Gluten sensitivity in sporadic and hereditary cerebellar ataxia

Gluten sensitivity, with or without classical celiac disease symptoms and i ntestinal pathology, has been suggested as a potentially treatable cause of sporadic cerebellar ataxia. Here, we investigated the prevalence of abnorm ally high serum immunoglobulin A (IgA) and IgG anti-gliadin antibody titers and typical human lymphocyte antigen (HLA) genotypes in 50 patients presen ting with cerebellar ataxia who were tested for molecularly characterized h ereditary ataxias. A high prevalence of gluten sensitivity was found in pat ients with sporadic (7/26; 27%) and autosomal dominant (9/24; 37%) ataxias, including patients with known ataxia genotypes indicating a hitherto unrec ognized association between hereditary ataxias and gluten sensitivity. Furt her studies are needed to determine whether gluten sensitivity contributes to cerebellar degeneration in patients with hereditary cerebellar ataxia. P atients with hereditary ataxia (including asymptomatic patients with known ataxia genotype) should be considered for screening for gluten sensitivity and gluten-free diet trials.