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Corticobasal degeneration

  • Article
  • 2021-03-07

Corticobasal degeneration is a progressive neurological condition characterized by loss of nerve cells and atrophy (shrinkage) of multiple areas of the brain, including the cerebral cortex and basal ganglia. Corticobasal degeneration is gradual. The first symptoms, usually starting at or around age 60, may first appear on one side of the body (unilateral), but eventually appear on both sides as the disease progresses. Symptoms are similar to Parkinson's disease, such as poor coordination, akinesia (no movement), rigidity (resistance to imposed movement), imbalance (disturbed balance); and limb dystonia (abnormal muscle postures). Other symptoms such as cognitive and visual-spatial disturbances, apraxia (loss of the ability to make familiar, purposeful movements), hesitant and faltering speech, myoclonus (twitching), and dysphagia (difficulty swallowing) may also occur. A person with corticobasal degeneration eventually becomes unable to walk.

Therapy

No treatment is available to slow the course of corticobasal degeneration and the symptoms of the disease are generally resistant to therapy. Medicines used to treat the symptoms of Parkinson's disease do not provide significant or sustained improvement. Clonazepam can help the myoclonus. Occupational, physical, and speech therapy can help manage disabilities.

Prognosis

Corticobasal degeneration is usually slow over 6 to 8 years. Death is usually caused by pneumonia or other complications from severe weakness, such as sepsis or pulmonary embolism.


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