Objective: To screened a well-defined cohort of patients with cerebellar ataxia for the presence of gluten sensitivity.

Background: Cerebellar ataxia is a heterogeneous group of disorders which can be familial or sporadic. Sensitivity to gluten has been implicated in the pathogenesis of sporadic cerebellar ataxia. Since there is a lack of data on the prevalence of gluten sensitivity in patients with ataxia from Asian countries, we screened a well-defined cohort of patients with cerebellar ataxia for the presence of gluten sensitivity.

Methods: A cohort of 192 well-characterized patients with progressive cerebellar ataxia, familial or sporadic, were screened for presence of gluten sensitivity using IgA antigliadin Ab (AGA), IgG-AGA, IgA anti-transglutaminase 2 Ab (TG2), and IgA antitransglutaminase 6 Ab (TG6) using commercially available ELISA kits. The data on their genetic testing for spinocerebellar ataxia (SCA) 1, 2, 3, 12, Friedreich’s ataxia (FRDA), and brain imaging were reviewed.

Results: Of 192 patients, 99 and 77 had sporadic and familial cerebellar ataxia, respectively. Genetic mutation for SCA types 1, 2, 3, 12 and FRDA was confirmed in 76 (40%) patients. Forty-two (21.8%) patients had either one or more serological test positive for gluten sensitivity; 20 (10.4%) patients for IgA-AGA, one of 141 (0.71%) for anti-TG2 Ab and 23 of 186 (12.3%) for IgA anti-TG6 Ab. 20 of 32 (63%) seropositive patients had cerebellar atrophy on brain imaging. None of 10 patients out of 42, who agreed to undergo duodenal biopsy examination, had evidence of celiac disease. Frequency of IgA-AGA and TG6 were significantly higher in our cohort in comparison to the cohort of other ethnicities.

Conclusions: Forty-two (21.8%) patients had either one or more serological test positive for gluten sensitivity. This study suggests a role of gluten sensitivity in patients with cerebellar ataxia. Gluten sensitivity in patients with ataxia may be playing a causative role or atleast a disease modifier.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/gluten-sensitivity-in-progressive-cerebellar-ataxia-patients-from-india/