Corticobasal Syndrome

What is Corticobasal Syndrome?

Corticobasal Syndrome, also known as corticobasal degeneration, is a rare and progressive neurological disorder. It is caused by an abnormal buildup of tau proteins in the brain, which interferes with normal nerve cell function. Over time, these damaged nerve cells die, leading to shrinkage in certain areas of the brain. Corticobasal syndrome is a type of frontotemporal dementia as it primarily affects the frontal and temporal regions of the brain, which are responsible for movement, thinking, behavior and language.

Who is Affected?

• This is a rare condition, but impacts men and women at similar rates
• It primarily impacts older adults

What are the Causes & Risk Factors?

Corticobasal syndrome is caused by abnormal build-up of tau protein in the brain cells, but it is not yet known what causes this abnormal build up.

Risk Factors You Cannot Control

• Increasing age

Risk Factors You Can Control

• None known at this time

Who is Most at Risk?

• Older adults are most at risk, though it is a rare disorder
• What Steps Can You Take to Reduce Your Risk?
• There are no known ways to prevent this rare disorder.

What are Signs & Symptoms of Corticobasal Syndrome?

Symptoms of corticobasal syndrome develop gradually and worsen over time.

Early symptoms often affect movement on one side of the body and may include:

• Stiffness or rigidity in one arm or leg
• Difficulty controlling movement on one side of the body
• Poor coordination or balance
• Muscle jerking or involuntary movements
• Difficulty performing purposeful movement despite having enough strength

As the disease progresses, additional symptoms may appear and can include:

• Trouble finding the right words
• Difficulty expressing thoughts clearly
• Problems with reading
• Changes in thinking or memory
• Personality or behavior changes

How is Corticobasal Syndrome Diagnosed?

There is no single test that can definitively diagnose corticobasal syndrome. The diagnosis is based on a detailed medical history, neurological examination and other tests to rule out similar conditions. Because symptoms overlap with other neurological disorders, diagnosis can be challenging.

What Tests are Used?

• Neurological and physical examinations
• Cognitive and language assessments
• Brain imaging (such as MRI or CT) to look for brain shrinkage patterns
• Additional lab or diagnostic imaging tests to rule out similar conditions

How is Corticobasal Syndrome Treated?

There is currently no cure for corticobasal syndrome and no treatments that can slow the progression of the disease.

Treatment focuses on managing symptoms and maintaining quality of life.

Lifestyle

• Maintaining regular daily routines
• Implementing fall prevention strategies
• Support for daily activities

Devices

• Mobility aids (canes, walkers, etc.)
• Adaptive tools to assist with daily tasks

Medical

•  Medications to help manage muscle stiffness or movement symptoms
• Medications to address mood or behavioral changes

Surgical

• There are no surgical treatments for corticobasal syndrome

Other

• Physical therapy to support mobility and balance
• Speech therapy to address communication and swallowing challenges
• Occupational therapy to assist with activities of daily living
• Caregiver education and support services

When to See Your Doctor

You should see a doctor if you or a loved one experiences ongoing stiffness, difficulty controlling movement on one side of the body or progressive changes in speech, thinking or behavior.

When to Seek Emergency Care

Seek immediate medical care if symptoms appear suddenly or are accompanied by severe confusion, sudden weakness, difficulty speaking or other neurological symptoms that could indicate a different medical emergency, such as a stroke.

What Should You Do Next?

If you are concerned about corticobasal syndrome or other type of dementia, talk to your primary care provider about your symptoms. They may refer you to a neurologist for further evaluation and treatment. Early evaluation can help guide symptom management and connect families and caregivers with supportive resources.