ATAXIA What is ataxia? Ataxia is a symptom, not a specific disease or diagnosis. Ataxia means clumsiness, or loss of coordination. Ataxia may affect the fingers and hands, the arms or legs, the body, speech or eye movements. This loss of coordination may be caused by a number of different medical or neurologic conditions; for this reason, it is important that a person with ataxia seek medical attention to determine the underlying cause of the symptom and to get the appropriate treatment. What causes ataxia? Most often, ataxia is caused by loss of function in the part of the brain which serves as the "coordination center", which is the cerebellum. The cerebellum is located toward the back and lower part of the head. The right side of the cerebellum controls coordination on the right side of the body, and the left side controls coordination on the left. The central part of the cerebellum is involved in coordinating the very complex movements of gait, or walking. Other parts of the cerebellum help to coordinate eye movements, speech and swallowing. Ataxia may also be caused by dysfunction of the pathways leading into and out of the cerebellum. Information comes into the cerebellum from the spinal cord and other parts of the brain, and signals from the cerebellum go out to the spinal cord and to the brain. Although the cerebellum does not directly control strength ("motor function") or feeling ("sensory function"), the motor and sensory pathways must work properly to provide the correct input into the cerebellum. Thus, a person with impaired strength or sensation may notice clumsiness or poor coordination, and the doctor may say that person has ataxia. How does a physician diagnose ataxia? Remember, ataxia is a symptom, not a diagnosis. If you have clumsiness or loss of coordination in an arm or both legs or slurred speech, the physician may say that you have an ataxic arm or ataxic gait or ataxic speech. Then the physician must determine why this has happened. To do this, the physician will first ask many questions about your ataxia, how it came on, whether it is getting worse, whether there are any other symptoms, and so on. A very important part of the evaluation is the neurologic examination. The physician can usually determine whether the ataxia is caused by trouble in the cerebellum, its associated pathways, or other parts of the nervous system by his neurologic examination. A careful neurologic and general physical examination can also determine whether other parts of the nervous system are impaired and whether a medical illness may be causing the ataxia. Blood tests and X-rays may be very helpful in the diagnosing specific medical or neurologic conditions that can cause ataxia, or in "ruling out" suspected causes. Here is a list of some medical and neurologic conditions that can cause ataxia to appear suddenly: * head trauma * stroke * brain hemorrhage * brain tumor * congenital abnormality (the back part of the brain was formed in an unusual way) * "post-infectious" (after a severe viral infection * exposure to certain drugs or toxins (e.g. alcohol, seizure medicine) * following cardiac or respiratory arrest Here is a list of some medical and neurologic conditions that can cause ataxia to appear gradually: * hypothyroidism * deficiencies of certain vitamins (e.g. Vitamin E, Vitamin B12) * exposure to certain drugs or toxins (e.g. heavy metals, seizure medicine, chronic alcohol exposure, certain cancer drugs) * related to certain kinds of cancer (e.g. ovarian, lung cancer) * congenital abnormality (the back part of the brain was formed in an unusual way) * multiple sclerosis * syphilis (locomotor ataxia) * hereditary disorders * "idiopathic" (unknown cause) cerebellar degeneration disorders You can see that the list of conditions that the physician must think about as causes of ataxia is rather long; proper diagnosis may require a number of examinations, X-rays, and tests. How is ataxia treated? There is no medicine which specifically treats the symptoms of ataxia. If ataxia is due to a stroke, a low vitamin level, or exposure to a toxic drug or chemical, then treatment would include treatment of the stroke, vitamin therapy, or avoiding the toxic drug or chemical. There is no reason to think that taking vitamins or thyroid will help ataxia that is not caused by vitamin or thyroid deficiency. The treatment of incoordination or ataxia, then, mostly involves the use of adaptive devices to allow the ataxic individual to maintain as much independence as possible. Such devices may include a cane, crutches, walker, or wheelchair for those with impaired gait; devices to assist with writing, feeding, and self cares if hand and arm coordination is impaired; and communication devices for those with impaired speech. Many patients with hereditary or idiopathic forms of ataxia have symptoms in addition to ataxia. Medication or other therapies might be appropriate for some of these symptoms, which could include tremor, stiffness, depression, spasticity, and sleep disorders, among others. How can the National Ataxia Foundation help? Although the National Ataxia Foundation is interested in all forms of ataxia, its major focus is on the hereditary and idiopathic ataxias. While the treatment and prognosis of ataxia due to other causes such as stroke or tumor depends primarily on the treatment of the underlying cause, for the hereditary and idiopathic ataxias, little is understood of the underlying cause and no definitive treatment is available. The National Ataxia Foundation is committed to education about ataxia, service to individuals afflicted with the various forms of ataxia, and promoting research to find the causes, better treatments, or a cure for ataxia. NAF can help by providing information for you, your family, and your physician about ataxia. If you have additional questions or if we can be of any assistance, please contact: NATIONAL ATAXIA FOUNDATION 750 Twelve Oaks Center 15500 Wayzata Blvd. Wayzata, MN 55391 (612) 473-7666 FAX: (612) 473-9289 Email: naf@mr.net Copyright 1993 by the National Ataxia Foundation, Inc. All rights reserved.