Ataxia: Causes, Symptoms, and Treatment

What Is Ataxia?

Ataxia is when you have poor muscle control that causes clumsy or awkward movements, like having trouble walking or balancing. It can affect your legs, arms, hands, eyes, and speech. Ataxia can have many different causes.

Ataxia is usually considered a symptom, but it can also refer to some specific diseases. 

Ataxia vs. apraxia

Both ataxia and apraxia may involve trouble with movement and speech, but they are different. Apraxia is a condition that affects your brain, making it hard for you to perform a familiar task or movement, even though your muscles are functioning properly. With ataxia, you lose the physical ability to control muscles.

Ataxia Symptoms

Symptoms of ataxia may include:

  • Unsteady walking
  • Walking with your feet unusually wide apart (called a wide gait)
  • Poor balance
  • Poor coordination
  • Trouble with fine motor tasks like writing
  • Trouble with speech
  • Trouble swallowing
  • Back-and-forth eye movements that you can’t control

Ataxia Types

There are many types of ataxia, and they are sometimes divided into categories based on what caused the ataxia.

Cerebellar ataxia

Your cerebellum is the part of your brain that’s in charge of balance and coordination. If part of your cerebellum is damaged, you can develop cerebellar ataxia. Sometimes it can also affect your spinal cord. It’s the most common form of ataxia.

Symptoms of cerebellar ataxia include:

  • Changes in your voice
  • Dizziness
  • Fatigue
  • Headaches
  • Muscle tremors (shaking or trembling)
  • Slurred speech
  • Trouble walking
  • Wide gait

Sensory ataxia

Sensory ataxia is the result of damage to nerves in your spinal cord or your peripheral nervous system. That is the part of your nervous system outside of the brain and spinal cord.

When you have sensory ataxia, you have less sensation in your feet and legs from the nerve damage, so you have less feedback from your brain telling you where your body is in relation to the ground. It’s also called proprioceptive ataxia.

Symptoms of sensory ataxia include:

  • Difficulty touching your finger to your nose when your eyes are closed
  • Inability to sense vibrations
  • Trouble walking in dim light
  • Walking with a “heavy step,” or stomping when you walk

Continued

Vestibular ataxia

Vestibular ataxia affects your vestibular system. This system is made up of your inner ear and ear canals, which contain fluid. They sense the movements of your head and help with your balance and spatial orientation.

When the nerves in your vestibular system are affected, you can have the following problems:

  • Blurred vision and other eye issues
  • Nausea and vomiting
  • Problems standing and sitting
  • Staggering when you walk
  • Trouble walking in a straight line
  • Vertigo, or dizziness

Friedreich’s ataxia

This type of ataxia is genetic, or inherited, and it’s caused by a defect in a gene called FXN. It’s a degenerative disease, meaning that the condition worsens over time, and it damages the cerebellum, spinal cord, and peripheral nerves. Symptoms usually start in childhood, between ages 5 and 15. They include:

  • Trouble walking
  • Loss of sensation in the legs at first, and then in the arms and torso
  • Poor reflexes
  • Tiredness
  • Slow or slurred speech
  • Loss of vision or hearing
  • Chest pain
  • Heart palpitations 
  • Shortness of breath

Limb ataxia

If you have limb ataxia, you lack coordination in your arms. This might mean you have:

  • Trouble writing 
  • Trouble buttoning or fastening clothes
  • Trouble picking up small objects

Ataxia-telangiectasia

Ataxia-telangiectasia is inherited, and symptoms usually start in childhood. 

Telangiectasias are spider veins, or broken blood vessels that look like fine red or pink lines near the surface of your skin. One symptom of ataxia-telangiectasia is these red lines in the corners of your eyes or on your cheeks. Other signs include:

  • Trouble walking
  • Slow or slurred speech
  • Difficulty swallowing
  • Slow eye movements
  • A weakened immune system
  • Increased risk of cancer

Truncal ataxia

This type of ataxia affects your trunk, or your torso. With truncal ataxia, your trunk may be unstable or unbalanced when you’re sitting, standing, or walking. 

Gluten ataxia

Gluten ataxia is a disease in which your body’s immune system attacks your nervous system in reaction to eating gluten. It may be related to celiac disease, which is caused by your body’s immune response to eating gluten. Symptoms include:

  • Trouble walking or moving your limbs
  • Poor coordination or balance
  • Trouble speaking
  • Trouble moving your eyes
  • A feeling of tingling in your extremities

Continued

Episodic ataxia

Episodic ataxia is when you have episodes of ataxia, but the rest of the time, you have no symptoms or only mild symptoms. The episodes can last between minutes and hours. This type of ataxia is inherited, and it’s unusual. 

Symptoms during an episode of ataxia may include:

  • Lack of balance and coordination
  • Trouble with speech
  • Muscle spasms
  • Involuntary eye movements
  • Vertigo (feeling like you’re spinning)
  • Migraines
  • Tinnitus (when you hear ringing or other noises)

Spinocerebellar ataxia

More than 40 types of spinocerebellar ataxia exist. These types of ataxia affect your cerebellum, a part of your brain that affects physical movement. Spinocerebellar ataxia is inherited. The causes and symptoms vary with different types, but common symptoms are:

  • Loss of coordination and balance
  • Trouble walking
  • Slow movements
  • Loss of muscle tone
  • Vision problems
  • Trouble with speech and swallowing
  • Trouble with memory and concentration

Symptoms usually appear when you’re an adult and worsen over time. The most common types of spinocerebellar ataxia (SCA) are called SCA1, SCA2, SCA3, and SCA6.

Other types of ataxia

Other types of ataxia exist as well. They include:

  • RFC1-associated ataxia, a type that begins later in life
  • Idiopathic late-onset cerebellar ataxia (ILOCA), which begins around age 50
  • Wilson’s disease, which involves copper building up in your organs
  • Congenital cerebellar ataxia, in which damage to the cerebellum is present at birth
  • Ataxia with vitamin E deficiency, in which your body has problems using vitamin E
  • Acquired ataxia, which may develop quickly

Symptoms vary from one type of ataxia to another, but symptoms like trouble with coordination are common in many of them. 

Ataxia Causes

Around 150,000 people in the U.S. deal with some form of ataxia. There are different causes for it. Some are genetic, some are acquired, like injuries, and some have no known cause.

Genetic. You can inherit a certain mutated, or changed, gene from one or both of your parents that causes ataxia. Or you may inherit a mutated gene that causes a disorder with ataxia as a symptom.

Continued

Some of the specific types of genetic ataxia include:

  • Ataxia-telangiectasia
  • Ataxia with oculomotor apraxia
  • Dominant spastic ataxias
  • Dominant spinocerebellar ataxias (SCA)
  • Episodic ataxia
  • Friedreich's ataxia
  • Recessive spastic ataxias
  • Wilson’s disease

Acquired. Acquired ataxia occurs when you have damage to your spinal cord or nerves. The damage might be from an injury or an illness.

Some of the causes of acquired ataxia could include:

You can also get ataxia if you have a reaction to certain medications, from alcohol or drug use, or from exposure to poison.

Idiopathic. When you haven’t inherited a mutated gene or had an illness or injury that could have caused your ataxia, it’s called idiopathic ataxia. Your doctor will diagnose you with idiopathic ataxia if they can’t find a medical reason for your ataxia symptoms.

The most common idiopathic ataxia is called multiple system atrophy, or MSA. Doctors haven’t pinned down possible causes for this group of ataxias. They may come from a combination of environmental factors and genetic causes.

Ataxia Diagnosis

To diagnose ataxia, your doctor may use a combination of your medical history, family history, exams, and testing.

Physical exam

In a physical exam, your doctor may check your balance and coordination, hearing, vision, reflexes, and memory.

Neurological exam and tests

A neurological exam may include checking your reflexes, coordination, speech, and other factors that can show how your brain and nervous system are functioning. Your doctor may also order an MRI or CT scan, imaging tests that look at the structure of your brain for problems.

Spinal fluid test

Your doctor may recommend tests of your spinal fluid. To do this, they’ll insert a needle into your lower back and draw out the fluid, and then send it to a lab for testing.

Genetic testing

Genetic blood tests can detect the mutations responsible for some types of inherited ataxia, but not all of them. Genetic tests can also help your doctor see if a genetic change is causing a condition that has led to your ataxia.

Continued

Ataxia Treatment

The best treatment for your ataxia symptoms depends on the type you have. There is no specific treatment for ataxia itself. If your ataxia is a symptom of another disorder or condition, your doctor will treat that disorder or condition.

If it’s due to a cause that you can avoid, like lack of vitamins or exposure to poison, your doctor will help you address the problem causing the ataxia.

If the cause of your ataxia is an underlying condition that can’t be cured or treated, your doctor can help you manage your symptoms. This may include medication and certain types of therapy.

Medication

Medication can help address symptoms such as:

  • Lack of balance and coordination
  • Vertigo or dizziness
  • Muscle cramps or spasms
  • Tremors

Speech therapy

A speech therapist can help address slurred speech and problems swallowing. For example, they can teach you exercises that strengthen the muscles you use when speaking, and they can help you use breathing techniques to improve your speech.

Other types of therapy

To help you manage your symptoms, your doctor may also recommend:

  • Counseling
  • Physical therapy, to improve coordination and mobility
  • Occupational therapy, to help with daily tasks like feeding yourself
  • Speech therapy
  • Support groups

Ataxia Complications

Complications of ataxia vary depending on the type you have. They may include:

  • Dizziness
  • Spasticity or rigidity (different types of stiffened muscles)
  • Tremor
  • Pain
  • Fatigue
  • Low blood pressure when sitting or standing
  • Bowel, bladder, or sexual dysfunction

Sometimes, ataxia can lead to breathing problems, which may need to be managed with continuous positive airway pressure devices (CPAP) or tracheostomy, a procedure that involves cutting a hole in the front of your neck and inserting a tube for breathing.

Also, if you fall or become confined to a bed because of your ataxia, that can lead to other problems, such as injury and pressure sores.

Living With Ataxia

Exercise can help with coordination and balance, but you should talk to your doctor about what exercise is right for you before you start any exercise program. Physical therapy may include learning exercises to help manage your ataxia.

Continued

Ataxia can lead to depression or anxiety, so seeing a counselor or therapist or joining a support group may help.

If you have ataxia, you may want to avoid alcohol and recreational drugs because they can make your ataxia worse.

Adaptive devices

Some devices can help you manage your ataxia by helping you move around and do daily tasks more easily. They include:

  • Wheelchairs, walkers, or hiking sticks to improve mobility
  • Modified utensils to help you eat more easily
  • Communication devices, such as voice amplifiers so others can hear you better
  • Alternative computer keyboards and mice 

When to Call the Doctor

Ataxia symptoms can overlap with symptoms of many other conditions. You should see your doctor if you have any of the following:

  • Balance or coordination problems
  • Slurred speech
  • Wide gait
  • Problems with tasks such as writing
  • Slow eye movements

Takeaways

Many different types of ataxia exist. Each has different causes and symptoms, but trouble with balance and coordination is a hallmark. If you have symptoms of ataxia, you should see your doctor since your ataxia may be a sign of a more serious problem.

Ataxia FAQs

What is ataxic gait?

If you have ataxic gait, your walk is staggering or clumsy, with your legs wide apart. You may struggle to walk in a straight line.

What is ataxia 3?

Ataxia 3, or SCA3, is a type of spinocerebellar ataxia that is also called Machado-Joseph disease. You might have a staggering walk, clumsiness in your arms and legs, trouble with speech and swallowing, and impaired eye movements.

What are the different types of ataxia in MS?

If you have multiple sclerosis, the types of ataxia you might have include cerebellar ataxia, sensory ataxia, and vestibular ataxia. 

What is ataxia type 7?

Type 7 ataxia, or SCA7, is a type of spinocerebellar ataxia that often involves vision problems and sometimes blindness.

What is the episodic ataxia type 3 gene?

Episodic ataxia type 3 is very rare, but researchers have connected it to the KCNA1 gene.

WebMD Medical Reference

Sources

Photo Credit: Thomas O. Crawford / Wikimedia Commons

SOURCES:

National Ataxia Foundation: “Causes of Ataxia.”

University of Minnesota: “About Ataxia.”

Baylor College of Medicine: “Ataxia.”

National Organization for Rare Disorders: “Autosomal Dominant Hereditary Ataxia.”

Johns Hopkins Medicine: “What is Ataxia?”

Dartmouth Medical School: “Disorders of the Nervous System.”

Mayo Clinic: “Ataxia.”

National Library of Medicine – Photo Caption

National Organization for Rare Diseases – Photo Caption

American Society of Cancer Oncology – Photo Caption

Beyond Celiac: “Gluten Ataxia and Celiac Disease.”

Cleveland Clinic: “Ataxia,” “Spasticity,” “Episodic ataxia,” “Neurological exam.”

Hospital for Special Surgery: “Telangiectasia and Autoimmune Disease.”

Johns Hopkins Medicine: “Ataxia,” “Ataxia Digest.”

Mayo Clinic: “Celiac disease,” “Tracheostomy,” “Ataxia.”

Medline Plus: “Tremor.”

National Ataxia Foundation: “11 Exercises for Ataxia Patients.”

National Ataxia Foundation: “What Is Ataxia?”

National Institute of Neurological Disorders and Stroke: “Spinocerebellar Ataxias including Machado-Joseph Disease.”

National Library of Medicine, MedGen: “Truncal ataxia.”

National Organization for Rare Disorders: “Spinocerebellar ataxia 7.”

New York-Presbyterian: “Ataxia.”

NHS (UK): “Ataxia.”

Radiopaedia: “Ataxia (clinical sign).”

Stanford Medicine: "Gait Abnormalities.”

StatPearls: “Apraxia.”

Ataxia - Practice Essentials and Interventions, Edited by Patricia Bozzetto Ambrosi: “Ataxia in Multiple Sclerosis: From Current Understanding to Therapy.”

Handbook of Clinical Neurology: “Chapter 30 - Spinocerebellar ataxia type 7,” “Chapter 17 - Cerebellar ataxia.”

Brain: “Primary episodic ataxias: diagnosis, pathogenesis and treatment.”

Continuum: “Ataxia.” 

Frontiers in Neurology: “The Diagnostic Accuracy of Truncal Ataxia and HINTS as Cardinal Signs for Acute Vestibular Syndrome.”

© WebMD, LLC. All rights reserved.

Pagination

091e9c5e813ea7dd091e9c5e822761fbnl-ctr-responsivemodule_nl-ctr-responsive_091e9c5e813ea7dd.xmlwbmd_pb_sharedmodule091e9c5e801c9c6f0144010/13/2021 12:58:100HTML