Olivopontocerebellar Atrophy

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What is olivopontocerebellar atrophy?

Olivopontocerebellar atrophy (OPCA) describes a group of rare neurological disorders that cause loss of coordination, muscle control, and balance. It involves the progressive (continuing to worsen) degeneration of nerve cells in the cerebellum (involved in coordinating intended movement and balance), the pons (a part of the brain stem), and other parts of the brain that are involved with movement and balance.

OCPA is associated with two distinct groups of disorders:

  1. A hereditary neurological disorder known as spinocerebellar ataxia (SCA)
  2. The disorder multiple system atrophy, which mostly appears sporadically (on its own)

OPCA may also be found in the brains of individuals with prion disorders and inherited metabolic diseases. OCPA affects both men and women. Symptoms generally begin in mid-adulthood and slowly progress, with death occurring about 20 years after onset.

Symptoms may include:

  • Inability to coordinate voluntary muscle movements (ataxia)
  • Trouble with balance when walking
  • Speech or swallowing problems
  • Vision problems including impaired eye muscle control and blurred or double vision
  • Muscle wasting (atrophy)
  • Unplanned muscle twitching (myoclonus)
  • Neuropathy (pain or a numbness or tingling sensation)
  • Tremor of the hand or arm

People with olivopontocerebellar ataxia also may have problems with short-term memory, problem solving, and overall decline in intellectual function (dementia).

Diagnosing OPCA involves a thorough medical exam, involving an evaluation of the person's family medical history for a similar condition, brain imaging studies, laboratory tests, genetic testing, and the presence of symptoms.

Currently there is no cure for OPCA. Different medications can be prescribed to manage symptoms such as ataxia, tremors, rigidity, stiffness, spasms, sleep disorders, and depression. Physical therapy may be beneficial in developing a regular exercise and stretching routine, as well as obtaining devices to assist in completing daily tasks. 

Learn About Clinical Trials
Clinical trials are studies that allow us to learn more about disorders and improve care. They can help connect patients with new and upcoming treatment options.

How can I or my loved one help improve care for people with olivopontocerebellar atrophy?

Consider participating in a clinical trial so clinicians and scientists can learn more about OPCA and related disorders. Clinical studies use human volunteers to help researchers learn more about a disorder and perhaps find better ways to safely detect, treat, or prevent disease.

All types of volunteers are needed—those who are healthy or may have an illness or disease—of all different ages, sexes, races, and ethnicities to ensure that study results apply to as many people as possible, and that treatments will be safe and effective for everyone who will use them.

For information about participating in clinical research visit NIH Clinical Research Trials and You. Learn about clinical trials for people with olivopontocerebellar degeneration at ClinicalTrials.gov, which provides information about ongoing and completed federal and privately supported clinical trials.

Where can I find more information about olivopontocerebellar atrophy?

The following resources may help:

National Organization for Rare Disorders (NORD)
Phone: 203-744-0100 or 800-999-6673; 844-259-7178 Spanish

Genetic and Rare Diseases (GARD) Information Center