Olivopontocerebellar atrophy is a disease that causes areas deep in the brain, just above the spinal cord, to shrink.
Causes of Olivopontocerebellar atrophy
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.
Olivopontocerebellar atrophy Symptoms
Symptoms tend to start 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
- Abnormal movements
- Bowel or bladder problems
- Difficulty swallowing
- Light-headedness when standing
- Muscle spasms
- Muscle stiffness or rigidity
- Nerve damage (neuropathy)
- Sexual function problems
Tests and Exams
A thorough medical and neurological examination, as well as a symptom and family history, are needed to make the diagnosis.
There are genetic tests to look for the causes of some forms of the disorder. However, no specific test is available in many cases. An MRI of the brain may show changes in the size of affected brain structures, especially as the disease gets worse. But it is possible to have the disorder and have a normal MRI.
Other tests may be done to rule out other conditions. These may include swallowing studies to see if a person can safely swallow food and liquid.
Treatment of Olivopontocerebellar atrophy
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.
- Infection from inhaling food into the lungs (aspiration pneumonia)
- Injury from falls
- Nutrition problems due to difficulty swallowing
When to Contact a Health Professional
Call your health care provider if you have any symptoms of olivopontocerebellar atrophy. You will need a referral to a neurologist.
Apostolova LG, DeKosky ST, Cummings JL. Dementias. In: Daroff RB, Fenichel GM, Jankovic J, Mazziotta JC, eds. Bradley's Neurology in Clinical Practice. 6th ed. Philadelphia, PA: Elsevier Saunders; 2012:chap 66.
Lang AE. Parkinsonism. In: Goldman L, Schafer AI, eds. Goldman's Cecil Medicine. 24th ed. Philadelphia, PA: Elsevier Saunders; 2011:chap 416.
|Review Date: 7/27/2014
Reviewed By: Joseph V. Campellone, MD, Department of Neurology, Cooper University Hospital, Camden, NJ. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.