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Ataxia

Overview

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, creating 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 abuse, certain medications, 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.

Cerebellum and brainstem

Persistent ataxia usually results from damage to the part of your brain that controls muscle coordination (cerebellum).

Symptoms

Ataxia can develop over time or come on suddenly. A sign of a number of neurological disorders, ataxia can cause:

  • Poor coordination
  • Unsteady walk and a tendency to stumble
  • Difficulty with fine motor tasks, such as eating, writing or buttoning a shirt
  • Change in speech
  • Involuntary back-and-forth eye movements (nystagmus)
  • Difficulty swallowing

When to see a doctor

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:

  • Lose balance
  • Lose muscle coordination in a hand, arm or leg
  • Have difficulty walking
  • Slur your speech
  • Have difficulty swallowing

Causes

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 can cause ataxia. Ataxia causes include:

  • Head trauma. Damage to your brain or spinal cord from a blow to your head, such as might occur in a car accident can cause acute cerebellar ataxia, which comes on suddenly.
  • Stroke. When the blood supply to a part of your brain is interrupted or severely reduced, depriving brain tissue of oxygen and nutrients, brain cells die.
  • Cerebral palsy. This is a general term for a group of disorders caused by damage to a child's brain during early development — before, during or shortly after birth — that affects the child's ability to coordinate body movements.
  • Autoimmune diseases. Multiple sclerosis, sarcoidosis, celiac disease and other autoimmune conditions can cause ataxia.
  • Infections. Ataxia can be an uncommon complication of chickenpox and other viral infections. It might appear in the healing stages of the infection and last for days or weeks. Normally, the ataxia resolves over time.
  • Paraneoplastic syndromes. These are rare, degenerative disorders triggered by your immune system's response to a cancerous tumor (neoplasm), most commonly from lung, ovarian, breast or lymphatic cancer. Ataxia can appear months or years before the cancer is diagnosed.
  • Tumor. A growth on the brain, cancerous (malignant) or noncancerous (benign), can damage the cerebellum.
  • Toxic reaction. Ataxia is a potential side effect of certain medications, especially barbiturates, such as phenobarbital; sedatives, such as benzodiazepines; and some types of chemotherapy. These 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.

  • Vitamin E, vitamin B-12 or thiamine deficiency. Not getting enough of these nutrients,because of the inability to absorb enough, alcohol abuse or other reasons, can lead to 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.

Hereditary ataxias

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.

Autosomal dominant ataxias

These include:

  • Spinocerebellar ataxias. Researchers have labeled more than 35 autosomal dominant ataxia genes, and the number continues to grow. Cerebellar ataxia and cerebellar degeneration are common to all types, but other signs and symptoms, as well as age of onset, differ depending on the specific gene mutation.
  • Episodic ataxia (EA). 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. 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.

Autosomal recessive ataxias

These include:

  • 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.

    Telangiectasias are 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.

  • Congenital cerebellar ataxia. This type of ataxia results from damage to the cerebellum that's present at birth.
  • Wilson's disease. People with this condition accumulate copper in their brains, livers and other organs, which can cause neurological problems, including ataxia. Early identification of this disorder can lead to treatment that will slow progression.
Cerebellum and brainstem

Persistent ataxia usually results from damage to the part of your brain that controls muscle coordination (cerebellum).

Cerebellum and brainstem

Persistent ataxia usually results from damage to the part of your brain that controls muscle coordination (cerebellum).

Autosomal dominant inheritance pattern

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).

Autosomal recessive inheritance pattern

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).

Diagnosis

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:

  • Imaging studies. A CT scan or MRI of your brain might help determine potential causes. An MRI can sometimes show shrinkage of the cerebellum and other brain structures in people with ataxia. It may also show other treatable findings, such as a blood clot or benign tumor, that could be pressing on your cerebellum.
  • Lumbar puncture (spinal tap). A needle is inserted into your lower back (lumbar region) between two lumbar bones (vertebrae) to remove a sample of cerebrospinal fluid. The fluid, which surrounds and protects your brain and spinal cord, is sent to a laboratory for testing.
  • Genetic testing. Your doctor might recommend genetic testing to determine whether you or your child has the gene mutation that causes one of the hereditary ataxic conditions. Gene tests are available for many but not all of the hereditary ataxias.

Treatment

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 infection, it's likely to resolve on its own. Your doctor might recommend treatment to manage symptoms, such as pain, fatigue or dizziness or adaptive devices or therapies to help with your ataxia.

Adaptive devices

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:

  • Hiking sticks or walkers for walking
  • Modified utensils for eating
  • Communication aids for speaking

Therapies

You might benefit from certain therapies, including:

  • Physical therapy to help your coordination and enhance your mobility
  • Occupational therapy to help you with daily living tasks, such as feeding yourself
  • Speech therapy to improve speech and aid swallowing

Preparing for an appointment

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.

What you can do

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:

  • Your symptoms, including any that may seem unrelated to the reason for which you scheduled the appointment, and when they began
  • Key personal information, including other conditions you have and family medical history
  • All medications, vitamins or supplements you take, including doses
  • Questions to ask your doctor

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:

  • What is likely causing my symptoms?
  • Other than the most likely cause, what are other possible causes?
  • What tests do I need?
  • Is my condition likely temporary or chronic?
  • What's the best course of action?
  • Are there devices that can help me with coordination?
  • I have other health conditions. How can I best manage them together?
  • Are there restrictions I need to follow?
  • Should I see a specialist?
  • Are there brochures or other printed material I can take with me? What websites do you recommend?
  • Do you know of ataxia research studies I might participate in?

Don't hesitate to ask other questions.

What to expect from your doctor

Your doctor is likely to ask you questions, such as:

  • Are your symptoms continuous or occasional?
  • How severe are your symptoms?
  • What seems to improve your symptoms?
  • What seems to worsen your symptoms?
  • Do you have family members who have had these types of symptoms?
  • Do you use alcohol or drugs?
  • Have you been exposed to toxins?
  • Have you had a virus recently?

What you can do in the meantime

Don't drink alcohol or take recreational drugs, which can make your ataxia worse.

Coping and support

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.

Last updated: August 16th, 2017

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