Corticobasal syndrome (CBS) is a rare condition that primarily affects your movement. It can also gradually worsen your speech, memory, and swallowing.
CBS is a form of atypical parkinsonism, also called Parkinson’s-plus syndrome. This means that it shares certain similarities with Parkinson’s disease. However, these two conditions have different causes and disease progression.
Some of the common features between CBS and Parkinson’s are:
Corticobasal degeneration (CBD) causes CBS. CBD is the gradual damage and death of brain cells (neurons) caused by the buildup of a toxic protein called tau.
Doctors and patients often use the terms “corticobasal syndrome” and “corticobasal degeneration” interchangeably. Still, it’s best to distinguish between them. A doctor can only confirm CBD
This article will take a closer look at CBS and CBD, including causes, diagnosis, treatment, and the outlook for people with these conditions.
In CBS, like other neurodegenerative diseases, your brain deteriorates over time because of a buildup of a misfolded protein. Researchers believe that the protein that’s involved in CBS — tau — normally works to stabilize the cellular skeleton of your neurons. However, they don’t yet know its exact function.
In people with CBS, tau is
CBS typically occurs randomly and thus isn’t inherited. In very rare cases, CBS can run in families. This points to a potential genetic link.
Can I prevent corticobasal degeneration?
Currently, doctors don’t know of any ways to prevent CBS. More research is required to pinpoint the causes of this condition. Knowing its causes may also help us better understand how to prevent CBS.
Symptoms of CBS and their progression vary significantly from one person to another. The first signs and symptoms of CBS are usually related to the impaired movement of your extremities:
- muscle rigidity or stiffness
- apraxia, or an inability to make purposeful movements such as buttoning a shirt or combing your hair
- alien hand syndrome, when one of your hands acts on its own “free will”
As your disease progresses, you may also experience the following:
- loss of sensation in one or more areas of your body
- sudden spasms causing uncontrolled movements (myoclonus and dystonia)
- balance issues
- slurred speech (dysarthria)
- difficulty understanding or verbally expressing your thoughts (aphasia)
- difficulty saying what you want to say despite knowing the right words (apraxia of speech)
- difficulty swallowing (dysphagia)
In the final stages of CBS, you may experience the following:
A doctor can only confirm CBD after death during an autopsy. However, a doctor may diagnose CBS based on its typical clinical features.
Even so, diagnosis of CBS is difficult. Doctors might confuse it with similar disorders, such as Alzheimer’s disease or progressive supranuclear palsy.
There are no specific tests to diagnose CBS. Still, your doctor may order CT or MRI scans to rule out other conditions. Researchers are currently studying whether positron emission tomography (PET) or single-photon emission computerized tomography (SPECT) can help identify features of CBD.
No treatment can slow down or reverse the progression of CBS. However, certain drugs can help manage the symptoms.
- Levodopa and baclofen can help control muscle rigidity.
- Clonazepam can help with muscle spasms.
- Botox injections can alleviate pain.
- Cholinesterase inhibitors (donepezil, rivastigmine, and galantamine) can help manage memory problems.
The following therapies may also help manage CBS symptoms:
Navigating neurodegenerative diseases such as CBS is a very challenging task. It can take years to arrive at a correct diagnosis. When doctors do diagnose CBS, many people (and their families) experience a range of emotions related to grief.
Here’s what you can do to help a loved one with CBS:
- Allow them a safe space to help process their emotions.
- Learn as much as you can about CBS and CBD and how to provide care.
- Recognize that CBS affects their abilities. Have realistic expectations when it comes to their daily tasks.
- Be patient and treat them with dignity and respect.
- Explore treatment options together.
- Recognize that caregiving can affect your own physical and mental health.
- Research support groups and other organizations that can help you. For example, CurePSP is one of the largest support networks for patients with CBS and their caregivers.
- Plan for the future.
Support for caregivers
Caring for someone with a neurodegenerative disease can be very rewarding. But it also can be very exhausting — physically, emotionally, and mentally. Caregiver burnout can cause depression, agony, and anxiety.
Here’s what you can do to prevent or relieve caregiver burnout:
- Find people with whom you can safely share what you’re going through. Examples include a friend, a therapist, or a support group.
- Practice self-care.
- Take breaks.
- Develop a routine.
- Try to stop negative thoughts.
- Consider relaxation activities, such as yoga, meditation, or deep breathing.
- Keep a sense of humor.
Because there’s no treatment for CBS, it will eventually lead to serious complications. For example, difficulty swallowing can cause choking or inhaling food or liquid into your airways. This can cause pneumonia, a potentially life threatening complication.
Because of these complications, people with CBS live an average of 6 to 8 years from the onset of symptoms.
Your situation might be different, though. Make sure to ask a doctor about your specific outlook.
CBS is a rare neurodegenerative disorder that gradually worsens your physical and cognitive functions. The buildup of a toxic protein in your brain cells called tau causes CBS.
CBS is very hard to diagnose because no specific test can identify this condition. No treatment exists for this condition, but some drugs and other therapies can help you manage its symptoms. Research on new therapies and diagnostic methods is ongoing, giving hope to people with this condition and their caregivers.