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

Overview

Journal Nutrients

Date 2020 Apr 5

PMID 32244870

Citations 13

Authors

Maxine D Rouvroye

Panagiotis Zis

Anne-Marie Van Dam

Annemieke J M Rozemuller

Gerd Bouma

Marios Hadjivassiliou

Affiliations

Soon will be listed here.

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 the neurological dysfunction is not yet clear. The aim of this review is 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 in cerebrum of patients with encephalopathy and patients with epilepsy. Such changes were not seen in skin biopsies from patients with small fiber neuropathies. The findings from this systematic review suggest an immune mediated pathogenesis for GRND. Future research should focus on the characterization of the inflammatory cell infiltrates and identifying target epitopes.

Citing Articles

Cerebellar degeneration in gluten ataxia is linked to microglial activation.

Floare M, Wharton S, Simpson J, Aeschlimann D, Hoggard N, Hadjivassiliou M Brain Commun. 2024; 6(2):fcae078.

PMID: 38510211 PMC: 10953628. DOI: 10.1093/braincomms/fcae078.

Unraveling the Immunopathological Landscape of Celiac Disease: A Comprehensive Review.

Patt Y, Lahat A, David P, Patt C, Eyade R, Sharif K Int J Mol Sci. 2023; 24(20).

PMID: 37895160 PMC: 10607730. DOI: 10.3390/ijms242015482.

Cerebellar presence of immune cells in patients with neuro-coeliac disease.

Rouvroye M, Bontkes H, Bol J, Lissenberg-Witte B, Byrnes V, Bennani F Acta Neuropathol Commun. 2023; 11(1):51.

PMID: 36966322 PMC: 10040112. DOI: 10.1186/s40478-023-01538-5.

Celiac Disease and Neurological Manifestations: From Gluten to Neuroinflammation.

Giuffre M, Gazzin S, Zoratti C, Llido J, Lanza G, Tiribelli C Int J Mol Sci. 2022; 23(24).

PMID: 36555205 PMC: 9779232. DOI: 10.3390/ijms232415564.

Review article: Systemic consequences of coeliac disease.

Laurikka P, Kivela L, Kurppa K, Kaukinen K Aliment Pharmacol Ther. 2022; 56 Suppl 1:S64-S72.

PMID: 35815828 PMC: 9543231. DOI: 10.1111/apt.16912.

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