The Neuropathology of Gluten-Related Neurological Disorders: A Systematic Review

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Main idea: The neuropathological findings in gluten-related neurological disorders are widespread and not limited by the cerebellum in the brain. Information on the nature of the chronic inflammation (e.g. lymphocytic infiltration) is still unclear and requires further research.

Abstract: Gluten-related neurological disorders (GRND) represent a spectrum of neurological manifestations that are triggered by gluten. In coeliac disease, a T-cell mediated enteropathy is triggered by gluten in genetically predisposed individuals. The underlying pathological mechanism of neurological dysfunction is not yet clear. This review aims to collate existing neuropathological findings in GRND as a means of aiding the understanding of the pathophysiology. A systematic search of the Pubmed Database yielded 188 articles, of which 32 were included, containing 98 eligible cases with a description of pathological findings in GRND.In gluten ataxia, loss of Purkinje cells, atrophy, gliosis, and astrocytosis were apparent, as well as diffuse lymphocytic infiltration and perivascular cuffing with lymphocytes. In patients with large-fiber neuropathy, nerve biopsies revealed axonopathy, loss of myelinated fibers, and focal and perivascular infiltration by inflammatory cells. Inflammatory infiltrate was also observed in muscle in myopathy and cerebrum of patients with encephalopathy and patients with epilepsy. Such changes were not seen in skin biopsies from patients with small fibre neuropathies. The findings from this systematic review suggest immune-mediated pathogenesis for GRND. Future research should focus on the characterization of the inflammatory cell infiltrates and identifying target epitopes.

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