What Is Ataxia?
‘Ataxia’ describes a group of neurological symptoms with many causes, but it also refers to certain degenerative neurological diseases. That means ataxia may be defined as a symptom or condition, depending on the underlying cause. Its literal meaning is ‘without coordination.’ People with ataxia often resemble someone who has had too much to drink. You may notice slurred speech, stumbling, falling, and a general lack of control over movement. People with ataxia may also walk with a distinctive gait using a wide stance. Ataxia is rare, but it can affect anyone from young children to older adults.
Causes of Ataxia
Ataxia is a result of damage to various parts of the brain and nervous system. Most often, it affects the part of the brain called the cerebellum. That is cerebellar ataxia. The cerebellum governs balance and coordination as well as eye movements, speech, and swallowing. Ataxia may affect all of these functions. Alcohol also affects the cerebellum, which is why the symptoms of intoxication look similar to ataxia symptoms.
Ataxia can be either acquired or genetic. There are many causes of acquired ataxia—it could be from trauma to the brain or spinal cord, an autoimmune disorder, lack of vitamin E or B12, alcohol abuse, or even chickenpox (children who develop ataxia after chickenpox often return to normal). If it appears suddenly, it is known as sporadic ataxia.
Genetic ataxia is often hereditary, meaning it runs in families, but it can also appear in someone with no known family history. Some forms of genetic ataxia are:
Friedreich's ataxia tends to appear before people reach their mid-teens. It damages the cerebellum, spinal cord, and peripheral nerves. Muscles weaken, particularly in the feet, lower legs, and hands. In addition to the characteristic gait and slurred speech, people may develop involuntary eye movements, hearing loss, and heart disease.
Ataxia-telangiectasia is a form of ataxia found in children. It affects various organs including the brain, and weakens the immune system. Telangiectasias are ‘spider’ veins, small red blood vessels in the corner of the eye or on the ears or face. Children may have developmental delays as well as slurred speech and difficulty with balance and walking. They are prone to respiratory infections and at increased risk for developing cancers, such as lymphoma or leukemia, as the disease progresses.
Diagnosis and Treatment of Ataxia
Doctors diagnose ataxia by its distinctive symptoms, through neurological exams and tests. They may also order an MRI or other imaging study. DNA testing can identify some forms of genetic ataxia, but not all.
There is a wide range of treatment options available that can help address the many symptoms of ataxia, but there is no cure for it. In certain cases of acquired ataxia where the doctor can determine and treat the underlying cause, the symptoms may go away. Sometimes, symptoms will disappear on their own in acquired ataxia.
Physical therapy and an exercise regimen can help people with ataxia control movement so they can remain independent longer. They can also use adaptive devices to help with daily living, from special eating utensils to mobility aids. Research for new medications to treat ataxia have advanced enough to enroll patients in experimental trials. Ataxia is a complicated disorder with many faces. It may affect life span, but in other cases people with ataxia have a normal life expectancy and may remain independent for many years.