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doi: 10.1097/01.NNN.0000359089.03185.c5
DEPARTMENT: ASK THE EXPERT: Your Question Answered

William Weiner, M.D., is chairman of the department of neurology at the University of Maryland School of Medicine and chief of Neurology at the University of Maryland Medical Center in Baltimore, MD.

Q What causes adult-onset ataxia, and how can it be treated?

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A The word “ataxia” is used to describe a symptom—lack of coordination—which can be associated with injuries or degenerative changes in the central nervous system. Examples of such injuries and changes include stroke, multiple sclerosis, head injury, or alcoholism. This is known as acquired ataxia.

Ataxia also indicates a group of specific degenerative and progressive diseases of the nervous system called the hereditary and sporadic ataxias. These diseases damage parts of the nervous system that control movement. Often the first apparent symptom of these disorders is difficulty with balance and walking. Symptoms of hereditary ataxias commonly begin in childhood, but one type—Friedreich's ataxia—has an adult onset in some cases. People with Friedreich's ataxia develop weakness in the muscles of the feet, lower legs, and hands. They often rely on a wheelchair within 15 years of the appearance of symptoms. As the disease progresses, patients may experience slow, slurred speech; rapid, involuntary eye movements; spinal curvature (scoliosis); and heart disease and heart failure.

People with sporadic ataxia have symptoms that usually begin in adulthood but no known family history of the disease. Sporadic ataxias may result from a new abnormality of the gene or as a result of an underlying disease, including thyroid disease, chronic hypoglycemia, stroke, and vitamin deficiencies.

The type of ataxia and the age of symptom onset indicate how severe the disability will become and whether the disease will lead to death. There is no specific treatment for ataxia and no cure for the hereditary ataxias, but adaptive devices (such as canes or walkers) and therapies (such as speech therapy to improve speech and aid swallowing) can help. To identify an ataxia, your physician should take your medical and family history and conduct a complete neurological evaluation, including an MRI scan of the brain. Genetic blood tests are now available to confirm a diagnosis for some types of hereditary ataxias.

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