Gluten is a protein complex present in wheat, barley and most cereals. Although many people have no problem digesting this protein, many people have a sensitivity to gluten or a disease better known as celiac disease. People who have trouble digesting gluten can develop digestive problems and cause bowel damage, faint when they eat something containing gluten. In some cases, the body’s reaction to gluten can become quite serious. In these cases the immune system starts attacking the central nervous system, which can cause gluten ataxia. Gluten ataxia is an autoimmune disease in which antibodies produced against gluten fragments during digestion attack the brain tissue by mistake. In particular, antibodies attack an area of the brain called a cerebellum, responsible for activities such as balance, posture, coordination of movements and language. Recent studies have shown deposits of antibodies against the transglutaminase-2 (TGase-2) and transglutaminase-6 (TGase-6) enzymes on the blood vessels of the cerebellum, adding support to a blood-brain barrier dysfunction. Interestingly, gluten ataxia is usually not related to intestinal manifestations or vitamin deficiency and improvement with a gluten-free diet is possible.
Symptoms of gluten ataxia start mild and gradually worsen over time. If left untreated, the condition could lead to permanent damage. There is also evidence that people suffering from gluten ataxia will eventually show signs of atrophy to the cerebellum, the progressive narrowing of this tissue. Unfortunately, gluten ataxia is a relatively new discovery and not yet widely accepted by the entire medical community. Some researchers have estimated that up to 40% of all people with ataxia of unknown origin could potentially have gluten-related ataxia. Other studies have indicated much lower numbers. A review of the studies indicated a prevalence of about 23% in patients with unexplained ataxia.
This can make diagnosis and treatment difficult. However, there are groups of researchers committed to disseminating information on this rare condition. This means that the actual number of cases is difficult to determine. Because gluten ataxia is a progressive condition, symptoms can begin mildly and almost unnoticed, and gradually progress to becoming debilitating. Symptoms of gluten ataxia are similar to the symptoms of other ataxia conditions, which can make it difficult to get an accurate diagnosis. Although gluten ataxia is a gluten sensitivity problem, digestive problems are generally not a symptom. Some of the most typical symptoms that a person could try include:
• problems with general movements
• balance disorders
• problems with coordination
• loss of precise movement skills
• language difficulties
• paresthesia of hands, feet and limbs.
Progression over time
Gluten ataxia is a slowly progressive disease, very similar to other types of ataxia that also affect the cerebellum. This can make diagnosis or recognition difficult as a specific and treatable condition. It is not uncommon for a person’s general movement skills to be affected first, which is usually demonstrated by the difficulty of walking. A person is more likely to have an abnormal way of walking, constantly stumbling and underestimating the phenomenon by imputing it to distraction. As the disease progresses, a person may begin to notice vertigo, bad balance when standing up, difficulty speaking or writing. When a person first experiences these symptoms, they are likely to regress by eliminating gluten from their diet. If the condition is not interrupted, however, the worsening symptoms may become permanent.
A person is unlikely to have a specific diagnosis of gluten ataxia. Many doctors will not even test it because of other more recognized forms of ataxia. The methods used to diagnose the condition are also relatively new. The researchers recommend the use of techniques that doctors use to diagnose celiac disease, such as blood tests. A positive result indicates that a person should start a strictly gluten-free diet. If the symptoms improve, there is a strong chance that the person will have gluten ataxia. If you want to prevent the condition from recurring, the person should stick to the strictly gluten-free diet throughout life, or at least access flour and baked goods (bread, pasta, pastry, pizza) occasionally. This can be a problem for many, given the enormous cultural and emotional content that surrounds these foods.
Treatment is relatively simple and involves the total removal of all gluten from a person’s diet. Even small quantities can continue the progression of gluten ataxia. Symptoms may take time to improve, even after gluten removal from the diet. It is essential that a person checks the ingredients of all food products to avoid accidentally consume gluten. Not all doctors agree that the adoption of a gluten-free diet will improve the symptoms; as such, they may not recommend or mention the elimination of gluten from a person’s diet as a potential cure. However, anecdotal evidence suggests that people who remove gluten from their diet see improvements in their symptoms of ataxia.
- edited by Dr. Gianfrancesco Cormaci, PhD, specialist in Clinical Biochemistry.
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