Definition

What is ataxia?

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.

How common is ataxia?

Please discuss with your doctor for further information.

Symptoms

What are the symptoms of ataxia?

The common symptoms of ataxia are:

  • Difficulty walking with an unsteady gait, which is often wide-based and staggering. This can lead to stumbling and falling.
  • Speech may be slurred and slow with an unusual rhythm to the words.
  • Swallowing may be difficult and the patient may choke, especially when drinking liquids.
  • Difficulty using the hands and fingers to button shirts, or pick up a fork or spoon to eat. Writing, keyboarding, sewing, and playing an instrument can be affected.
  • Nystagmus is the term used to describe rapid involuntary eye movements where the eyes jerk back and forth. This can cause blurred vision and difficulty reading or moving the eyes from one word to the next.
  • Fatigue is a common complaint, likely because the muscles of the body have to work harder to overcome the lack of coordination to perform their daily activities.

There may be some symptoms not listed above. If you have any concerns about a symptom, please consult your doctor.

When should I see my doctor?

If you have any signs or symptoms listed above or have any questions, please consult with your doctor. Everyone’s body acts differently. It is always best to discuss with your doctor what is best for your situation.

Causes

What causes ataxia?

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.
  • 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.
  • 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.
  • 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.

Risk factors

What increases my risk for ataxia?

Please discuss with your doctor for further information.

Diagnosis & treatment

The information provided is not a substitute for any medical advice. ALWAYS consult with your doctor for more information.

How is ataxia diagnosed?

A doctor who suspects the patient may have ataxia will refer them to a neurologist – a doctor specialized in diagnosis, treatment, and prevention of conditions and illnesses related to the nervous system.

The specialist will check the patient’s medical history for causal factors, such as brain injury, as well as the patient’s family history for indications of inheritance. The following tests may also be ordered:

  • MRI or CT scan – to determine whether there is any brain damage.
  • Genetic tests – to determine whether the patient has inherited ataxia.
  • Blood tests – some types of ataxia affect the make-up of blood.
  • Urine tests – urinalysis may suggest specific systemic abnormalities that are linked to some forms of ataxia. If the specialist suspects Wilson’s disease, the patient may be asked for a 24-hour urine collection to determine how much copper is in the system.

In some cases, the specialist may not be able to determine the cause of ataxia; this is known as sporadic or idiopathic ataxia.

Reaching a conclusive diagnosis of ataxia can sometimes take a long time because the symptoms could point to several other conditions.

How is ataxia treated?

Although ataxia is generally not curable, a great deal can be done to ease symptoms and improve the quality of life of the patient. Treatment for coordination and balance problems usually involves the use of adaptive devices that help the patient attain as much independence as possible. These may include the use of a cane (walking stick), crutches, walker, or a wheelchair.

Symptoms such as tremor, stiffness, spasticity, sleep disorders, muscle weakness, depression (or frustration, sadness, and anger) may be addressed with targeted physical therapy, speech therapy, medications, and counseling.

  • Occupational therapy – the occupational therapist can help the patient manage better around the house and at work; this may involve some home adaptations, wheelchair assessments, and making the kitchen more practical for the patient.
  • Speech therapy – the speech therapist can help with swallowing, coughing, choking, and speech problems. If speech becomes very difficult, the speech therapist can help the patient learn how to use speech aids.
  • Orthopedic care – this can help patients with curvature of the spine (scoliosis).
  • Physical therapy – a physical therapist can help maintain strength and improve mobility.
  • Counseling – patients with ataxia commonly become frustrated and depressed; this usually results from having to cope with some symptoms that affect physical mobility and coordination. Talking to a well-qualified counselor, such as a psychotherapist, can help.
  • Supplements and nutrition – some patients with ataxia have very low levels of vitamin E and require supplements and/or a special diet. As sensitivity to gluten may occur among some patients with ataxia, a gluten-free diet may help.
  • Medication – some patients with ataxia telangiectasia are prescribed gamma-globulin injections to boost their immune systems. There are also drugs for muscle spasms and uncontrollable eye movements.

Lifestyle changes & home remedies

What are some lifestyle changes or home remedies that can help me manage ataxia?

The following lifestyles and home remedies might help you cope with ataxia:

  • 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.

If you have any questions, please consult with your doctor to better understand the best solution for you.

Hello Health Group does not provide medical advice, diagnosis or treatment.

Sources

Review Date: September 8, 2017 | Last Modified: September 8, 2017

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