Gluten ataxia is a rare neurological autoimmune disorder that involves the body’s reaction to the gluten protein that can be found in rye, wheat, Spelt, Kamut, and barley. It is an idiopathic sporadic ataxia with positive serum antigliadin antibodies (IgA and/or IgG), even in the absence of duodenal enteropathy. It can cause irreversible damage to the cerebellum. When a person who is predisposed intakes gluten, an autoimmune reaction takes place and causes swelling and damage to the lining of the small intestine.
This gradually causes villous degeneration and makes it harder for the small intestine to absorb nutrients.
Gluten ataxia is similar to celiac disease as both disorders are caused by gluten and they both affect the intestines, more specifically the villi or microvilli. The only difference is that with gluten ataxia, the cerebellum is impacted instead of or in addition to the intestines.
A person with gluten ataxia may or may not suffer from celiac disease.
The damage caused to the cerebellum may result to problems with gross motor skills and gait which leads to problems with coordination and also possibly resulting in progressive disability in a few cases.
Gluten ataxia progresses slowly but if it not diagnosed and treated promptly, the disease may progress and cause irreversible brain damage and lifelong problems with balance, motor control, and speech. It can also cause shrinking and damage of the cerebellum.
Gluten ataxia has first been identified over a decade ago and since it is relatively new and some physicians do not agree that this disease actually exists, there is still no way to diagnose or test it.
2What Causes Gluten Ataxia?
As gluten ataxia is a relatively new disorder, not all doctors agree that gluten ataxia actually exists. There are also no studies that could find a connection between cerebellar ataxia and antigliadin antibodies.
Gluten ataxia forms part of disorders that have something to do with gluten sensitivity including dermatitis herpetiformis and celiac disease.
Gluten ataxia accounts for up to 40% of cases of idiopathic sporadic ataxia. A high prevalence of antigliadin antibody positivity has been noticed among individuals affected by sporadic ataxia.
Dr. Marios Hadjivassiliou, a neurologist who is practicing in the United Kingdom who first describe gluten ataxia and is the top researcher in the field of gluten ataxia, performed a study on 68 individuals with gluten ataxia and remarked that 78% of them carried one or both of the primary genes for celiac disease, HLA-DQ8, and HLA-DQ2. The rest of the group carried HLA-DQ1, which Dr. Hadjivassiliou has thought has something to do with neurological symptoms resulting from gluten ingestion.
Dr. Hadjivassiliou also says that there are about 41% of all ataxia patients with no known cause that may have gluten ataxia. Others have placed estimates figures around 11.5% to 36%.
New research suggests that transglutaminase 6, a new antibody, may be an indication for gluten ataxia too.
3What are the Symptoms of Gluten Ataxia?
The symptoms of gluten ataxia can range from progressive balance difficulties up to problems swallowing. These symptoms are similar to the symptoms of other types of ataxia making it much more difficult to give a proper diagnosis. Patients with gluten ataxia are generally diagnosed in their late 40s to early 50s but there are a few instances where the illness develops in teens or young children. Gluten ataxia equally affects both women and men.
The first few symptoms of gluten ataxia involve the gross motor skills making the patients extremely uncoordinated.
An estimated 60% of patients show evidence of “sensorimotor axonal neuropathy”, a nerve damage that can cause loss of sensation, sensations of tingling, and even pain in the extremities. These symptoms are normally mild, and they don’t necessarily contribute to the ataxia.
Here are the following symptoms of gluten ataxia based on medical studies:
- Progressive balance difficulties
- Swallowing problems
- Unsteadiness on the feet
- Double vision
- Difficulty in controlling the bladder
- Inability to control the power or the speed of a physical movement
- Poor coordination in physical movements
- Speech impediments or slurring of words
- Poor control of muscle movement
- Clumsiness with a tendency to make missteps or stumble
- Problem with fine motor skills
- Abdominal pain
- Tingling in extremities
- Lactose intolerance
- Nausea and Vomiting
- Weight loss
- Nerve damage to arms and legs
The symptoms may appear gradually or they may also appear suddenly by they won’t include digestive symptoms that could indicate celiac disease.
Gluten ataxia symptoms most of the time, cannot be distinguished from the symptoms of the other types of ataxia. An individual who has gluten ataxia has symptoms that begin with mild balance issues, like having trouble moving his/her legs and having unsteady feet.
As symptoms gradually progress, a few people remark that they may talk and walk like as if they are drunk. The eyes are likely to be affected, potentially moving back and forth involuntarily and rapidly, as the autoimmune damage to the cerebellum progresses.
In addition, the fine motor skills may also suffer, making it much harder to zip zippers, work with writing instruments, or even to manipulate buttons on the clothes.
4How is Gluten Ataxia diagnosed?
Since not all physicians or neurologist accept that gluten ataxia exists as a valid diagnosis or disease, not all doctors will test an individual for the condition even if they manifest symptoms of it. There has only been recent development on coming up with a consensus on ways to test for gluten ataxia. A lot of celiac experts and/or gastroenterologists believe that there is good evidence for celiacs to have neurologic disorders such as gluten ataxia.
There is really no straightforward diagnosis for gluten ataxia. There are no recognized medical tests to diagnose gluten ataxia. However, there have been various medical studies that have outlined the symptoms of gluten ataxia.
Gluten ataxia diagnosis makes use of certain blood tests used for celiac disease excluding the tests that are deemed to be the most accurate ones to test for celiac disease. A positive result shown on any of these tests pushes the physician to prescribe a gluten-free diet to the patient.
When an individual is diagnosed with celiac disease and symptoms of gluten ataxia suddenly occur, it is easier to diagnose and confirm gluten ataxia.
5How is Gluten Ataxia treated?
An early diagnosis and treatment with a strict gluten-free diet can help improve ataxia and hamper its progression although there has been variable responsiveness to gluten-free diet treatment.
According to Dr. Hadjivassiliou, the neurological symptoms brought about by gluten ingestion have been deemed to take longer to treat than the gastrointestinal symptoms, and they seem to be more sensitive to lower amounts of trace gluten in food intake. It might, therefore, be possible to cause more damage to oneself if an individual with gluten ataxia continues to intake little amounts of gluten.