Corticobasal Degeneration

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. Initial symptoms include stiffness as well as shaky, slow or clumsy movements as well as difficulty with speech or comprehension. Other symptoms are loss of movement control as well as loss of memory and intellectual ability.

The brain tissue of patients with CBD show characteristic cell changes that also appear in patients with two other disorders — frontotemporal dementia and progressive supranuclear palsy. These changes, involving a brain protein called tau, may provide researchers with some initial clues regarding the causes of corticobasal degeneration. Currently, there are no known risk factors, such as toxins or infections.

Symptoms may first appear on one side of the body, but eventually affect both sides as the disease progresses. 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 in the past decade and the UCSF Memory and Aging Center is actively involved in researching the cause and course of the disease. As a result, counseling, support and treatments of symptoms now are available.

You may need a referral from your primary care doctor. Please check with your medical benefits or health insurance representative.

See books recommended by the UCSF Memory and Aging Center.

Reviewed by health care specialists at UCSF Medical Center.
Last updated February 8, 2008