Ataxia

What is ataxia?

Ataxia means without coordination. People with ataxia lose muscle control in their arms and legs. This may lead to a lack of balance, coordination, and trouble walking. Ataxia may affect the fingers, hands, arms, legs, body, speech, and even eye movements.

The symptom of ataxia can be caused by many things including:

• Stroke
• Multiple sclerosis
• Tumors
• Alcoholism
• Nerve damage 
• Metabolic disorders
• Vitamin deficiencies

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

While the term ataxia usually describes symptoms, it also describes a group of specific degenerative diseases of the central nervous system called the hereditary and sporadic ataxias:

• Hereditary ataxias. This type is caused by a defect in a gene that a person is born with. Hereditary ataxias are degenerative disorders that may progress over a number of years. How severe the disability depends on the type of ataxia, the age of onset of symptoms, and other factors. Certain types of hereditary ataxias start in childhood while others start in the adult years.
• Sporadic ataxias. This type usually starts in adulthood and has no known family history.

What causes ataxia?

A defective gene makes abnormal proteins that cause the nerve cell degeneration leading to ataxia. As the disease progresses, muscles become less and less responsive to the commands of the brain. This causes balance and coordination to worsen.

What are the symptoms ataxia?

Symptoms and time of onset may vary according to the type of ataxia. Typically, the most common include:

• Balance and coordination problems (affected first)
• Poor coordination of hands, arms, and legs
• Slurring of speech
• Wide-based gait (manner of walking)
• Difficulty with writing and eating
• Slow eye movements

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

How is ataxia diagnosed?

Along with a thorough medical history, family history, and complete neurological and physical exam, these tests may be done:

• Lab tests (including blood and urine studies)
• Genetic testing. Tests done to determine if a person has certain gene changes (mutations) or chromosome changes which are known to increase risk for certain inherited conditions
• Magnetic resonance imaging (MRI). A test that uses large magnets, radiofrequency energy, and  a computer to make detailed images of organs and structures in the body

These tests may also be used to rule out other conditions that can cause ataxia. Certain conditions can cause ataxia to develop suddenly, such as head injury, stroke, brain hemorrhage, infections, exposure to certain drugs, and also if the heart or breathing slow or stop.

Some conditions can cause ataxia to appear gradually, such as hypothyroidism, alcohol abuse, certain vitamin deficiencies, chronic exposure to certain drugs, multiple sclerosis, and other disorders.

How is ataxia treated?

There is no cure for the hereditary ataxias. And, there is no medicine to treat the specific symptom of ataxia.

If ataxia is due to a stroke, a low vitamin level, or exposure to a toxic drug or chemical, then treatment is aimed at treating those specific conditions.

The treatment for the lack of coordination or imbalance mostly involves the use of adaptive devices to allow the person to maintain as much independence as possible. These devices may include a cane, crutches, a walker, or a wheelchair. Physical therapy, speech therapy, and medicines to help symptoms, such as tremor, stiffness, depression, spasticity, and sleep disorders may also help.

Research is being done on cerebellar and spinocerebellar degeneration, including work aimed at finding the cause(s) of ataxias and ways to treat, cure, and ultimately prevent them.

What are the complications of ataxia?

Progression of the different types of ataxia may vary with each specific syndrome. In the worst case scenario, the person may have untreatable rigidity, breathing trouble, or choking which can lead to death. Some of the most difficult symptoms require management with continuous positive airway pressure devices (CPAP), tracheostomy, or a feeding tube.

Falling or becoming chair- or bed-bound may lead to other life threatening complications, such as injury, pressure sores, infection, and blood clots. Dementia, behavioral problems, and depression may influence compliance and care. Other complications of ataxia may include:

• Dizziness
• Spasticity
• Rigidity
• Tremor
• Pain
• Fatigue
• Low blood pressure with sitting or standing
• Bowel or bladder dysfunction
• Sexual dysfunction

Many things can be done to improve the quality of life of the person with ataxia.

When should I call my healthcare provider?

Symptoms and time of onset may vary according to the type of ataxia. Each person may experience symptoms differently.  Contact your provider if you have any of the following symptoms: 

• Balance and coordination problems
• Lack of coordination in hands, arms, or legs
• Slurring of speech
• Wide-based gait (manner of walking)
• Difficulty with writing and eating
• Slow eye movements

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

Key points about ataxia

• People diagnosed with ataxia lose muscle control in their arms and legs, which may lead to a lack of balance, coordination, and trouble walking.
• Ataxia may affect the fingers, hands, arms, legs, body, speech, and even eye movements.
• Typically the most common symptoms of ataxia are listed below:
  • Balance and coordination are affected first
  • Poor coordination of hands, arms, and legs
  • Slurring of speech
  • Wide-based gait (manner of walking)
  • Difficulty with writing and eating
  • Slow eye movements
• Certain injuries or illnesses can cause ataxia to appear suddenly, such as head injury, stroke, brain hemorrhage, infections, exposure to certain drugs, or if breathing or the heart stops.
• Some conditions can cause ataxia to appear gradually, such as hypothyroidism, alcohol abuse, certain vitamin deficiencies, chronic exposure to certain drugs, multiple sclerosis, and other disorders.

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 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 provider if you have questions.