MedlinePlus Medical Encyclopedia: Olivopontocerebellar atrophy

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Olivopontocerebellar atrophy

Contents of this page:
Illustrations Alternative names Definition Causes, incidence, and risk factors Symptoms	Signs and tests Treatment Expectations (prognosis) Complications Calling your health care provider

Illustrations

Central nervous system

Alternative names Return to top

OPCA; Olivopontocerebellar degeneration

Definition Return to top

Olivopontocerebellar atrophy is a neurodegenerative illness that causes certain brain areas (which may include the olivary nucleus, the pons, and the cerebellum) to shrink.

Causes, incidence, and risk factors Return to top

The cause of sporadic olivopontocerebellar atrophy is not known, but the disease is progressive. This condition can be inherited or affect people without a known family history (sporadic form). Symptoms tend to start sooner in people with the familial form. This disease is slightly more common in men than in women.

Symptoms Return to top

Many symptoms are associated with olivopontocerebellar atrophy but the predominant feature is progressive ataxia (clumsiness) and difficulties with balance. There may be slurring of speech and difficulty walking. Other symptoms may include:

tremor
abnormal movements
abnormal eye movements
sexual dysfunction
bowel or bladder problems
stiffness or rigidity
spasticity
neuropathy
difficulty swallowing

Signs and tests Return to top

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 a small cerebellum or brainstem, or atrophied olives. This is helpful in making the diagnosis but lack of these findings do not necessarily rule this condition out.

Other tests may be done to rule out other diagnoses. Swallowing studies can be done to evaluate a patient's ability to swallow food and liquid safely.

Treatment Return to top

There is no specific treatment or cure for this disease. Therapy is aimed at supportive care and prevention of complications. This may include evaluation of swallowing and use of techniques to prevent choking; speech and physical therapy, and use of walking aids to help with balance and prevent falls.

Expectations (prognosis) Return to top

Prognosis is generally poor because the disease is progressive and incurable. However, different people progress at different rates and it may be years before some become significantly disabled.

Complications Return to top

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

Calling your health care provider Return to top

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

Update Date: 7/13/2004

Updated by: Joseph V. Campellone, M.D., Division of Neurology, Cooper University Hospital, Camden, NJ. Review provided by VeriMed Healthcare Network.

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