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Alternative Names Return to topOPCA; Olivopontocerebellar degeneration
Definition Return to top
Olivopontocerebellar atrophy is a disease that causes areas deep in the brain, just above the spinal cord, to shrink.
Causes Return to top
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 Return to top
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:
Exams and Tests Return to top
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 Return to top
There is no specific treatment or cure for this disease. Therapy is aimed at treating symptoms and preventing complications. This may include:
Outlook (Prognosis) Return to top
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.
Possible Complications Return to top
When to Contact a Medical Professional Return to top
Call your health care provider if you have any symptoms of olivopontocerebellar atrophy. You will need a referral to a neurologist.Update Date: 9/25/2008 Updated 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.