Saanichton, BC

Dr. Miguel A. Lipka

Corticobasal Degeneration

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Corticobasal degeneration (CBD) is a rare neurological disease associated with progressive brain degeneration. The disease, also known as corticobasal ganglionic degeneration, leads to the loss of brain tissue in the cortex, or outer layer of the brain, especially the area in the upper, front section of the brain.

The gradual loss of brain tissue and symptoms typically begin between ages 45 and 70. Women are affected more often than men.

Currently, there are no known risk factors, such as toxins or infections.

Initial symptoms include stiffness as well as shaky, slow or clumsy movements as well as difficulty with speech or comprehension. Other early symptoms may include:

  • Balance -- Difficulty walking and balancing
  • Memory -- Short-term memory problems, such as repeating questions or misplacing objects
  • Muscle Control -- Difficulty controlling the muscles of the face and mouth
  • Speech -- Progressive difficulty speaking or articulating as well as difficulty comprehending language

Other symptoms are loss of movement control as well as loss of memory and intellectual ability. Symptoms may first appear on one side of the body, but eventually affect both sides as the disease progresses. There may be difficulties in completing specific tasks, such as opening a door or brushing one's teeth or using tools such as a can opener. When a leg is affected, a patient may have problems with complex movements such as dancing. As the disease progresses, a patient may begin to trip and fall. A patient also may experience uncontrollable movement of an arm or leg.

Some patients experience memory or behavioral problems. Patients may have difficulty with expression of language, such as finding the right word or name. Reading, writing and simple mathematical calculations also may be impaired. In addition, personality changes, inappropriate behavior and repetitive and compulsive activities similar to those in frontotemporal dementia are common in CBD.

A person with the disease often becomes immobile five years after symptoms emerge. Within 10 years, pneumonia or other bacterial infections may lead to life-threatening complications.

In the past, patients have been diagnosed on the basis of movement problems that appear similar to Parkinson's disease. CBD patients also experience many symptoms that are not characteristic of Parkinson's and it is sometimes referred to as a "Parkinson's-plus" syndrome.

Because the exact cause of CBD is unknown, no formal treatment for the disease exists. Instead, treatments focus on minimizing the appearance or effect of the symptoms resulting from CBD. The most easily treatable symptom of CBD is parkinsonism, and the most common form of treatment for this symptom is the application of dopaminergic drugs. However, only moderate improvement is seen and the relief from the symptom is generally not long-lasting.

Palliative therapies, including the implementation of wheelchairs, speech therapy, and feeding techniques, are often used to alleviate many of the symptoms that show no improvement with drug administration.