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Olivopontocerebellar atrophy |
| Definition: |
| Olivopontocerebellar atrophy is a neurodegenerative illness that causes certain brain areas (which may include the olivary nucleus, the pons, and the cerebellum) to shrink.
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| Alternative Names: |
| OPCA; Olivopontocerebellar degeneration
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| Causes, incidence, and risk factors: |
| This condition can be inherited but it most commonly affects people without a known family history (sporadic form). Sporadic cases tend to affect people in their 50s while familial cases usually start earlier. The cause of sporadic olivopontocerebellar atrophy is not known, but the disease is progressive. |
| Symptoms: |
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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:
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| Signs and tests: |
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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: |
| 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): |
| 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: |
| 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: |
| Call your health care provider if you have any symptoms of olivopontocerebellar atrophy. Referral to a neurologist is necessary. |
Reviewed By: Elaine T. Kiriakopoulos, M.D., MSc, Department of Neurology, Beth Israel Deaconess Medical Center, Harvard University, Boston, MA. Review provided by VeriMed Healthcare Network.
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