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What Is Gluten Ataxia?

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Updated March 22, 2012

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What Is Gluten Ataxia?
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Gluten ataxia, an autoimmune neurological condition involving your body's reaction to the gluten protein found in wheat, barley and rye, can irreversibly damage the part of your brain called the cerebellum, according to practitioners who first identified the condition about a decade ago.

This damage potentially can cause problems with your gait and with your gross motor skills, resulting in loss of coordination and possibly leading to significant, progressive disability in some cases. However, because gluten ataxia is so relatively new, and not all physicians agree that it exists, there's as of yet no accepted way to test for it or to diagnose it.

But that may be changing: in early 2012, a group of top researchers in the field of celiac disease and gluten sensitivity issued a consensus statement on how practitioners can diagnose all gluten-related conditions, including gluten ataxia.

In Gluten Ataxia, Antibodies Attack the Cerebellum

When you have gluten ataxia, the antibodies your body produces in response to gluten ingestion mistakenly attack your cerebellum, the part of your brain responsible for balance, motor control and muscle tone. The condition is autoimmune in nature, which means it involves a mistaken attack by your own disease-fighting white blood cells, spurred on by gluten ingestion, as opposed to a direct attack on the brain by the gluten protein itself.

Left unchecked, this autoimmune attack usually progresses slowly, but 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 their brains — 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.

How Many People Suffer From Gluten Ataxia?

Because gluten ataxia is such a newly-defined condition and not all physicians accept it as of yet, it's not clear how many people might suffer from it.

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%.

Since ataxia itself is a fairly rare condition — affecting only 8.4 people out of every 100,000 in the U.S. — that means fewer still actually have gluten ataxia. However, the estimates of how many people with celiac disease and gluten sensitivity who have neurological symptoms are much higher.

More on the numbers: How Many People Have Gluten Ataxia?

Gluten Ataxia: Gluten-Induced Neurological Problems

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 — 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 your cerebellum progresses, your eyes likely will become involved, potentially moving back and forth rapidly and involuntarily.

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

More on symptoms: Gluten Ataxia Symptoms

Diagnosis Not Straightforward for Gluten Ataxia

Since not all physicians accept gluten ataxia as a valid diagnosis, not all doctors will test you for the condition if you show symptoms. In addition, experts in the field of gluten-induced disease only recently have developed a consensus on how to test for gluten ataxia.

Testing involves the use of specific celiac disease blood tests, although not the tests that are considered the most accurate to test for celiac disease. If any of those tests shows a positive result, then the physician should prescribe a strict gluten-free diet.

If ataxia symptoms stabilize or improve on the diet, then it's considered a strong indication that the ataxia was gluten-induced, according to the consensus statement.

More on testing and diagnosis: Gluten Ataxia Diagnosis

Gluten Ataxia Treatment Involves Strict Gluten-Free Diet

If you're diagnosed with gluten ataxia, you need to follow a very strict gluten-free diet with absolutely no cheating, according to Dr. Hadjivassiliou.

There's a reason for this: the neurological symptoms spurred by gluten ingestion seem to take longer to improve than the gastrointestinal symptoms, and seem to be more sensitive to lower amounts of trace gluten in your diet, Dr. Hadjivassiliou says. Therefore, it's possible that you might be doing more damage to yourself if you continue to ingest small amounts of gluten.

Of course, not all physicians agree with this assessment, or even necessarily with the advice to eat gluten-free if you have otherwise unexplained ataxia and high levels of gluten antibodies. However, it does seem to be backed up by anecdotal reports from people with diagnosed gluten ataxia and from people with severe neurological problems associated with celiac disease: Those people say the neurological symptoms take much longer to resolve, and some stabilize, but never improve.

More on treatment strategies: Gluten Ataxia Treatment

Bottom Line: More Research Needed on Gluten Ataxia

The number of potential gluten ataxia sufferers is very small when compared with the numbers of people with celiac disease, and it's also small when compared with estimates for how many people have gluten sensitivity.

However, many people with celiac disease and gluten sensitivity also suffer from neurological symptoms, which often include gluten-related peripheral neuropathy and migraine. Some also complain of balance problems that do seem to resolve once they go gluten-free.

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

Sources:

Bushara K. Neurologic presentation of celiac disease. Gastroenterology. 2005 Apr;128(4 Suppl 1):S92-7.

Fasano A. et al. Spectrum of gluten-related disorders: consensus on new nomenclature and classification. BMC Medicine. BMC Medicine 2012, 10:13 doi:10.1186/1741-7015-10-13. Published: 7 February 2012

Hadjivassiliou M. et al. Dietary Treatment of Gluten Ataxia. Journal of Neurology, Neurosurgery and Psychiatry. 2003;74:1221-1224.

Hadjivassiliou M. et al. Gluten sensitivity as a neurological illness. Journal of Neurology, Neurosurgery and Psychiatry. 2002;72:560-563 doi:10.1136/jnnp.72.5.560.

Hadjivassiliou M. et al. Gluten ataxia in perspective: epidemiology, genetic susceptibility and clinical characteristics. Brain. 2003 Mar;126(Pt 3):685-91.

Hadjivassiliou M. et al. Gluten Ataxia. The Cerebellum. 2008;7(3):494-8.

Rashtak S. et al. Serology of celiac disease in gluten-sensitive ataxia or neuropathy: role of deamidated gliadin antibody. Journal of Neuroimmunology. 2011 Jan;230(1-2):130-4. Epub 2010 Nov 6.

Zelnik N. et al. Range of neurologic disorders in patients with celiac disease. Pediatrics. 2004 Jun;113(6):1672-6.

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