March 2014 Issue

Gluten Ataxia
By David Yeager
Today’s Dietitian
Vol. 16 No. 3 P. 16

Research suggests that gluten may cause this rare but serious medical condition.

Dietitians are familiar with the fact that celiac disease is characterized by a permanent intolerance to the protein gluten. But many are less familiar with research that suggests that being gluten intolerant is also associated with a mysterious neurological condition called gluten ataxia.

Ataxia, apart from its association with gluten, is a condition that affects the brain, causing problems with balance and coordination. Some patients with sporadic ataxia develop a progressive condition that tends to occur later in life and often is linked to gait abnormalities and problems with coordination. If the ataxia is idiopathic, it’s believed that an immune response to gluten may be the cause. But gluten ataxia is extremely difficult to diagnose because currently there are no tests that can positively identify it.

“There are no unique clinical symptoms that would distinguish gluten ataxia from other types of ataxia,” says Armin Alaedini, PhD, an assistant professor in the department of medicine at Columbia University Medical Center who specializes in autoimmune disorders. “Patients usually present with gradual onset of gait ataxia, which is sometimes accompanied by symptoms of peripheral neuropathy. The term ‘gluten ataxia’ generally is used to refer to cases of cerebellar ataxia of unknown cause, also known as sporadic ataxia, which is associated with increased levels of gluten antibodies [also known as] antigliadin antibodies.”

To further complicate matters, while some studies have suggested that as many as 20% of people with sporadic ataxia may test positive for antigliadin antibodies, up to 15% of people with no ataxia symptoms also may test positive for antigliadin antibodies, suggesting that there are additional mechanisms at work in the development of ataxia, says Brent L. Fogel, MD, PhD, an assistant professor in the neurology department at the UCLA David Geffen School of Medicine. Just as a patient who tests positive for antigliadin antibodies may or may not have celiac disease, testing positive does not necessarily mean the patient has ataxia. In addition, there are many patients with sporadic ataxia who have no gluten sensitivity. Many of the patients Fogel sees at UCLA are older than 50, have gait difficulties, and may have mild neuropathy. “This describes about 70% of the people who walk into my clinic,” he says. “So it’s really hard to say, based on someone’s symptoms, that gluten ataxia is responsible.”

Fogel says he routinely screens for antigliadin antibodies but rarely encounters cases of suspected gluten ataxia, which may be just a reflection of the patients seen at the UCLA clinic.

Looking Deeper
To further study the association between gluten and ataxia, researchers and neurologists at Columbia University and UCLA have begun a research trial that will assess the prevalence of gluten sensitivity in a population of US patients with sporadic ataxia compared with a control group. Currently, more than 80 patients have signed up, and recruitment will last a few more months, Alaedini says.

Once the study begins, it will take about one year to perform the analyses. While it’s difficult to say with certainty how often gluten sensitivity causes ataxia, it may be more common than clinicians realize. “Gluten sensitivity is one of the most common causes of sporadic ataxia with no family history [to explain it] and, unfortunately, remains underdiagnosed,” says Marios Hadjivassiliou, MD, a professor of neurology and the neuroinflammation research theme lead at Sheffield Teaching Hospitals NHS Foundation Trust at Sheffield University in the United Kingdom. “There’s an urgent need to increase awareness of this type of neurological manifestation of sensitivity to gluten because the damage to the balance center can be irreversible with time.”

Hadjivassiliou is the physician who discovered and named gluten ataxia and the lead author of a study that found gluten to be the single biggest cause of sporadic idiopathic ataxia.1 Patients with sporadic idiopathic ataxia were significantly more likely to test positive for antigliadin antibodies than patients whose ataxia could be explained by other causes.1,2 Although the antibodies more often are associated with celiac disease, patients rarely exhibit gastrointestinal symptoms.1,2 Hadjivassiliou recommends that any patient with sporadic idiopathic ataxia be screened for antigliadin antibodies.

Unfortunately, if a patient is diagnosed with gluten ataxia, there are few treatment options. Alaedini says immunomodulatory therapy with IV immunoglobulin and a gluten-free diet have been found to be promising in some studies. Typically, it takes six months to one year before physicians can determine the diet’s efficacy, Fogel says. If patients haven’t shown any improvement in symptoms within that time frame, it’s unlikely that gluten ataxia is the cause, he says.

Eating Gluten Free
Because adhering to a gluten-free diet can be challenging for patients, it’s important for physicians to work closely with dietitians. Hadjivassiliou says good nutrition advice is the key to treating gluten ataxia. RDs provide crucial support for patients who are transitioning to a gluten-free diet.

“It’s unfortunately one of the hardest diets for people to stay on, particularly if they’re used to the typical American diet,” Fogel says. “It’s difficult to get somebody to switch over to gluten free and stay on it without the assistance of a dietitian to help them do it in a palatable way. People aren’t going to be successful on their own. We wouldn’t put somebody on that type of a diet without getting them involved with a professional who can help them stay on it.”

To ease the transition, Marlisa Brown, MS, RD, CDE, CDN, a consultant, a chef, and the author of Gluten-Free, Hassle Free and Easy Gluten-Free, begins by asking patients about their dining habits. Cooking at home, although challenging, offers more control over the diet than eating out. Brown helps patients modify favorite recipes and suggests gluten-free foods that are similar to what they already enjoy so that the change isn’t too abrupt.

For those who eat out more frequently, the biggest challenge is the possibility of cross-contamination, Brown says. Even something as basic as butter can be problematic depending on how it’s handled. Brown provides her clients with “safe/not safe” lists and “question foods” lists, and she recommends clients question anything they’re unsure about. She also offers names of eating establishments that have good reputations for serving gluten-free fare. Even with her support, clients still have some difficulty sticking to a gluten-free diet.

“I try to focus on things that are going to make them happier. The only thing that sometimes upsets them is when I talk about the fact that they can’t consume any gluten at all,” Brown says. “That’s the hardest part, that they can’t even have a crumb. Somebody can put out a cheese platter but, if there are crackers on that platter, you can’t even have the cheese.”

Educating patients about what to avoid also extends to food shopping. Rachel Begun, MS, RDN, a food and nutrition consultant and expert in gluten-related disorders, says clients must become avid label readers to successfully eliminate gluten. Gluten is used in a wide range of products, so it’s important for dietitians to educate clients about how to detect gluten in packaged foods, she says.

The three grains that contain gluten are wheat, rye, and barley, but only wheat is required to be highlighted on package labels because it’s also a significant allergen. Begun says the Food Allergen Labeling and Consumer Protection Act requires that significant allergens be highlighted on packaging. Yet, because an allergic reaction is different from an autoimmune reaction, rye, barley, and gluten aren’t required to be highlighted. Gluten-free labeling is voluntary.

In addition, clients need to be careful about oats, Begun says. Oats don’t contain gluten, but often they’re contaminated with gluten during harvesting, transportation, and manufacturing.

“My recommendation for anybody on a gluten-free diet is to focus the diet on naturally gluten-free foods because those are many of the foods that we promote for a healthful diet anyway, such as fruits and vegetables; low-fat dairy; lean cuts of meat; plant-based proteins such as beans, nuts, and seeds; and, obviously, gluten-free whole grains,” Begun says. “Certified gluten-free products provide an extra layer of protection to insure they’re gluten free and there hasn’t been any cross-contamination.”

— David Yeager is a freelance writer and editor based in Royersford, Pennsylvania.

 

References
1. Hadjivassiliou M, Grünewald RA, Sharrack B, et al. Gluten ataxia in perspective: epidemiology, genetic susceptibility and clinical characteristics. Brain. 2003;126(3):685-691.

2. Hadjivassiliou M, Grünewald RA, Chattopadhyay AK, et al. Clinical, radiological, neurophysiological, and neuropathological characteristics of gluten ataxia. Lancet. 1998;352(9140):1582-1585.