Health Encyclopedia

Olivopontocerebellar atrophy

Definition

Olivopontocerebellar atrophy is a disease that causes areas deep in the brain, just above the spinal cord, 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).

This disease is slightly more common in men than in women.

Symptoms

Symptoms tend to start sooner in people with the inherited form. 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:

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

Signs and tests

A thorough medical and brain/nervous system (neurological) examination, as well as a symptom and family history are needed 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, especially as the disease gets worse. This is helpful in making the diagnosis, but 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:

Speech and physical therapy
Techniques to prevent choking
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

Choking
Infection from inhaling food into the lungs (aspiration pneumonia)
Injury from falls
Nutrition problems due to difficulty swallowing

Calling your health care provider

Call your health care provider if you have any symptoms of olivopontocerebellar atrophy. You will need a referral to a neurologist.

Review Date:9/25/2008
Reviewed By:Daniel B. Hoch, MD, PhD, Assistant Professor of Neurology, Harvard Medical School, Department of Neurology, Massachusetts General Hospital. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

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