Ataxia

What is ataxia?

Ataxia is a loss of muscle control. People with ataxia lose muscle control in their arms and legs. This may lead to a lack of balance and coordination and trouble walking. Ataxia may affect the movements of:

• Fingers

• Hands

• Arms

• Legs

• Body

• Eyes

It can also affect the muscles that are used for speech.

What causes ataxia?

Many things can cause the symptoms of ataxia, including:

• Stroke

• Multiple sclerosis

• Tumors

• Alcoholism

• Nerve damage

• Metabolic disorders

• Low levels of some vitamins

• Immune system problems that attack the brain

• Brain infections

In these cases, treating the condition that caused ataxia may improve it.

The word ataxia usually describes symptoms. But it also describes a group of certain degenerative diseases of the central nervous system. These are:

• Hereditary ataxia. This type is caused by a defect in a gene that a person is born with. Hereditary ataxia may progress over a number of years. How severe the disability is depends on the type of ataxia, the age when the symptoms start, and other factors. Some types of hereditary ataxia start in childhood. Others start in the adult years.

• Sporadic ataxia. This type usually starts in adulthood. There is no known family history.

With hereditary ataxia, a defective gene makes abnormal proteins. These cause nerve cell damage and lead to ataxia. As the disease gets worse, muscles react less and less to the commands of the brain. This causes balance and coordination to get worse over time.

What are the symptoms of ataxia?

Symptoms and when they start may vary, depending on the type of ataxia. The most common symptoms include:

• Balance and movement problems (affected first)

• Poor movement of hands, arms, and legs

• Slurring of speech

• Wide-based gait when walking

• Trouble writing and eating

• Slow eye movements

• Falls

The symptoms of ataxia may look like other conditions or medical problems. Make sure to see your healthcare provider for a diagnosis.

How is ataxia diagnosed?

Your healthcare provider will ask about your health history and your family’s health history. You will also have a neurological and physical exam. And you may have tests such as:

• Lab tests. These include blood and urine tests.

• Genetic tests. These tests are done to see if a person has certain gene changes (mutations) or chromosome changes that are known to increase the risk for some inherited conditions.

• Magnetic Resonance Imaging (MRI). This is a test that uses large magnets, radio waves, and a computer to make detailed images of organs and structures in the body.

• Nerve conduction studies (NCS/NVC) and electromyography (EMG). This test is done to check the nerves in the arms and legs to see if they are working normally.

These tests may also be used to look for other conditions that can cause ataxia to start suddenly. Certain conditions can cause ataxia to develop suddenly. These include:

• Head injury

• Stroke

• Brain hemorrhage

• Infections

• Exposure to certain drugs

• Temporary loss of oxygen supply to the brain, such as if the heart or breathing slows or stops

Some conditions can cause ataxia to appear slowly. These include:

• Hypothyroidism

• Alcohol abuse

• Low levels of some vitamins

• Chronic exposure to certain things in your environment, drugs, or medicines

• Multiple sclerosis

• Other disorders

How is ataxia treated?

Ataxia may be short-term (temporary), such as being under the influence of alcohol, medicine, or drugs. Or it can be long-term (permanent) from a stroke or other brain or nerve injury. It can also get worse (be progressive) from a degenerative disorder. Your prognosis depends on the cause.

There is no cure for hereditary ataxia. But treatments can help manage symptoms. Treating other causes of ataxia can also help reduce symptoms.

If ataxia is caused by a stroke, a low vitamin level, or contact with a toxic drug or chemical, then treatment focuses on those causes.

If the ataxia is caused by your immune system attacking the brain, you may have treatments to suppress your immune system. Looking for and treating the cause of the immune defect (such as a tumor in the body) can also improve the symptoms.

The treatment for the lack of coordination or imbalance is mostly done with the use of adaptive devices and physical therapy. These can help you to be as independent as possible. These devices may include a cane, crutches, a walker, or a wheelchair. Physical therapy, speech therapy, and medicines can help lessen symptoms. These can help with tremor, stiffness, depression, spasticity, and sleep disorders.

People who are prone to fall may get help by making changes to their home. These changes can include removing fall and trip hazards, living on a single floor, or using a stair lift.

Research is being done on cerebellar and spinocerebellar degeneration. This includes work to find the causes of ataxias and ways to treat, cure, and prevent them.

What are possible complications linked to ataxia?

Each type of ataxia may progress differently. Falling or becoming chair- or bed-bound may lead to injury, pressure sores, infection, and blood clots. Depending on the cause of the ataxia, some people may have other symptoms that affect different parts of the nervous system. These include dementia, behavioral problems, and depression. Other complications that may occur with some forms of ataxia may include:

• Dizziness

• Spasticity

• Rigidity

• Tremor

• Pain

• Fatigue

• Bowel or bladder dysfunction

• Sexual dysfunction

• Falls with injury

In severe cases, a person may have rigidity that is not treatable, breathing trouble, or choking, which can lead to death. Some of the most difficult symptoms need to be managed with special care. These may include a continuous positive airway pressure device (CPAP), tracheostomy, or a feeding tube. Many things can be done to improve the quality of life of the person with ataxia.

Key points about ataxia

• People with ataxia lose muscle control in their arms and legs. This may lead to a lack of balance and coordination and trouble walking.

• Ataxia may affect the fingers, hands, arms, legs, body, speech, and eye movements.

• Some injuries or illnesses can cause ataxia to appear suddenly. These include head injury, stroke, brain hemorrhage, infections, and other problems.

• Some conditions can cause ataxia to appear gradually. These include hypothyroidism, alcohol abuse, low levels of some vitamins, and other problems.

• Getting regular follow-up care with medical specialists such as doctors, physical therapists, and speech therapists can help manage symptoms.

Next steps

Tips to help you get the most from a visit to your healthcare provider:

• Know the reason for your visit and what you want to happen.

• Before your visit, write down questions you want answered.

• Bring someone with you to help you ask questions and remember what your provider tells you.

• At the visit, write down the name of a new diagnosis and any new medicines, treatments, or tests. Also write down any new instructions your healthcare provider gives you.

• Know why a new medicine or treatment is prescribed and how it will help you. Also know what the side effects are.

• Ask if your condition can be treated in other ways.

• Know why a test or procedure is recommended and what the results could mean.

• Know what to expect if you do not take the medicine or have the test or procedure.

• If you have a follow-up appointment, write down the date, time, and purpose for that visit.

• Know how you can contact your healthcare provider if you have questions.