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Nevertheless, inadvertent exposure to gluten is the main cause of persistent villous atrophy, and must be ruled out before a diagnosis of refractory disease is made. [114] People with poor basic education and understanding of gluten-free diet often believe that they are strictly following the diet, but are making regular errors.
Nevertheless, in some cases, a gluten challenge with a subsequent biopsy may be useful to support the diagnosis, for example in people with a high suspicion for coeliac disease, without a biopsy confirmation, who have negative blood antibodies and are already on a gluten-free diet. [2] Gluten challenge is discouraged before the age of 5 years ...
This lack of recovery is mainly caused by inadvertent exposure to gluten. [10] [95] People with poor basic education and understanding of the gluten-free diet often believe that they are strictly following the diet, but are making regular errors.
A strict gluten-free diet is the first-line treatment, which should be started as soon as possible. It is effective in most of these disorders. When dementia has progressed to an advanced degree, the diet has no beneficial effect. Cortical myoclonus appears to be treatment-resistant on both gluten-free diet and immunosuppression. [14]
This condition is known as refractory coeliac disease (RCD), defined as malabsorption due to gluten-related enteropathy (villous atrophy or elevated intraepitheal lymphocytes) after initial or subsequent failure of a strict gluten-free diet (usually 1 year) and after exclusion of any disorder mimicking coeliac disease.
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The specific carbohydrate diet (SCD) is a restrictive diet originally created to manage celiac disease; it limits the use of complex carbohydrates (disaccharides and polysaccharides). Monosaccharides are allowed, and various foods including fish, aged cheese and honey are included.
Early diagnosis and treatment with a gluten-free diet can improve ataxia and prevent its progression. The effectiveness of the treatment depends on the elapsed time from the onset of the ataxia until diagnosis, because the death of neurons in the cerebellum as a result of gluten exposure is irreversible. [29] [30]