Health Encyclopedia

Olivopontocerebellar atrophy

Definition

Olivopontocerebellar atrophy is a disease that causes certain brain areas to shrink.

Alternative Names

OPCA; Olivopontocerebellar degeneration

Causes, incidence, and risk factors

Olivopontocerebellar atrophy can be passed down through families (inherited form), or it may affect people without a known family history (sporadic form).

The cause of olivopontocerebellar atrophy in those without a history of the disease is not known. The disease slowly gets worse (is progressive).

Symptoms tend to start sooner in people with the familial form. This disease is slightly more common in men than in women.

Symptoms

Many symptoms are associated with olivopontocerebellar atrophy, but the main symptom is clumsiness (ataxia) that slowly gets worse. There may also be problems with balance, slurring of speech, and difficulty walking. Other symptoms may include:

Tremor
Abnormal movements
Abnormal eye movements
Sexual function problems
Bowel or bladder problems
Muscle stiffness or rigidity
Muscle spasms
Nerve damage
Difficulty swallowing

Signs and tests

A thorough medical and neurological examination as well as a good history of symptoms and family history are necessary to make the diagnosis. There are no specific tests for this condition.

An MRI of the brain may show changes in the size of the brain structures. This is helpful in making the diagnosis, but lack of these findings does not necessarily rule out the condition.

Other tests may be done to rule out other conditions. This may include swallowing studies to see if a person can safely swallow food and liquid.

Treatment

There is no specific treatment or cure for this disease. Therapy is aimed at treating symptoms and preventing complications. This may include techniques to prevent choking, speech and physical therapy, and walking aids to help with balance and prevent falls.

Expectations (prognosis)

The disease slowly gets worse, and there is no cure. The outlook is generally poor. However, it may be years before someone becomes significantly disabled.

Complications

Complications may include injury from falls, choking, and aspiration pneumonia (infection following inhalation of food into the lungs), and nutrition problems due to difficulty swallowing.

Calling your health care provider

Call your health care provider if you have any symptoms of olivopontocerebellar atrophy. Referral to a neurologist is necessary.

Review Date:9/10/2006
Reviewed By:Daniel Kantor, M.D., Director of the Comprehensive MS Center, Neuroscience Institute, University of Florida Health Science Center, Jacksonville, FL. Review provided by VeriMed Healthcare Network.

ADAM Quality A.D.A.M., Inc. is accredited by URAC, also known as the American Accreditation HealthCare Commission (www.urac.org). URAC's accreditation program is the first of its kind, requiring compliance with 53 standards of quality and accountability, verified by independent audit. A.D.A.M. is among the first to achieve this important distinction for online health information and services. Learn more about A.D.A.M.'s editorial process. A.D.A.M. is also a founding member of Hi-Ethics (www.hiethics.com) and subscribes to the principles of the Health on the Net Foundation (www.hon.ch).

The Agency for Health Care Administration (Agency) and this website do not claim the information on, or referred to by, this site is error free. This site may include links to websites of other government agencies or private groups. Our Agency and this website do not control such sites and are not responsible for their content. Reference to or links to any other group, product, service, or information does not mean our Agency or this website approves of that group, product, service, or information.

Additionally, while health information provided through this website may be a valuable resource for the public, it is not designed to offer medical advice. Talk with your doctor about medical care questions you may have.