Corticobasal Degeneration
Overview
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 brain tissue of patients with CBD show cell changes that also appear in patients with two other disorders — frontotemporal dementia and progressive supranuclear palsy. These changes involve a brain protein called tau and may provide researchers with some initial clues regarding the causes of corticobasal degeneration. Currently, there are no known causes, such as toxins or infections.
Our approach to corticobasal degeneration
As an international leader in the field of dementia disorders, we are actively researching the cause and progression of corticobasal degeneration. This work has led to better patient care through counseling, support and medications to treat symptoms such as memory loss and movement problems.
Awards & recognition
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Among the top hospitals in the nation
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Best in the West and No. 3 in the nation for neurology & neurosurgery
Signs & symptoms
The gradual loss of brain tissue and symptoms typically begin between ages 45 and 70. Initial symptoms include stiffness; shaky, slow or clumsy movements; and difficulty with speech and comprehension.
Other symptoms include:
- Balance. Difficulty walking and balancing
- Memory. Short-term memory problems, such as repeating questions or misplacing objects
- Muscle control. Difficulty controlling muscles of the face and mouth
- Speech. Progressive difficulty speaking and difficulty comprehending language
Symptoms related to muscle control usually begin on one side of the body and spread gradually to the other.
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.
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.
Some patients experience memory or behavioral problems. They 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. 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.
Significant advances in the understanding of CBD have been made and the UCSF Memory and Aging Center is actively involved in researching the cause and course of the disease.
Diagnosis
Early in the course of the disease, it may be difficult to distinguish corticobasal degeneration (CBD) from other neuro-degenerative diseases or forms of dementia. Diagnosis involves a comprehensive neurological exam, combined with one or more types of laboratory evaluations:
These scan provide images of the brain in the areas most frequently involved in the disease — the upper and front sections of the cortex or outer layer of the brain, and the basal ganglia at the base of the forebrain, which is associated with functions such as motor skills and learning.
Treatments
Currently, there are no treatments to slow the progress of corticobasal degeneration (CBD). Instead, individual symptoms are targeted with specific medications or therapy. For example, rigidity and difficulty walking may partially respond to treatments for Parkinson's disease.
Muscle contractions and and twitching may respond to muscle relaxants or anti-seizure medications.
Memory and behavior problems may respond to treatments for Alzheimer's disease or depression.
Other therapies include:
- Occupational therapy may be used to design equipment that supports the activities of daily living and maintains functional independence.
- Physical therapy is important for maintaining a patient's range of motion. This may prevent pain and contraction or the shortening of muscles as well as help maintain mobility.
- Speech therapy may be recommended to improve articulation and volume of the voice.
The UCSF Memory and Aging Center is involved in research to better understand the cause and course of the disease, which has led to counseling, support and medications to treat symptoms.
UCSF Health medical specialists have reviewed this information. It is for educational purposes only and is not intended to replace the advice of your doctor or other health care provider. We encourage you to discuss any questions or concerns you may have with your provider.
