Olivopontocerebellar atrophy is a disease that causes areas deep in the brain, just above the spinal cord, to shrink.
OPCA; Olivopontocerebellar degeneration; Multiple system atrophy – cerebellar predominance; MSA-C
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).
Researchers have identified certain genes that are involved in the inherited form of this condition.
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. The average age of onset is 54 years old.
Symptoms tend to start sooner at a younger age 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
Bowel or bladder problems
Lightheadedness when standing
Muscle stiffness or rigidity
Nerve damage (neuropathy)
Sexual function problems
Signs and tests
A thorough medical and 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 affected 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.
There is no specific treatment or cure for this disease. Therapy is aimed at treating symptoms and preventing complications. This may include:
Tremor medications, such as those used to treat Parkinson's disease
Speech and physical therapy
Techniques to prevent choking
Walking aids to help with balance and prevent falls
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.
Call your health care provider if you have any symptoms of olivopontocerebellar atrophy. You will need a referral to a neurologist.
Lang A. Parkinsonism. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier; 2007:chap 433.
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.