Corticobasal Degeneration

What is Corticobasal Degeneration?

Corticobasal degeneration is a rare, progressive disease that affects the neurological system. This disorder stands out from other neurological disorders because patients experience cell loss and tissue deterioration in certain areas of the brain. The symptoms of this disorder are difficult to decipher from other neurological disorders.

The cause of this disease is unknown and still has scientists confused. The signs and symptoms are usually caused by other disorders that cause degeneration of the nervous system. This disorder is usually diagnosed based on signs and symptoms, which must meet a certain criteria and severity.

While doctors diagnose this disorder based on signs and symptoms, an accurate diagnosis cannot be made except on autopsy after the patient has passed away.

What are the Symptoms of Corticobasal Degeneration?

There are many factors that doctors must take into consideration when they are diagnosing corticobasal degeneration. These factors include the symptoms, progression, presentation and severity, which can differ greatly from patient to patient. It is important for patients to have a close, open relationship with their doctor to ensure they get the best care. Even if they think a symptom is unrelated, their doctor should know about it.

Symptoms include

  • Tightening and stiffening of the muscles, which gets worse over time
  • Inability or difficulty in making voluntary movements
  • Difficulty directing movements appropriately
  • Difficulty coordinating movements
  • Development of involuntary movements with no ability to stop them
  • Appearance of clumsy movement
  • Slight tremors during times of activity or muscle tension
  • Extreme, exaggerated slowness when trying to move affected limbs
  • Involuntary muscle spasms
  • Inability to voluntarily move limbs
  • Movement may become painful

Symptoms typically affect one side of the body more than the other side. Typically, individuals experience symptoms in the arms long before the legs are affected. However, in rare cases, the legs are affected by the disease first.

Corticobasal Degeneration Causes

Corticobasal degeneration is a condition that is not well understood by modern medicine. What is currently known about the cause of this condition is that a protein called tau is involved in its development. This protein is in the brain naturally, and it is found in larger amounts in patients who have corticobasal degeneration. When this protein becomes too abundant in the patient’s brain cells, the result is the deterioration of cells. Over time, this deterioration leads to the symptoms that corticobasal degeneration is known for. There is no known cause for the accumulation of this protein in brain cells and no way to predict who will develop it.

There is currently no evidence that being exposed to any infections or toxic substances will affect tau or cause it to become too abundant in the brain. Corticobasal degeneration appears to be caused by chance rather than by an inherited predisposition for the condition. However, there have been rare cases in which multiple cases have been reported in families, keeping open the possibility of an inherited condition which is not yet understood. In some cases, people with this condition have been found to have a specific variant of the tau gene. However, not all people with this variant will develop the condition. This has made the gene’s role in corticobasal degeneration uncertain.

How is Corticobasal Degeneration Treated?

Unfortunately, there is no standard treatment for corticobasal degeneration. Treatment is mostly focused on the symptoms of the disease. The treatment usually works in the early stages of the disorder, but over time the symptoms may prove to be resistant to treatment.

Treatment includes

Many patients are treated with medications that are used to treat Parkinson’s disease. Seizure medications are typically used to treat muscle spasms and myoclonic jerks that are associated with the disease.

Some patients find physical therapy beneficial to maintaining muscle control and speech throughout the earlier stages of the disease.

Corticobasal Degeneration Prevention

Because there is no known cause of this condition, there is currently no way to prevent developing it. For those who have this condition in their family, there is a small chance of developing it. This makes it important for those with a familial condition to be screened for it if they begin to develop the symptoms of corticobasal degeneration. Shaking, muscle weakness and tremors can indicate that testing should be done to rule out this condition.

Resources
Last Reviewed:
September 19, 2016
Last Updated:
December 08, 2017
Content Source: