Corticobasal Degeneration

Information & Advice for Caregivers

Corticobasal Degeneration is rare type of dementia that is both progressive and terminal. It presents with a unique set of characteristics and challenges in the home setting.

Several different areas of the brain degenerate is corticobasal degeneration, also known as CBD. The most common areas include the cerebral cortex and the basal ganglia.

As the disease progress, cerebral atrophy, or shrinking of the brain, occurs. There are also abnormal levels of tau proteins present in the brain cells. Slightly more women seem to be affected by this disease than men.

This type of dementia typically begins around the age of 60 and progresses gradually. The disease is terminal and most people live around 8 years after diagnosis.

Cause and Risk Factors

Scientists have discovered a connection between the tau gene and corticobasal degeneration. However, at this time there is not a genetic test that can be run to determine if a person is at risk and there is no indication in current studies that this type of dementia is hereditary. It seem to appear at random.

There are no apparent risk factors associated with the development of CBD. Diagnosis is made by evaluating symptoms and progression of the disease. In later stages, MRI and other brain scans may be able to pick up on the cerebral atrophy.

Symptoms of Corticobasal Degeneration

The symptoms of CBD usually appear around the age of 60 and often are easily confused with both Parkinson's dementia and Primary Progressive Aphasia. Typically the presenting signs are motor difficulties.

Although each individual is unique, the symptoms that are characteristic of corticobasal degeneration include:

  • Parkinson-like symptoms: Muscle rigidity, tremors, slow movements and poor balance
  • Difficulty swallowing
  • Alien Hand Syndrome: An inability to control movements of the hand and a feeling that the extremity does not belong to oneself. Approximately 60% of CBD patients experience this syndrome.
  • Jerking movements
  • Sensory loss
  • Mild Cognitive Impairment including memory loss and difficulty with executive function (planning, reasoning, judgment etc)

Home Care Challenges with CBD

The person suffering from corticobasal degeneration is at risk for injury associated with falls and aspiration, swallowing liquid into the lungs, often resulting in pneumonia. Treatment at home should focus on keeping the person as healthy as possible as well as preventing injuries associated with this disease.

    Preventing Falls

  • Chair alarms: These alarms can help alert you if your loved one is attempting to stand and walk without assistance
  • Bed Alarms: Used while someone is in bed, these alarms utilize either pressure sensitive pads or a magnetic tab
  • Wheelchair seat belt: Used appropriately, these devices can help prevent someone from standing up out of a wheelchair without assistance.
  • Mobility Aides: Including canes, walkers, reachers and wheelchairs
  • Monitor for injury: People with CBD have frequent issues with bumps, bruises, cuts, scrapes and skin tears. Monitor the skin frequently and notify your physician if you believe any skin issue is becoming infected.

    Preventing Aspiration

  • Moist foods: Gravies and sauces can help make foods easier to swallow
  • Thickened liquids: Powders can be added to any liquid to make it thicker and easier to swallow
  • Adaptive feeding devices: There are many adaptive devices that can be used to make eating safe and allow the person independence for longer
  • Feed slowly: A person with a swallowing disorder should never be rushed to eat. Each bite of solid food should be followed by a sip of thickened liquids.
  • Monitor for infection: Always be aware of breathing patterns, coughing, weight loss or temperature which can indicate infection.

A point to remember is that you may not be aware at the time that the person you are caring for is aspirating. Sometimes it occurs in such small amounts that it does not trigger coughing. When aspiration occurs in a situation like this, it is referred to as silent aspiration.

Caring for someone at home with corticobasal degeneration is certainly feasible. Therapies, including speech therapy, occupational therapy and physical therapy, can help keep your loved one as strong as possible for as long as possible.

Keep both you and your loved one safe from potential injury at all times. Contact your insurance company to see if home assistance is covered to help you with at least some of the physical burden of caring for your loved one.

At this point, there is no cure and no treatment that slows the progression of the disease process. Medications to manage specific symptoms can be beneficial to some people. Research is ongoing to identify both a cause and a course of treatment.

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