Olivopontocerebellar degeneration

Alternative names
OPCA; 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.

Causes, incidence, and risk factors
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

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

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.

Johns Hopkins patient information

Last revised: December 7, 2012
by Sharon M. Smith, M.D.

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