What Is Gluten Ataxia?

Gluten ataxia is a rare neurological condition in which an autoimmune response to gluten can damage the part of the brain called the cerebellum. This can potentially cause problems with your gait and gross motor skills.

People with this condition may have loss of coordination, which in some cases may become significant.

This article looks at gluten ataxia, its causes, symptoms, and diagnosis. It also discusses what you can do to manage the condition.

What Is Gluten Ataxia?

Ataxia is a degenerative condition involving the nervous system. People with ataxia have problems with coordination and balance and may have slurred speech or problems with fine motor skills.

When you have gluten ataxia, your body produces antibodies as a response to gluten, a protein found in wheat, barley and rye. These antibodies mistakenly attack your cerebellum, the part of your brain responsible for balance, motor control, and muscle tone. This is what causes the ataxia.

This response is triggered when you eat gluten, Ataxia is not caused by a direct attack on the brain by the gluten protein itself.

How Rare Is Gluten Ataxia?

Because gluten ataxia is such a newly-defined condition, not all healthcare providers accept it yet. This makes it hard to know how many people might have the condition.

Dr. Marios Hadjivassiliou, a consultant neurologist at Sheffield Teaching Hospitals in the United Kingdom and the neurologist who first described gluten ataxia, says as many as 41% of all people with ataxia with no known cause might, in fact, have gluten ataxia.

Other estimates have placed those figures lower—somewhere in the range of 11.5% to 36%.

Gluten Ataxia Symptoms

Gluten ataxia symptoms are indistinguishable from symptoms of other forms of ataxia. If you have gluten ataxia, your symptoms may start out as mild balance problems. For example, you might be unsteady on your feet or have trouble moving your legs.

As symptoms progress, some people say they walk or even talk as if they're drunk. As the autoimmune damage to the cerebellum worsens, the eyes likely will become involved, potentially moving back and forth rapidly and involuntarily.

In addition, fine motor skills may suffer, making it more difficult for you to work writing instruments, zip zippers, or manipulate buttons on your clothing.

Complications

The resulting problems in balance and motor control eventually are irreversible due to brain damage.

Up to 60% of patients with gluten ataxia have evidence of cerebellar atrophy—literally, shrinkage of that part of the brain—when examined with magnetic resonance imaging (MRI) technology. In some people, an MRI also will reveal bright white spots on the brain that indicate damage.

Gluten Ataxia Diagnosis

Because gluten ataxia is a relatively new discovery, not all healthcare providers agree that it exists. For this reason, there hasn't been a universally accepted way to test for or diagnose it.

But that may be changing. In 2011, a group of top researchers in the field of celiac disease and non-celiac gluten sensitivity issued a consensus statement on how practitioners can diagnose all gluten-related conditions, including gluten ataxia.

Gluten ataxia only affects people who have celiac disease or gluten sensitivity. If you have celiac disease, it's important to be aware that this increases your risk of developing gluten ataxia.

If you have symptoms of gluten ataxia, your general practitioner can order specific blood tests that are used to help diagnose gluten sensitivity and celiac disease. These tests may include:

• IgA anti gliadin antibody (AGA)
• IgA anti tissue trans-glutaminase (Anti-TTG)
• Anti endomysial antibodies

These tests involve taking a small amount of blood and sending it to a lab for analysis. It's important not to adopt a gluten-free diet before taking one of these tests, as this could cause an inaccurate result. 

Unfortunately, these tests aren't sensitive enough to definitively confirm or rule out gluten ataxia. The most reliable way to diagnose the condition is to see if symptoms improve after you adopt a strict gluten-free diet.

Your healthcare provider may also refer you to a neurologist, who can help rule out other causes of ataxia, such as multisystem atrophy. Multisystem atrophy is distinguished from gluten ataxia because it also affects autonomic functions like digestion, breathing, and bladder control. 

Given the newness of this condition and the controversy over its acceptance as a valid diagnosis, however, it's possible that some healthcare practitioners may not consider it.

Gluten Ataxia Diet and Treatment

If you're diagnosed with gluten ataxia, you need to follow a very strict gluten-free diet.

There hasn't been a lot of research into the impact of a gluten-free diet on gluten ataxia, but a few smaller studies seem to point to its effectiveness. A 2017 study concluded that a gluten-free diet helped increase brain function in people with gluten ataxia.

Anecdotal reports from people with diagnosed gluten ataxia and from people with severe neurological problems and celiac disease also seem to support the effectiveness of a gluten-free diet in reversing gluten ataxia.

In the meantime, you may also be referred to a number of specialists for treatment of the ataxia symptoms themselves. This might include:

• A physical therapist 
• A speech-language pathologist
• A neurologist

Physical and occupational therapy can help you cope with symptoms. You may benefit from using an adaptive device such as a cane or walker. A specialist can also help you learn to prevent falls and other complications of ataxia such as choking or inhaling food.

Summary

Gluten ataxia is an autoimmune condition that causes your immune system to attack part of your brain as a response to gluten ingestion. People with this condition have balance and coordination problems.

There is some evidence that adopting a strict gluten-free diet may reverse gluten ataxia. In the meantime, other treatments like physical therapy may be helpful.

A Word From Verywell

The number of people with gluten ataxia is small compared to the number of people with celiac disease or gluten sensitivity. However, some people with celiac disease and gluten sensitivity also have other neurological symptoms, including gluten-related peripheral neuropathy and migraine.

It's possible that, as more studies are conducted on gluten ataxia, researchers will find even stronger links between neurological problems, celiac disease, and gluten sensitivity.

In the meantime, if you have symptoms similar to those of gluten ataxia, talk to your healthcare provider. You may require testing to determine if you have another condition that can cause similar symptoms.