Corticobasal syndrome (CCS): causes, symptoms, treatment

Corticobasal syndrome (CBS) is a rare condition that primarily affects your movements. 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 some similarities with Parkinson’s disease. However, these two conditions have different causes and disease progression.

Some common characteristics between CBS and Parkinson’s disease 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. However, it is better to distinguish them. A doctor can only confirm CBD after death during an autopsy, when they can diagnose CBS based on typical symptoms.

This article will take a closer look at CBS and CBD, including the causes, diagnosis, treatment, and outlook for people with these conditions.

In CBS, like other neurodegenerative diseases, your brain deteriorates over time due to a buildup of a misfolded protein. Researchers believe the protein involved in CBS – tau – normally works to stabilize the cell skeleton of your neurons. However, they do not yet know its exact function.

In people with CBS, tau is badly folded, causing it to build up, eventually damaging and killing your neurons. Why tau is misfolded in some people is unclear. It could be linked to environmental changes and aging.

CBS usually occurs randomly and is therefore not inherited. In very rare cases, CBS can run in families. This indicates a potential genetic link.

CBS symptoms and their progression vary widely from person to person. The first signs and symptoms of CTS are usually Related altered movement of your extremities:

  • muscle stiffness or stiffness
  • bradykinesia
  • tremors
  • apraxia or inability to make deliberate movements such as buttoning a shirt or combing 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 feeling in one or more areas of your body
  • sudden spasms leading to uncontrolled movements (myoclonus and dystonia)
  • balance problems
  • 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 CBS Final Stages, you may encounter the following:

A doctor can only confirm CBD after death during an autopsy. However, a doctor can diagnose CBS based on its typical clinical features.

Even so, the 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 scans or MRIs to rule out other conditions. Researchers are currently studying whether positron emission tomography (PET) or single photon emission tomography (SPECT) can help identify characteristics of CBD.

No treatment can slow or reverse the progression of CTS. However, certain medications can help manage symptoms.

  • Levodopa and baclofen can help control muscle stiffness.
  • Clonazepam can relieve muscle spasms.
  • Botox injections can relieve pain.
  • Cholinesterase inhibitors (donepezil, rivastigmine, and galantamine) can help manage memory problems.

The following therapies may also help manage the symptoms of CBS:

Navigating neurodegenerative diseases such as CTS is a very difficult task. It can take years to arrive at a correct diagnosis. When doctors diagnose CTS, many people (and their families) experience a range of grief-related emotions.

Here’s what you can do to help a loved one with CTS:

  • Give them a safe space to help them process their emotions.
  • Learn as much as possible about CBS and CBD and how to provide care.
  • Acknowledge that CBS affects their abilities. Have realistic expectations for 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.
  • Look for support groups and other organizations that can help you. For instance, CurePSP is one of the largest support networks for CTS patients and their caregivers.
  • Plan for the future.

Because there is no treatment for CBS, it will eventually lead to serious complications. For example, difficulty swallowing can cause choking or inhaling food or liquids into the airways. This can cause pneumonia, a life-threatening complication.

Because of these complications, people with CTS live on average 6 to 8 years old from the onset of symptoms.

However, your situation may be different. Be sure to ask a doctor about your specific outlook.

CBS is a rare neurodegenerative disease that progressively deteriorates your physical and cognitive functions. The buildup of a toxic protein in your brain cells called tau causes CBS.

CBS is very difficult to diagnose because no specific test can identify this condition. There is no treatment for this condition, but certain medications and other therapies can help you manage its symptoms. Research into new therapies and diagnostic methods is ongoing, giving hope to people with this condition and their caregivers.

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