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.
Symptoms of corticobasal syndrome may include:
- Balance and gait problems
- Difficulty moving one or both sides of the body (bradykinesia or slowed movements)
- Involuntary (uncontrolled) muscle movements, such as tremors (rhythmic shaking), jerks, or dystonia (muscle contractions)
- Memory problems or dementia
- Movement coordination problems
- Trouble with speaking and swallowing
- Vision problems due to abnormal eye movements
Symptoms of corticobasal syndrome also overlap with conditions other than Parkinson's disease. These conditions include:
- Brain tumors
- Dementia (especially frontotemporal dementia) or Alzheimer's disease
- Multiple system atrophy
- Progressive supranuclear palsy
- Stroke
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.
Related specialties
Patient stories
Essential tremor patient finds relief
When his essential tremor symptoms became severe, Jim McDevitt turned to Dr. Iahn Cajigas and the team at Penn Medicine for help.
Essential tremor treatment rekindles a man's passion for art
A tremor forced Mike Burton to abandon his love of art. Now he’s painting again, thanks to a noninvasive procedure that eliminated his shaking.