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sporadic

What is Sporadic Olivopontocerebellar Atrophy?

Olivopontocerebellar atrophy (OPCA) is almost certainly not a single disease, but a group of diseases. In this brochure, we discuss OPCA that has occurred "sporadically", which means that no one else in the family has ever had the same disorder. In the brochure "Hereditary Olivopontocerebellar Atrophy", we describe forms of ataxia that are known to run in families.

Many people do not receive a diagnosis of OPCA until they have seen many different physicians. Physicians also use different terms when they diagnose OPCA. Here is a list of some of the terms, or diagnoses, that physicians may use:
  • olivopontocerebellar atrophy or degeneration
  • olivocerebellar atrophy or degeneration
  • Multisystem atrophy
  • ataxia
  • Marie's ataxia
  • Holmes ataxia
  • Menzel's ataxia
  • ataxia with Parkinsonism, autonomic neuropathy,
  • corticospinal features or dementia
  • spastic ataxia

In addition many patients with sporadic OPCA may have been diagnosed as having Parkinson's disease, multiple sclerosis, or other neurologic conditions before the diagnosis of OPCA becomes clear.

What are the symptoms of OPCA?

The symptoms of OPCA are different from one person to the next, which is one reason why the diagnosis may be hard to make. Most patients with sporadic OPCA develop difficulty with balance and coordination of the legs and arms (ataxia), and many develop slurred speech (dysarthria). If the ataxia becomes severe, it can interfere with the person's ability to work at his job, to walk independently, to write or feed himself.

Many patients with sporadic OPCA develop symptoms in addition to ataxia; occasionally these symptoms come on earlier or are most obvious than the ataxia. These symptoms may include weakness, stiffness of the muscles, or muscle spasms; numbness or tingling of the hands or feet; tremor (shaking) of the hand or arm, slowness of movements, and decreased movements; loss of thinking or memory skills; and difficulty controlling the bladder or bowels, or feeling faint when standing up. Some patients with sporadic OPCA have troubles with sleep; others note fatigue as a troublesome symptom.
The symptoms of OPCA usually come on in mid-adult life and progress slowly over a course of many years.

What causes OPCA?

There are probably many different causes for OPCA, because OPCA is really a number of different disorders. When a person with symptoms suggesting OPCA sees a physician, the physician may do a large number of tests to look for medical and neurologic diseases that we already know about that can cause these symptoms. Among the medical and neurologic diseases that can cause or be associated with ataxia and/or some of the other symptoms described above are:
  • cancer (particularly lung and ovarian)
  • chronic alcohol use or abuse
  • Vitamin B12, thiamine, or Vitamin E deficiency
  • hypothyroidism
  • certain drugs, in particular drugs to treat epilepsy
  • strokes, tumors, or cysts in the cerebellum or brainstem
  • hydrocephalus
  • residual effects of encephalitis or suffocation
  • exposure to certain toxins, such as heavy metals (lead, thallium)
  • a number of rare "enzyme" or "metabolic" disorders
  • multiple sclerosis
  • peripheral neuropathy (various types)

      You can see why it may take a long time and a number of tests before all these possibilities are considered and a diagnosis of OPCA is made. Sometimes the diagnosis may never be entirely certain. There is no single test that proves that a person has sporadic OPCA; rather, the diagnosis is usually made in a patient with a appropriate symptoms in whom other conditions have been "ruled out."

      Commonly, a person who has OPCA will undergo a number of blood tests, imaging of the brain and/or spinal cord by CT (computerized tomography) or MRI (magnetic resonance imaging), and (depending on the specific symptoms) evaluation for cancer, multiple sclerosis, or peripheral neuropathy before a diagnosis is made.

      What is the treatment for sporadic OPCA?

      There is no specific treatment for sporadic OPCA. If an underlying cause for ataxia is identified, such as a vitamin deficiency, hypothyroidism, or alcoholism, then treatment of the underlying problem may prevent the symptoms from worsening. Otherwise, treatments are directed at the specific symptoms. For instance, stiffness or spasms can often be improved with medications, as can sleep disorders, depression, and tremor. A physical therapist may be very helpful in establishing a routine of exercise, stretching, and obtaining devices or appliances to assist in walking and otber daily activities.

      As symptoms progress, each individual with OPCA may have needs that are special or unique to that person. Some patients need devices to assist with eating or special diets to help avoid choking; others may need adaptations to their residence to accommodate wheelchairs or walkers. Some individuals need assistance with bathing, dressing, or other daily affairs; often these needs can be met by family, friends, or aides in the home setting.

      Scientific and medical research is ongoing in the United States and many other countries, to find out the cause or causes of OPCA, and to devise better treatments for it. The National Ataxia Foundation is proud to fund a number of these research project each year, through its "Million Dollars for Research" Campaign and Annual Research Drive. Research breakthroughs are reported as soon as possible in the National Ataxia Foundation's newsletter "Generations", and at the Annual Membership meeting held each year in February or March.

      Can sporadic OPCA be passed from one person to another?

      There is no evidence to suggest that any form of sporadic OPCA is contagious in any way, so there is no need to avoid a person who has OPCA for fear of "catching it". Many people with ataxia or OPCA have a hereditary form, which can be passed on genetically from parent to child. It is easy to tell that OPCA IS hereditary if your parent, or brother, or daughter also has OPCA. It is much more difficult to be certain that OPCA is NOT hereditary if you are the only person in the family ever to have had OPCA. We recommend that persons with sporadic OPCA discuss genetic questions carefully with the physician or genetic counselor who knows them best, because the answers may be different for different individuals or families.

      What should I do if I have sporadic OPCA?

      Persons with sporadic OPCA need to do a number of things. First they should be as certain of their diagnosis as possible, and as comfortable with their physician as possible. It is best when faced with a chronic neurologic condition to have a neurologist who knows you well, and with whom you feel comfortable discussing new problems as they arise.

      It is important to learn about the disease, so that you know what to expect now and in the future. We recommend that our patients and families plan ahead for the future, so that medical or financial crises can be avoided.

      It is easy to become angry or frustrated or even depressed when faced with a diagnosis like sporadic OPCA. It is better, however, to face the diagnosis with a good attitude, in good general physical, medical, and mental condition, and to make the time ahead valuable and fun. Patients with OPCA can do all sorts of things; often it just takes a little extra time or planning.

      The National Ataxia Foundation publishes a quarterly newsletter, "Generations", which includes a lot of information from and about people with differnet kinds of ataxia and OPCA. Local NAF Chapters and support groups are available in a number of communities around the country, and for those who do not live near a support group, a computer network (bulletin board) for ataxia has been established. NAF also sponsors a "Pen-Pal" program that many have found quite helpful as it gives you the opportunty to correspond with others with ataxia to share common concerns. We encourage all persons with sporadic OPCA to become knowledgeable, active and involved. If we work together, not only will we improve our own lives, but also those of others with OPCA, both now and in the future.

      Please note: this information is Copyright 1993 by the National Ataxia Foundation, Inc. All rights reserved. - Find their site: http://www.ataxia.org