Olivopontocerebellar Atrophy

Written by Lydia Krause | Published on July 23, 2012
Medically Reviewed by Brenda B. Spriggs, MD, MPH, FACP

Olivopontocerebellar Atrophy

Olivopontocerebellar atrophy (OPA) is a neurological disorder that causes degeneration and atrophy of nerve tissue in the cerebellar part of the brain. OPA is seen in a number of multiple system atrophy disorders. These disorders differ by the site of damage within the brain.

Symptoms of OPA are like those of many multiple system atrophy disorders. The most identifiable is ataxia, which is difficulty in controlling deliberate muscle movement. Similarities in clinical presentation between neurological disorders are a major challenge in diagnosing OPA.

Neurological disorders, such as Parkinson’s disease, share symptoms of tremor and balance problems. Diagnostic imaging tests are able to assist neurologists in looking for brain damage.

There is no cure for OPA. Doctors are able to offer treatment to help patients live as long as possible. The amount of time patients live differs because brains waste away at different rates when treatment no longer helps.

Risk Factors of Olivopontocerebellar Atrophy

Risk factors depend upon the type of OPA. People with a family history of OPA are at risk for inheriting the condition. People with no family history of OPA may have the disease because of occupational and environmental factors.

Causes of Olivopontocerebellar Atrophy

OPA is known to be inherited or passed through families. The disease may also appear suddenly where there is no family history of the disease.

People with no family history may get the disease in later life because of environmental contact. Laboratory workers who are exposed to chemicals and workers with close contact with chemicals such as carbon disulfide or carbon dioxide have been shown to be at greater risk of developing OPA.

OPA is known to affect people for the first time when they are in their fifties. The fact that the disease attacks older people is thought to support the environmental contact theory. By the time people reach their fifties, they have been exposed to many chemicals found in food, plastic, makeup etc.

Symptoms of Olivopontocerebellar Atrophy

OPA causes symptoms common to many neurological disorders. The most well-known symptom is ataxia. Ataxia appears as difficulties in controlling deliberate muscle movement. Patients who have ataxia may say they are “simply being clumsy.” However, their clumsiness is actually a warning sign of their OPA.

Some of the more common symptoms of OPA may also include:

  • tremor (shaking in the hands )
  • bradykinesia (a slowness in the start of motion)
  • slurred speech
  • balance problems (such as weaving side to side when you walk)
  • muscle spasms
  • muscle stiffness and rigidity
  • nerve damage (neuropathy)
  • abnormal, involuntary eye movements

Diagnosis of Olivopontocerebellar Atrophy

The diagnosis of OPA depends on medical symptoms and signs obtained by using diagnostic testing. Neurologists are normally called when doctors see signs pointing to a neurological disorder.

Neurologists are trained to look at neurological signals and make a decision about their cause. Symptoms are not always enough, so images may be taken of the brain.

Neurological disorders that are related to OPA include Parkinson’s disease, which is a nerve disorder that causes shaking of the hands.

Imaging tests used for OPA include:

  • computed tomography (CT) scans cross-sectional high detail X-rays that show brain damage
  • magnetic resonance imaging (MRI) – imaging technique that uses radio waves to show detail images of the structure of organs

Treatment of Olivopontocerebellar Atrophy

There is no cure for OPA. Treatments are focused upon lessening symptoms and helping patients remain self-sufficient, so they are able to care for themselves.

Treatments for OPA include:

  • anti-tremor medications (Levadopa pills like those used for Parkinson’s disease)
  • walking aids (canes or walkers for balance)
  • mechanical wheelchairs that help patients as the disease gets worse because they have a high chance of falling. They can remain more independent in a power-driven wheelchair.
  • occupational therapy

Outlook of Olivopontocerebellar Atrophy

No cures are offered for OPA. Patients will lose more of their bodily skills as the disease progresses. Doctors are only able to give help to reduce symptoms. Physical and occupational therapists help patients to live without assistance from a caretaker. Sooner or later, however, not even those options will help because brain atrophy will cause death.

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Article Sources:

  • Horslen, S. P., Clayton, P. T., Harding, B. N., Gall, N. A., Keir, G., & Winchester, B. (1991). Olivopontocerebellar atrophy of neonatal onset and disialotransferrin developmental deficiency syndrome. Archives of Disease in Childhood, 66(9), 1027-1032.
  • Olivopontocerebellar atrophy. (August 27, 2010). PubmedHealth. Retrieved July 2012, from http://http:/www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001765/
  • Rodriguez, M. E., Artieda, J., Zubieta, J. L., & Obeso, J. A. (1994). Reflex Myoclonus in Olivopontocerebellar Atrophy. Journal of Neurology, Neurosurgery & Psychiatry, 57(3), 316-319.

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