Corticobasal syndrome

What is corticobasal syndrome?

Corticobasal syndrome (CBS), also called corticobasal degeneration, is a progressive brain disease. It happens when nerve cells in the brain begin to shrink (atrophy) and die (neurodegeneration). Over time, these changes can affect the ability to control movement, think, speak, and swallow.

Penn Medicine’s movement disorders care specialists diagnose and care for people with complex and rare disorders like corticobasal syndrome. At our Movement Disorders Center, our team sees more people with CBS than many other programs, giving us deep experience with this uncommon condition. Our researchers work on the leading edge of discovery about the causes of these disorders. We create custom treatment plans based on the latest research to help you manage your symptoms and maintain your independence.

How corticobasal syndrome affects the body

CBS is rare, affecting about five out of every 100,000 people. It usually begins after age 60 and it hasn’t been reported in people younger than 40. The disease affects everyone differently, but symptoms often start on just one side of the body before eventually spreading to the other side.

CBS can look a lot like Parkinson’s disease. When a condition shares symptoms with Parkinson's but is a different disease, it’s called atypical Parkinsonism.

Causes of corticobasal neurodegeneration

Scientists don't fully understand why people develop corticobasal neurodegeneration. The condition results in damage to the front and side regions (frontotemporal lobes) of the brain.

Researchers have discovered that a protein called tau clumps together in brain tissue in people with corticobasal syndrome. The abnormal buildup of tau damages brain cells, leading to the symptoms of corticobasal syndrome. Alzheimer's disease is another example of a disorder caused by abnormal tau protein buildup (tauopathy).

Corticobasal syndrome is not considered an inherited condition, but there are some rare cases where it runs in families. Scientists are working to uncover the genes that may be responsible.

Tests used to diagnose corticobasal syndrome

Corticobasal syndrome is diagnosed by a neurologist, a doctor specialized in disorders of the nervous system, including the brain. Your neurologist will ask about your symptoms and perform a comprehensive physical examination.

There is no specific test for corticobasal syndrome. Diagnosis is what's called a diagnosis of exclusion. This means that your doctor diagnoses corticobasal syndrome only after ruling out other conditions.

Managing symptoms of corticobasal syndrome

There’s no cure for corticobasal syndrome, but treatments can help manage symptoms. Your care team will work with you on a customized treatment plan.

Your provider may suggest medications to help with memory, muscle stiffness, or spasms. Physical and occupational therapy are also helpful for learning new ways to handle daily tasks so you can stay independent for as long as possible. Speech therapists can provide exercises to help you communicate more clearly and swallow safely.

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