Learn about this rare condition that affects your nerve cells and may cause difficulties in speech, movement and thinking.
Corticobasal degeneration, also called corticobasal syndrome, is a rare condition in which areas of your brain shrink and your nerve cells degenerate and die over time. The disease affects the area of the brain that processes information and brain structures that control movement. This degeneration results in growing difficulty in movement on one or both sides of your body.
The condition may cause you to have poor coordination, stiffness, difficulty thinking, trouble with speech or language, or other problems.
Signs and symptoms of corticobasal degeneration (corticobasal syndrome) include:
Corticobasal degeneration progresses over six to eight years. Eventually, people with corticobasal degeneration lose the ability to walk.
Corticobasal degeneration (corticobasal syndrome) can be caused by several underlying pathologies. Most commonly, corticobasal degeneration is characterized by a buildup of tau in brain cells, which may lead to their deterioration and the symptoms of corticobasal degeneration. Half of the people who have signs and symptoms of corticobasal degeneration have corticobasal degeneration. The second most common cause of corticobasal degeneration is atypical Alzheimer's disease.
Other causes include progressive supranuclear palsy, Pick's disease and Creutzfeldt-Jakob disease.
The symptoms of corticobasal degeneration (corticobasal syndrome) progress to serious complications, such as pneumonia, blood clots in the lungs, or sepsis, a life-threatening response to an infection. Corticobasal degeneration complications ultimately lead to death.
A diagnosis of corticobasal degeneration (corticobasal syndrome) is made based on your signs and symptoms. However, your signs and symptoms could be due to another degenerative disease such as progressive supranuclear palsy, Alzheimer's disease, Pick's disease and Creutzfeldt-Jakob disease. You may need an imaging test, such as a CT scan or MRI, to rule out these other conditions. Sometimes, these tests are performed every few months to look for abnormal changes in the brain.
Researchers are looking at whether positron emission tomography (PET) and single-photon emission computerized tomography (SPECT) scans can uncover changes in the brain that are consistent with corticobasal degeneration. However, more research needs to be done in this area.
There are no treatments that help slow the progression of corticobasal degeneration (corticobasal syndrome). Your doctor may recommend medications to try to manage your symptoms.
Getting 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 problems. A dietitian may be helpful in ensuring the proper nutrition and reducing the risk of inhaling food into the lungs (aspiration).
You may start by seeing your primary care provider. 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, such as fasting before having a specific test. Make a list of:
Take a family member or friend along, if possible, to help you remember the information you're given.
For corticobasal degeneration, some basic questions to ask your doctor include:
Don't hesitate to ask other questions.
Your doctor is likely to ask you several questions, such as: