What Is Corticobasal Syndrome?
Corticobasal syndrome (CBS), also referred to as corticobasal degeneration, is a progressive brain disease in which nerve cells in the brain begin to shrink (atrophy) and die. Over time, this nerve degeneration (neurodegeneration) affects the ability to control movement, think, speak, and swallow.
Corticobasal syndrome has symptoms that closely mimic those caused by Parkinson’s disease. Conditions that are not Parkinson’s, but which share symptoms are called atypical Parkinsonism.
Corticobasal syndrome is rare. The syndrome affects five out of every 100,000 people.
Corticobasal Syndrome Symptoms
Symptoms of corticobasal syndrome often begin in people aged 60 or older. The disease has never been diagnosed in anyone under 40.
Corticobasal syndrome affects each person differently. Symptoms usually start on one side of the body and spread to the other side. Symptoms worsen as more nerve cells in the brain are affected.
Symptoms of corticobasal syndrome may include:
- Balance and gait problems
- Difficulty moving one or both sides of the body (bradykinesia or slowed movements)
- Involuntary (uncontrolled) muscle movements, such as tremors (rhythmic shaking), jerks, or dystonia (muscle contractions)
- Memory problems or dementia
- Movement coordination problems
- Trouble with speaking and swallowing
- Vision problems due to abnormal eye movements
Symptoms of corticobasal syndrome also overlap with conditions other than Parkinson’s disease. These conditions include:
Causes of Corticobasal Neurodegeneration
Scientists don’t fully understand why people develop corticobasal neurodegeneration. The condition results in damage to the front and side regions (frontotemporal lobes) of the brain.
Researchers have discovered that a protein called tau clumps together in brain tissue in people with corticobasal syndrome. The abnormal buildup of tau damages brain cells, leading to the symptoms of corticobasal syndrome. Alzheimer’s disease is another example of a disorder caused by abnormal tau protein buildup (tauopathy).
Corticobasal syndrome is not considered an inherited condition, but there are some rare cases where it runs in families. Scientists are working to uncover the genes that may be responsible.
How Is Corticobasal Syndrome Diagnosed?
Corticobasal syndrome is diagnosed by a neurologist, a doctor specialized in disorders of the nervous system, including the brain. Your neurologist will ask about your symptoms and perform a comprehensive physical examination.
There is no specific test for corticobasal syndrome. Diagnosis is what’s called a diagnosis of exclusion. This means that your doctor diagnoses corticobasal syndrome only after ruling out other conditions.
To rule out other possible causes, your doctor may recommend:
- Blood tests: These tests look for signs in the blood that point to other conditions that might explain your symptoms.
- Imaging studies: These studies can rule out conditions such as stroke or brain tumors. They may also show specific regions of degeneration within the brain that commonly shrink due to corticobasal syndrome. Brain imaging studies may include:
- Computed tomography (CT) scans: These imaging tests use X-rays and computer processing to create images of the brain.
- Magnetic resonance imaging (MRI) scans: These imaging tests use pulses of a magnetic field, radio waves, and computer processing to produce complex images of the brain.
- Neuropsychological testing: This testing can help doctors better understand how the changes in your brain are affecting memory and brain function.
- Spinal tap (lumbar puncture): During this test, we insert a tiny needle into your low back to collect cerebrospinal fluid (CSF) from the space next to your spinal cord. CSF is the fluid that surrounds and nourishes the brain and spinal cord. CSF analysis can rule out autoimmune conditions, infections, or Alzheimer’s disease-related changes.
Corticobasal Syndrome Treatments
Although there is no cure for corticobasal syndrome, treatments can help manage symptoms. Your doctor will work with you on a customized treatment plan.
Your doctor may recommend:
- Medications: While medications are not always effective for corticobasal syndrome symptoms, some people find relief with drugs such as:
- Levodopa and other drugs used to treat Parkinson’s disease may reduce slowed movements and stiffness in some people with corticobasal syndrome.
- Baclofen is a muscle relaxer that targets nerves in the spinal cord to decrease muscle stiffness and spasms.
- Botulinum toxin injections temporarily weaken or paralyze a specific muscle group. These injections can decrease muscle pain and tightness around joints with contractures (permanent muscle tightness and shortening resulting in abnormal joint positions and stiffness).
- Clonazepam and other benzodiazepines may help decrease involuntary muscle spasming (myoclonus).
- Cholinesterase inhibitors, such as donepezil, galantamine, and rivastigmine can help manage memory problems and other symptoms.
- Nutritional consultation: A licensed dietitian can develop a meal plan to help you get balanced nutrition that’s easy to eat if you have swallowing problems.
- Occupational therapy: Occupational therapists can help teach new ways to perform daily tasks to help you stay independent.
- Physical therapy: Physical therapists can give specific exercises to improve balance, reduce falls, and manage muscle contractions. They can also help you learn to use walking devices, such as a walker or cane, to prevent falls.
- Speech therapy: Speech therapists can teach exercises to improve communication and coordinate swallowing to prevent aspiration.
Care for Corticobasal Syndrome at Penn Medicine
Expert neurologists at Penn Medicine’s Movement Disorders Center care for more people with corticobasal syndrome than many other centers. As the leading movement disorders center in the Philadelphia region, we have the skill and experience to diagnose rare conditions like corticobasal syndrome.
Our physician-researchers work on the leading edge of discovery about the causes of corticobasal syndrome and other movement disorders. We create custom treatment plans based on the latest research designed to bring you relief and protect your quality of life.
Make an Appointment
Please call 800-789-7366 or make an appointment.
Penn Programs & Services for Corticobasal Syndrome
Movement disorders specialists at our Movement Disorders Center offer the most advanced diagnosis and treatment for neurological movement disorders, like Parkinson’s and essential tremor.