With corticobasal degeneration, a progressive brain disease, you eventually lose your ability to walk and talk. Early symptoms are similar to Parkinson’s disease. A gene change may cause corticobasal degeneration. While there aren’t any treatments to slow or stop the loss of brain cells, speech, physical and occupational therapy may be beneficial.
Corticobasal degeneration is a neurological (brain) disorder. It damages cells in your brain, causing parts of it to shrink (atrophy). The disease’s symptoms may first appear on one side of your body, but eventually affect both sides as the disease progresses. With progression, you gradually lose your ability to talk, swallow and move. The disease could also affect other areas of thinking, including judgment and memory.
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“Corticobasal” refers to the two parts of your brain the disease affects. “Degeneration” refers to deterioration or loss of function.
The disease affects your:
Corticobasal degeneration is a rare brain disease. Diagnosis is challenging. Estimates indicate that as many as 3,000 Americans may have the condition, but only about 700 have received the correct diagnosis.
Corticobasal syndrome is a term used to specify that the symptoms, taken together, are similar to corticobasal degeneration. Many underlying neurodegenerative diseases can be caused by corticobasal syndrome, including:
Researchers currently think corticobasal degeneration is a form of frontotemporal degeneration and distinct from Alzheimer’s disease. The breakdown of cells here affects your brain’s frontal and temporal lobes.
The condition occurs when a tau, a protein normally found in brain cells, abnormally clumps together. These clumps, or neurofibrillary tangles, cause your brain cells to degenerate or die. This leads to problems with movement, speech and memory.
Medical experts don’t know why some people develop corticobasal degeneration. The condition rarely affects members of the same family.
Nine in 10 people with the condition have a gene change (mutation) on chromosome 17. Called the H1 haplotype, this gene change may increase the production of tau, causing the protein to clump together. Or it may cause a carbon atom and hydrogens (a methyl group) to stick to the tau gene, affecting its function. But this gene mutation isn’t the only factor — the same mutation is present in 3 out of 4 people in the general population.
Signs of corticobasal degeneration typically appear when you’re in your 60s. Some people show symptoms as early as in their 40s.
The disease affects everyone differently. It often first occurs on one limb or one side of your body and then progresses to the rest of your body. Symptoms worsen as the disease affects more of your brain.
Corticobasal degeneration symptoms include:
Some symptoms of corticobasal degeneration mimic those of other conditions, such as:
To make a diagnosis, your healthcare provider will conduct a physical exam. They carefully evaluate your symptoms. While this condition happens due to a gene change, no genetic test can detect it or predict it.
You may also receive:
For corticobasal degeneration, therapies focus on helping compensate for the difficulties with coordination and speech. Medication therapies may help manage muscle jerks or help improve attention or mood-related changes. Treatments for other movement disorders like Parkinson’s disease aren’t often effective. There isn’t a treatment to cure the disease.
Your healthcare provider may recommend:
A person with corticobasal degeneration has increasing difficulty taking care of themselves. People may live five to 10 years after the onset of symptoms. Many of these individuals die from disease complications resulting from falls, lack of mobility or infections related to their inability to maintain their personal hygiene.
The disease increases the risk of life-threatening complications, such as:
Call your healthcare provider if you or a loved one experiences:
You may want to ask your healthcare provider:
A note from Cleveland Clinic
Corticobasal degeneration is a rare, slow-progressing brain disease that affects memory, communication and movement. It causes symptoms similar to Parkinson’s disease: muscle spasms and twitches, tremors and slowed movements. It can also affect your ability to talk, swallow and complete simple tasks like buttoning a shirt. As the disease progresses, dementia or memory loss may occur. There currently isn’t a cure for the disease. But therapies for speech and movements may help you maintain independence longer.
Last reviewed by a Cleveland Clinic medical professional on 03/16/2022.
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