Corticobasal degeneration is a rare, progressive neurological disorder that causes loss of independence and the inability to speak, remember, move, think, and reason over time. Although there is no cure for corticobasal degeneration, treatments may help manage symptoms and complications.
At UPMC, our specialists collaborate to coordinate care and develop the best treatment plans for people diagnosed with corticobasal degeneration, which may offer opportunities to participate in research, including clinical trials for drug therapy.
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What Is Corticobasal Degeneration?
Corticobasal degeneration is a rare, progressive neurological disorder that causes loss of independence and the inability to speak, remember, move, think, and reason over time. Although there is no cure for corticobasal degeneration, treatments may help manage symptoms and complications.
Is corticobasal degeneration the same as dementia?
Corticobasal degeneration is a neurological disorder that may cause symptoms of dementia.
What is the difference between Parkinson’s disease and corticobasal degeneration?
Corticobasal degeneration and Parkinson’s disease are neurological disorders that progressively worsen and affect movement. However, the two disorders are distinct conditions caused by different proteins that damage nerve cells in the brain.
Corticobasal degeneration typically causes symptoms that affect movement on one side of the body, such as muscle jerks, stiffness, and slowed movement, and may not respond to medications used to treat Parkinson’s disease. Although Parkinson’s disease causes similar symptoms, it typically affects both sides of the body and responds well to a common medication called levodopa.
How common is corticobasal degeneration?
Corticobasal degeneration is rare. The National Organization for Rare Disorders (NORD) estimates that the disorder affects about five out of 100,000 people, with approximately one new diagnosis per 100,000 people each year.
What causes corticobasal degeneration?
Corticobasal degeneration results from damage and death of brain cells, mainly in two areas of the brain — the cerebral cortex and the basal ganglia. These areas control memory, thinking, and movement.
Research indicates that abnormal clusters of a protein called tau may cause damage and death of brain cells, leading to corticobasal degeneration symptoms. Conditions such as corticobasal degeneration and progressive supranuclear palsy (PSP), which may be associated with abnormal tau buildup in the brain, are often called tauopathies.
Corticobasal degeneration risk factors
Researchers aren’t sure what increases the risk of corticobasal degeneration. However, some studies suggest you may be at higher risk if you have a gene mutation that increases your likelihood of developing the abnormal clusters of tauopathies.
Complications of corticobasal degeneration
As corticobasal degeneration worsens over time, it may cause loss of independence and other complications, including:
- Blood clots.
- Infections.
- Injury resulting from falls or other accidents.
- Pneumonia.
- Pulmonary embolism.
- Sepsis.
How can I prevent corticobasal degeneration?
There is nothing you can do to prevent corticobasal degeneration.
What Are the Signs and Symptoms of Corticobasal Degeneration?
Symptoms of corticobasal degeneration typically appear between the ages of 50 and 70 and get progressively worse over time. However, symptoms can sometimes start as early as age 40.
Depending on what part of the brain is affected and the extent of the damage, corticobasal degeneration may cause problems with:
- Memory
- Movement
- Speech
- Swallowing
- Vision
Although corticobasal degeneration affects each person differently and progresses at different rates, common symptoms include:
- Changes in behavior or mood.
- Dementia symptoms that affect your ability to think, reason, and remember.
- Difficulty walking.
- Loss of balance or coordination.
- Loss of control of one part of the body, such as an arm or hand.
- Loss of feeling in one part of your body.
- Muscle tremors, spasms, twitches, or stiffness.
- Problems with fine motor skills, such as writing or tying shoes.
- Slower-than-normal movement.
- Speech, swallowing, and communication problems.
What are the first symptoms of corticobasal degeneration?
One of the initial symptoms of corticobasal degeneration may be a loss of coordination or muscle control that impacts only one part or side of your body. This might initially appear as clumsiness, making it hard to hold objects or walk. You may also observe speech issues that make it difficult to remember words or participate in conversations.
When should I see a doctor about my corticobasal degeneration symptoms?
If you are experiencing symptoms of corticobasal degeneration, you should schedule an appointment with your doctor. Although there is no cure for the condition, getting early treatment for your symptoms may improve your quality of life.
How Do You Diagnose Corticobasal Degeneration?
An official corticobasal degeneration diagnosis can’t be made until after death, when the location and extent of brain degeneration can be confirmed. However, based on your symptoms, you may be diagnosed with corticobasal syndrome during your lifetime.
Several conditions can cause symptoms similar to those of corticobasal degeneration, in which case it may be referred to as corticobasal syndrome, so it may take time for your doctors to diagnose the condition. You may need to see several different medical providers and have different types of tests to rule out other conditions before your doctor can make a diagnosis.
What to expect during your visit
During your first visit, your doctor will:
- Ask about your symptoms.
- Order tests.
- Perform a physical and neurological exam.
- Review the medications you are taking.
- Review your personal and family medical history.
Tests to diagnose corticobasal syndrome
Your doctor may order tests to help diagnose corticobasal syndrome, including:
- Blood and urine tests — Performed to rule out other conditions.
- CT scan — Creates images of the brain and other structures to check for damaged or abnormal areas.
- Electromyogram (EMG) — Uses electrical impulses to test how well your muscles are working.
- Nerve conduction study — Uses electrical impulses to test how well your nerves are transmitting electrical signals to your muscles.
- Neuropsychological testing — Assesses your ability to think, reason, and remember.
- Nuclear medicine tests — Include tests such as fluorodeoxyglucose positron emission tomography (FDG-PET) and dopamine transporter (DAT), which use radioactive materials to diagnose brain abnormalities.
- Magnetic resonance imaging (MRI) — Creates images of your brain to look for damaged or abnormal areas.
- Skin biopsy — Involves taking a small skin sample to check for abnormal proteins associated with neurodegenerative disorders.
- Swallowing studies — Determine how well the muscles involved in swallowing are working.
Corticobasal degeneration prognosis
There is no cure for corticobasal degeneration, and symptoms get worse over time. In most cases, symptoms progress slowly over many years, but the rate of progression varies from person to person. Although treatment may help manage symptoms and preserve your quality of life, you may eventually experience loss of independence and challenges with thinking and memory.
What is the life expectancy of someone with corticobasal degeneration?
According to the National Institute of Neurological Disorders and Stroke, most people with corticobasal degeneration survive for six to eight years after symptoms begin. However, some people may survive for shorter or longer periods of time.
How Do You Treat Corticobasal Degeneration?
There is no cure for corticobasal degeneration, and there is no treatment that will slow or stop symptom progression. The goals of treatment are to manage symptoms and improve quality of life.
Treatment options may include:
Medications
Your doctor may prescribe medications to manage various symptoms, including memory problems and physical issues such as muscle stiffness, jerks, and movement difficulties.
Physical, occupational, and speech therapy
Physical therapy may help people with corticobasal degeneration maintain muscle strength and function. Occupational therapy experts can provide strategies to assist with performing everyday activities, as well as braces, wheelchairs, and other devices to improve mobility and safety. Speech therapy can help with voice, speech, communication, or swallowing problems.
Why Choose UPMC for Corticobasal Degeneration Care?
When you choose UPMC for corticobasal degeneration care, you will receive:
- Leading-edge neurological care — Our team specializes in providing evidence-based care for all types of neurodegenerative disorders, including treatment to manage symptoms and optimize quality of life.
- Access to advanced clinical trials — As a leader in neurological research, UPMC offers clinical trials that aren’t available at other centers. That means our patients may receive access to promising therapies years before they become widely available.
- Multidisciplinary treatment provided by a team of experts — Our neurodegenerative disease experts are part of a team that includes neurologists, nurses, physical therapists, occupational therapists, social workers, clinical psychologists, speech and swallowing therapists, and dietitians.
