Corticobasal degeneration (corticobasal syndrome)

Overview

Corticobasal degeneration, also called corticobasal syndrome, is a rare disease that causes areas of the brain to shrink. Over time, nerve cells break down and die.

Corticobasal degeneration affects the area of the brain that processes information and brain structures that control movement. People with this disease have trouble with movement on one or both sides of the body. Trouble with movement gets worse over time.

Symptoms also may include poor coordination, stiffness, trouble thinking, and trouble with speech or language.

Symptoms

Symptoms of corticobasal degeneration (corticobasal syndrome) include:

Trouble moving on one or both sides of the body, which gets worse over time.
Poor coordination.
Trouble with balance.
Stiffness.
Postures of the hands or feet that can't be controlled. For example, the hand may form a clenched fist.
Muscle jerks.
Trouble swallowing.
Changes in eye movements.
Trouble with thinking and language skills.
Speech changes, such as slow and halting speech.

Corticobasal degeneration gets worse over 6 to 8 years. Eventually, people with the disease lose the ability to walk.

Causes

Corticobasal degeneration (corticobasal syndrome) can have several causes. Most commonly, the disease results from a buildup of a protein called tau in brain cells. The buildup of tau may lead to the breakdown of the cells. This can cause symptoms of corticobasal degeneration.

Half of the people who have symptoms have corticobasal degeneration. But the second most common cause of corticobasal degeneration symptoms is Alzheimer's disease. Other causes of corticobasal degeneration include progressive supranuclear palsy, Pick's disease or Creutzfeldt-Jakob disease.

Risk factors

There are no known risk factors for corticobasal degeneration (corticobasal syndrome).

Complications

People with corticobasal degeneration (corticobasal syndrome) may develop serious complications. People with the disease may develop pneumonia, blood clots in the lungs or a dangerous response to an infection, known as sepsis. Complications often lead to death.

Diagnosis

A diagnosis of corticobasal degeneration (corticobasal syndrome) is made based on your symptoms, exam and testing. However, your symptoms could be due to another disease that affects the brain. Conditions that cause similar symptoms include progressive supranuclear palsy, Alzheimer's disease, Pick's disease or Creutzfeldt-Jakob disease.

You may need an imaging test such as an MRI or CT scan to rule out these other conditions. Sometimes, these tests are performed every few months to look for changes in the brain.

Positron emission tomography (PET) scans can identify brain changes related to corticobasal degeneration. However, more research needs to be done in this area.

Your healthcare professional may test your blood or cerebrospinal fluid for amyloid and tau proteins. This can determine whether Alzheimer's disease is the cause of your symptoms.

Treatment

There are no treatments that help slow the progression of corticobasal degeneration (corticobasal syndrome). But if your symptoms are due to Alzheimer's disease, new medicines may be available. Your healthcare professional may recommend medicines to try to manage your symptoms.

Occupational and physical therapy may help you manage the disabilities caused by corticobasal degeneration. Walking devices may help with mobility and prevent falls. Speech therapy can help with communication and swallowing. A dietitian may help you ensure you get proper nutrition and lower the risk of inhaling food into the lungs, known as aspiration.

Preparing for an appointment

You may start by seeing your healthcare professional. Or you may be referred immediately to a specialist, such as a neurologist.

Here's some information to help you get ready for your appointment.

What you can do

When you make the appointment, ask if there's anything you need to do in advance. For example, you might ask if you need to fast before a specific test. Make a list of:

Your symptoms, including any that seem unrelated to the reason for your appointment.
Key personal information, including major stresses, recent life changes and family medical history.
All medicines, vitamins or other supplements you take, including the doses.
Questions to ask.

Take a family member or friend along to help you remember the information you're given.

For corticobasal degeneration, some basic questions to ask include:

What's likely causing my symptoms?
Other than the most likely cause, what are other possible causes for my symptoms?
What tests do I need?
Is my condition likely temporary or chronic?
What's the best course of action?
What are the alternatives to the primary approach you're suggesting?
I have these other health conditions. How can I best manage them together?
Are there restrictions I need to follow?
Should I see a specialist?
Are there brochures or other printed material I can have? What websites do you recommend?

Don't hesitate to ask other questions.

What to expect from your doctor

Your healthcare professional is likely to ask you several questions, such as:

When did your symptoms begin?
Have your symptoms been continuous or occasional?
How bad are your symptoms?
What, if anything, seems to improve your symptoms?
What, if anything, appears to worsen your symptoms?