Corticobasal degeneration

What is corticobasal degeneration?
Corticobasal degeneration is a progressive neurological disorder characterized by nerve cell loss and atrophy (shrinkage) of multiple areas of the brain including the cerebral cortex and the basal ganglia. Corticobasal degeneration progresses gradually. Initial symptoms, which typically begin at or around age 60, may first appear on one side of the body (unilateral), but eventually affect both sides as the disease progresses. Symptoms are similar to those found in Parkinson disease, such as poor coordination, akinesia (an absence of movements), rigidity (a resistance to imposed movement), disequilibrium (impaired balance); and limb dystonia (abnormal muscle postures). Other symptoms such as cognitive and visual-spatial impairments, apraxia (loss of the ability to make familiar, purposeful movements), hesitant and halting speech, myoclonus (muscular jerks), and dysphagia (difficulty swallowing) may also occur. An individual with corticobasal degeneration eventually becomes unable to walk.

Is there any treatment?
There is no treatment available to slow the course of corticobasal degeneration, and the symptoms of the disease are generally resistant to therapy. Drugs used to treat Parkinson disease-type symptoms do not produce any significant or sustained improvement. Clonazepam may help the myoclonus. Occupational, physical, and speech therapy can help in managing disability.

What is the prognosis?
Corticobasal degeneration usually progresses slowly over the course of 6 to 8 years. Death is generally caused by pneumonia or other complications of severe debility such as sepsis or pulmonary embolism.

What research is being done?
The NINDS supports and conducts research studies on degenerative disorders such as corticobasal degeneration. The goals of these studies are to increase scientific understanding of these disorders and to find ways to prevent, treat, and cure them.

Clinical Trials
Throughout the U.S. and Worldwide

Organizations
National Organization for Rare Disorders (NORD)
55 Kenosia Avenue
Danbury, CT 06810
https://www.rarediseases.org
203-744-0100,Voice Mail 800-999-NORD (6673)

CUREPSP (Foundation for PSP|CBD and Related Brain Diseases)
30 E. Padonia Road, Ste. 201
Timonium, MD 21093
https://www.psp.org/
410-785-7004, 800-457-4777

Association for Frontotemporal Degeneration (AFTD)
Radnor Station Building #2, Suite 320
290 King of Prussia Road
Radnor, PA 19087
https://www.theaftd.org
267-514-7221, 866-507-7222

This information was developed by the National Institute of Neurological Disorders and Stroke.

National Institute of Neurological Disorders and Stroke.NINDS Corticobasal Degeneration Information Page. Available at: https://www.ninds.nih.gov/health-information/disorders/corticobasal-degeneration. Accessed April 24, 2023.

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