Abstract
Gluten ataxia (GA) is defined as sporadic ataxia with positive serological markers for gluten sensitivity and in the absence of an alternative etiology for the ataxia. It is the commonest immune-mediated ataxia and part of the spectrum of gluten-related disorders. GA may be associated with coeliac disease (gluten-sensitive enteropathy) but the presence of enteropathy is not a prerequisite for the diagnosis. A range of serological markers for gluten sensitivity and coeliac disease (CD) is available but it is important to appreciate the correct utility in the context of diagnosing GA. Some markers are specific to the presence of enteropathy, while others are sensitive to the whole spectrum of gluten-related disorders. GA responds well to a strict gluten-free diet with the best outcomes observed in patients where the diagnosis and treatment have been made early, before the loss of cerebellar reserve and permanent ataxia. The pathophysiology is closely linked to transglutaminases, a group of enzymes that are involved not only in tissue repair but also interact with gliadin, forming immunogenic peptides that trigger an autoimmune reaction leading to gut, skin, and neural tissue injury.
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Hadjivassiliou, M. (2023). Gluten Ataxia. In: Gruol, D.L., Koibuchi, N., Manto, M., Molinari, M., Schmahmann, J.D., Shen, Y. (eds) Essentials of Cerebellum and Cerebellar Disorders. Springer, Cham. https://doi.org/10.1007/978-3-031-15070-8_96
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