

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Early in the course of the disease, it may be difficult to distinguish corticobasal degeneration (CBD) from other neuro-degenerative diseases or forms of dementia. Diagnosis involves a comprehensive neurological exam, combined with one or more types of laboratory evaluations:

These scan provide images of the brain in the areas most frequently involved in the disease — the upper and front sections of the cortex or outer layer of the brain, and the basal ganglia at the base of the forebrain, which is associated with functions such as motor skills and learning.

Currently, there are no treatments to slow the progress of corticobasal degeneration (CBD). Instead, individual symptoms are targeted with specific medications or therapy. For example, rigidity and difficulty walking may partially respond to treatments for Parkinson's disease.

Muscle contractions and and twitching may respond to muscle relaxants or anti-seizure medications.

Memory and behavior problems may respond to treatments for Alzheimer's disease or depression.

Other therapies include:

  • Occupational therapy may be used to design equipment that supports the activities of daily living and maintains functional independence.
  • Physical therapy is important for maintaining a patient's range of motion. This may prevent pain and contraction or the shortening of muscles as well as help maintain mobility.
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Reviewed by health care specialists at UCSF Medical Center.

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