Locations:
Corticobasal degeneration (corticobasal syndrome) is a rare brain condition that causes you to lose your ability to speak, move and remember. Symptoms are progressive, so they slowly get worse over time. While there aren’t any treatments to slow or stop changes to your brain, therapies are available to help manage the condition.
Advertisement
Cleveland Clinic is a non-profit academic medical center. Advertising on our site helps support our mission. We do not endorse non-Cleveland Clinic products or services. Policy
Corticobasal degeneration, also known as corticobasal syndrome, is a neurological disorder that damages and destroys brain cells. It affects your ability to talk, swallow, move and remember. Over time, these symptoms get worse (progress).
Advertisement
Cleveland Clinic is a non-profit academic medical center. Advertising on our site helps support our mission. We do not endorse non-Cleveland Clinic products or services. Policy
“Corticobasal” refers to the two parts of your brain the disease affects:
“Degeneration” means deterioration or loss of function. In this case, it’s the loss of function of these two parts of your brain.
Corticobasal degeneration may shorten your life expectancy and lead to life-threatening complications. As symptoms affect more of your body, you may find it difficult to safely maintain your independence.
Corticobasal degeneration symptoms may affect your ability to:
You might also experience the following symptoms:
Advertisement
The condition affects everyone differently. It often happens in stages. You might first notice symptoms targeting one limb, like an arm, or only one side of your body. Then, it progresses to your other arm and legs. You may feel clumsy when walking, and then, not feel safe walking without support. You might stumble over a few words in a conversation, and then, not be able to make coherent sentences over time.
Symptoms worsen as the disease affects more of your brain. This happens gradually.
Symptoms of corticobasal degeneration typically start between ages 50 and 70. The average onset age is 64. Symptoms may start at age 40, but no reported cases happen before age 40.
The deterioration (breakdown) and death of brain cells and tissue cause corticobasal degeneration. The breakdown usually starts in your cerebral cortex and basal ganglia. The condition may affect other areas of your brain as it progresses.
Researchers believe that a protein called tau plays a role in corticobasal degeneration. Tau is a protein found in brain cells. It abnormally clumps together with this condition. These clumps, or neurofibrillary tangles, cause your brain cells to degenerate or die. This leads to problems with movement, speech and memory.
The exact reason why tau tangles happen isn’t well understood. Many people with corticobasal degeneration have a genetic variant (mutation) on chromosome 17 called the H1 haplotype. This gene change may increase the production of tau, causing the protein to clump together. Or it may cause a carbon atom and hydrogens (a methyl group) to stick to the tau gene, affecting its function.
Researchers also know that this gene isn’t the only factor that could cause corticobasal degeneration. Most people in the general population have this gene and don’t have symptoms of corticobasal degeneration. Research is ongoing to learn more about the cause of this condition.
Medical experts don’t know why some people develop corticobasal degeneration. The condition rarely affects members of the same biological family.
Corticobasal degeneration increases your risk of life-threatening complications as the disease progresses, including:
Corticobasal degeneration is different, in that you don’t receive an official diagnosis until after death. This happens because a provider or pathologist can locate and confirm brain degeneration. During your lifetime, your healthcare provider will diagnose the same condition as “corticobasal syndrome” based on your presenting symptoms.
Advertisement
To make a diagnosis, your healthcare provider will conduct a physical exam. They’ll carefully evaluate your symptoms.
As many conditions share similar symptoms, your provider will run tests to determine the cause. These may include:
In addition, your provider may order nuclear medicine scans like FDG-PET (fluorodeoxyglucose positron emission tomography) and DAT (dopamine transporter). A skin biopsy may also help make a diagnosis.
Your healthcare provider may recommend different medications for different corticobasal degeneration symptoms.
Medications for muscle symptoms may include:
Your provider may recommend one of the following cholinesterase inhibitors for memory issues:
Some medications may not work well to treat symptoms. Your provider may offer different types to see which one works best for your body.
There are also side effects associated with each type of medication. Your provider will discuss these with you and schedule regular follow-up visits to make sure the medications are working as expected and not causing any problems. Let your provider know if you notice any side effects.
Advertisement
There isn’t an available treatment option to slow the progression of symptoms or cure the condition.
The following therapies may help you manage symptoms of corticobasal syndrome:
Your provider will work with you to find management techniques that fit best with the symptoms that affect you. These may look different from person to person and can change over the course of the condition.
Call a healthcare provider if you or a loved one experiences symptoms of corticobasal degeneration like:
The prognosis (outlook) is poor for corticobasal degeneration. It’s a progressive disease, which means it’ll get worse over time. It usually happens slowly over many years. Your provider will offer treatment options to help you cope and manage symptoms. Treatment options may reduce the severity of symptoms, but it won’t stop them from progressing. As symptoms get worse, it’ll be more challenging to maintain independence and take care of yourself.
Advertisement
As the condition progresses slowly, you’ll have time to plan for your future. This doesn’t need to happen immediately after receiving a diagnosis. You should take time to think about what’s best for you. You’ll need to work with your providers and people you trust to organize your wishes and sort the care you’d like to receive.
On average, many people live between six to eight years after the first symptoms of corticobasal degeneration appear. This isn’t the case for everyone, and many people live much longer than this. Your healthcare provider is the best person to talk to about your situation because it may not match statistics.
Some symptoms of corticobasal degeneration mimic those of other conditions, such as:
It’s important to see a healthcare provider right away if you notice any symptoms because quick treatment, especially for conditions like a stroke, may be lifesaving.
It can be difficult to manage the emotions that come with receiving a diagnosis like corticobasal degeneration. You may feel anxious or scared about what the future could look like. Symptoms take years to progress, and you’re probably trying to figure out how to make the most of each moment. This can get very overwhelming quickly. You may choose to speak with a mental health professional to help you through this difficult time.
There currently isn’t a cure for the disease. However, therapies are available to help you manage symptoms and maintain independence for as long as possible. Your providers may also help you sort out your end-stage care needs, so it’s one fewer thing you have to worry about down the road.
Remember that you’re not alone through all of this. Don’t hesitate to contact your care team if you need support.
It can be unsettling when your brain stops working like it used to. Cleveland Clinic’s cognitive decline experts help you manage the symptoms and provide support.
Last reviewed on 03/12/2024.
Learn more about the Health Library and our editorial process.
