Often caused by an underlying condition, ataxia affects muscle control and coordination and impacts movement, speech and swallowing.
Ataxia describes a lack of muscle control or coordination of voluntary movements, such as walking or picking up objects. A sign of an underlying condition, ataxia can affect various movements and create difficulties with speech, eye movement and swallowing.
Persistent ataxia usually results from damage to the part of your brain that controls muscle coordination (cerebellum). Many conditions can cause ataxia, including alcohol misuse, certain medication, stroke, tumor, cerebral palsy, brain degeneration and multiple sclerosis. Inherited defective genes also can cause the condition.
Treatment for ataxia depends on the cause. Adaptive devices, such as walkers or canes, might help you maintain your independence. Physical therapy, occupational therapy, speech therapy and regular aerobic exercise also might help.
Ataxia can develop over time or come on suddenly. A sign of a number of neurological disorders, ataxia can cause:
If you aren't aware of having a condition that causes ataxia, such as multiple sclerosis, see your doctor as soon as possible if you:
Damage, degeneration or loss of nerve cells in the part of your brain that controls muscle coordination (cerebellum), results in ataxia. Your cerebellum comprises two portions of folded tissue situated at the base of your brain near your brainstem. This area of the brain helps with balance as well as eye movements, swallowing and speech.
Diseases that damage the spinal cord and peripheral nerves that connect your cerebellum to your muscles also can cause ataxia. Ataxia causes include:
Toxic reaction. Ataxia is a potential side effect of certain medications, especially barbiturates, such as phenobarbital; sedatives, such as benzodiazepines; antiepileptic drugs, such as phenytoin; and some types of chemotherapy. Vitamin B-6 toxicity also may cause ataxia. These causes are important to identify because the effects are often reversible.
Also, some medications you take can cause problems as you age, so you might need to reduce your dose or discontinue the medication.
Alcohol and drug intoxication; heavy metal poisoning, such as from lead or mercury; and solvent poisoning, such as from paint thinner, also can cause ataxia.
For some adults who develop sporadic ataxia, no specific cause can be found. Sporadic ataxia can take a number of forms, including multiple system atrophy, a progressive, degenerative disorder.
Some types of ataxia and some conditions that cause ataxia are hereditary. If you have one of these conditions, you were born with a defect in a certain gene that makes abnormal proteins.
The abnormal proteins hamper the function of nerve cells, primarily in your cerebellum and spinal cord, and cause them to degenerate. As the disease progresses, coordination problems worsen.
You can inherit a genetic ataxia from either a dominant gene from one parent (autosomal dominant disorder) or a recessive gene from each parent (autosomal recessive disorder). In the latter case, it's possible neither parent has the disorder (silent mutation), so there might be no obvious family history.
Different gene defects cause different types of ataxia, most of which are progressive. Each type causes poor coordination, but each has specific signs and symptoms.
Episodic ataxia (EA). There are eight recognized types of ataxia that are episodic rather than progressive — EA1 through EA7, plus late-onset episodic ataxia. EA1 and EA2 are the most common. EA1 involves brief ataxic episodes that may last seconds or minutes. The episodes are triggered by stress, being startled or sudden movement, and often are associated with muscle twitching.
EA2 involves longer episodes, usually lasting from 30 minutes to six hours, which also are triggered by stress. You might have dizziness (vertigo), fatigue and muscle weakness during your episodes. In some cases, symptoms resolve in later life.
Episodic ataxia doesn't shorten life span, and symptoms might respond to medication.
Friedreich's ataxia. This common hereditary ataxia involves damage to your cerebellum, spinal cord and peripheral nerves. Peripheral nerves carry signals from your brain and spinal cord to your muscles. In most cases, signs and symptoms appear well before age 25.
The rate of disease progression varies. The first indication generally is difficulty walking (gait ataxia). The condition typically progresses to the arms and trunk. Muscles weaken and waste away over time, causing deformities, particularly in your feet, lower legs and hands.
Other signs and symptoms that might develop as the disease progresses include slow, slurred speech (dysarthria); fatigue; rapid, involuntary eye movements (nystagmus); spinal curvature (scoliosis); hearing loss; and heart disease, including heart enlargement (cardiomyopathy) and heart failure. Early treatment of heart problems can improve quality of life and survival.
Ataxia-telangiectasia. This rare, progressive childhood disease causes degeneration in the brain and other body systems. The disease also causes immune system breakdown (immunodeficiency disease), which increases susceptibility to other diseases, including infections and tumors. It affects various organs.
Telangiectasia is the formation of tiny red "spider" veins that might appear in the corners of your child's eyes or on the ears and cheeks. Delayed motor skill development, poor balance and slurred speech are typically the first indications of the disease. Recurrent sinus and respiratory infections are common.
Children with ataxia-telangiectasia are at high risk of developing cancer, particularly leukemia or lymphoma. Most people with the disease need a wheelchair by their teens and die before age 30, usually of cancer or lung (pulmonary) disease.
If you have ataxia, your doctor will look for a treatable cause. Besides conducting a physical exam and a neurological exam, including checking your memory and concentration, vision, hearing, balance, coordination, and reflexes, your doctor might request laboratory tests, including:
There's no treatment specifically for ataxia. In some cases, treating the underlying cause resolves the ataxia, such as stopping medications that cause it. In other cases, such as ataxia that results from chickenpox or other viral infections, it's likely to resolve on its own. Your doctor might recommend treatment to manage symptoms, such as depression, stiffness, tremor, fatigue or dizziness, or suggest adaptive devices or therapies to help with your ataxia.
Ataxia caused by conditions such as multiple sclerosis or cerebral palsy might not be treatable. In that case, your doctor may be able to recommend adaptive devices. They include:
You might benefit from certain therapies, including:
Research has shown that transcranial magnetic stimulation may help improve gait and postural control in people with ataxia, but more research is needed. Some studies have indicated that aerobic exercise also may be beneficial for some people with idiopathic ataxic syndromes.
The challenges you face when living with ataxia or having a child with the condition might make you feel alone or lead to depression and anxiety. Talking to a counselor or therapist might help. Or you might find encouragement and understanding in a support group, either for ataxia or for your underlying condition, such as cancer or multiple sclerosis.
Although support groups aren't for everyone, they can be good sources of information. Group members often know about the latest treatments and tend to share their own experiences. If you're interested, your doctor might be able to recommend a group in your area.
You're likely to start by seeing your family doctor or a general practitioner. In some cases, your doctor may refer you to a neurologist.
Here's some information to help you get ready for your appointment.
When you make the appointment, ask if there's anything you need to do in advance, such as fasting before having a specific test. Make a list of:
Take a family member or friend along, if possible, to help you remember the information you get.
For ataxia, basic questions to ask your doctor include:
Don't hesitate to ask other questions.
Your doctor is likely to ask you questions, such as:
Don't drink alcohol or take recreational drugs, which can make your ataxia worse.
December 24th, 2020