Definition
Ataxia describes a lack of muscle control during voluntary movements, such as walking or picking up objects. A sign of an underlying condition, ataxia can affect movement, speech, eye movement and swallowing.
Persistent ataxia usually results from damage to your cerebellum the part of your brain that controls muscle coordination. Many conditions can cause ataxia, including alcohol abuse, stroke, tumor, cerebral palsy and multiple sclerosis. An inherited defective gene also can cause ataxia.
Treatment for ataxia depends on the cause. Adaptive devices, such as walkers or canes, might help you maintain your independence. Physical therapy, occupational therapy and speech therapy also might help.
Ataxia can develop over time or come on suddenly. Ataxia, a sign of a number of neurological disorders, may cause:
If you aren't aware of having a condition that causes ataxia, such as multiple sclerosis, see your doctor 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 pingpong-ball-sized portions of folded tissue situated at the base of your brain near your brainstem. The right side of your cerebellum controls coordination on the right side of your body; the left side of your cerebellum controls coordination on the left.
Diseases that damage the spinal cord and peripheral nerves that connect your cerebellum to your muscles also may cause ataxia. Ataxia causes include:
For some adults who develop sporadic ataxia, no specific cause can be found. This is known as sporadic degenerative ataxia, which 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 may 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.
These include:
Episodic ataxia. There are seven recognized types of ataxia that are episodic rather than progressive EA1 through EA7. 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, that also are triggered by stress. With this type of ataxia, you may experience dizziness (vertigo), fatigue and muscle weakness during your episodes. In some cases of episodic ataxia, symptoms resolve in later life.
Episodic ataxia doesn't shorten life span, and symptoms may respond to medication.
These include:
Friedreich's ataxia. This, the most 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 before the age of 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 may 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.
Ataxia-telangiectasia. This rare, progressive childhood disease causes degeneration in the brain and other body systems. The disease causes immune system breakdown (immunodeficiency disease), which increases susceptibility to other diseases. It affects various organs.
Telangiectasias are tiny red "spider" veins that may appear in the corners of your child's eyes or on the ears and cheeks. Although they're characteristic of the disease, your child may not develop them. 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 in their teens or early 20s.
In an autosomal dominant disorder, the mutated gene is a dominant gene located on one of the nonsex chromosomes (autosomes). You need only one mutated gene to be affected by this type of disorder. A person with an autosomal dominant disorder in this case, the father has a 50 percent chance of having an affected child with one mutated gene (dominant gene) and a 50 percent chance of having an unaffected child with two normal genes (recessive genes).
To have an autosomal recessive disorder, you inherit two mutated genes, one from each parent. These disorders are usually passed on by two carriers. Their health is rarely affected, but they have one mutated gene (recessive gene) and one normal gene (dominant gene) for the condition. Two carriers have a 25 percent chance of having an unaffected child with two normal genes (left), a 50 percent chance of having an unaffected child who also is a carrier (middle), and a 25 percent chance of having an affected child with two recessive genes (right).
You're likely to start by seeing your family doctor or a general practitioner. However, in some cases, your doctor may refer you immediately to a neurologist.
Here's some information to help you get ready for your appointment.
Preparing a list of questions will help you make the most of your time with your doctor. For ataxia, some basic questions to ask include:
Don't hesitate to ask other questions.
Examples of questions your doctor may ask, include:
Don't drink alcohol or take recreational drugs, which can make your ataxia worse.
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 may request laboratory tests, including:
There's no treatment specifically for ataxia. In some cases, treating the underlying cause resolves the ataxia. In other cases, such as ataxia that results from chickenpox or other viral infection, it's likely to resolve on its own over time. Your doctor may recommend adaptive devices or therapies to help with your ataxia.
For ataxia caused by conditions such as multiple sclerosis or cerebral palsy, ataxia 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:
The challenges you face when living with ataxia, such as loss of independence, or having a child with the condition, may make you feel alone or lead to depression and anxiety. Talking to a counselor or therapist may lessen your sense of isolation and help you cope. Or you may 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 may be able to recommend a group in your area.
Last updated: March 29th, 2014
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