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 of corticobasal degeneration (corticobasal syndrome) include:
Corticobasal degeneration gets worse over 6 to 8 years. Eventually, people with the disease lose the ability to walk.
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.
There are no known risk factors for corticobasal degeneration (corticobasal syndrome).
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.
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.
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.
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.
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:
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:
Don't hesitate to ask other questions.
Your healthcare professional is likely to ask you several questions, such as:
