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Neurological Manifestations of Neuropathy and Ataxia in Celiac Disease: A Systematic Review

IBM Watson Health, Cambridge, MA 02142, USA
Takeda Development Centre Europe Ltd., London WC2B 4AE, UK
Takeda Pharmaceuticals International Co, Cambridge, MA 02139, USA
Royal Hallamshire Hospital and University of Sheffield, Sheffield S10 2RX, UK
Department of Medicine, Celiac Disease Center, Columbia University Medical Center, New York, NY 10032, USA
Author to whom correspondence should be addressed.
These authors contributed equally to this work.
Nutrients 2019, 11(2), 380;
Received: 23 January 2019 / Revised: 6 February 2019 / Accepted: 7 February 2019 / Published: 12 February 2019
(This article belongs to the Special Issue Extraintestinal Manifestations of Coeliac Disease)
PDF [612 KB, uploaded 21 February 2019]
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Celiac disease (CD) is an immune-mediated gastrointestinal disorder driven by innate and adaptive immune responses to gluten. Patients with CD are at an increased risk of several neurological manifestations, frequently peripheral neuropathy and gluten ataxia. A systematic literature review of the most commonly reported neurological manifestations (neuropathy and ataxia) associated with CD was performed. MEDLINE, Embase, the Cochrane Library, and conference proceedings were systematically searched from January 2007 through September 2018. Included studies evaluated patients with CD with at least one neurological manifestation of interest and reported prevalence, and/or incidence, and/or clinical outcomes. Sixteen studies were included describing the risk of gluten neuropathy and/or gluten ataxia in patients with CD. Gluten neuropathy was a neurological manifestation in CD (up to 39%) in 13 studies. Nine studies reported a lower risk and/or prevalence of gluten ataxia with a range of 0%–6%. Adherence to a gluten-free diet appeared to improve symptoms of both neuropathy and ataxia. The prevalence of gluten neuropathy and gluten ataxia in patients with CD varied in reported studies, but the increased risk supports the need for physicians to consider CD in patients with ataxia and neurological manifestations of unknown etiology. View Full-Text
Keywords: celiac disease; gluten neuropathy; gluten ataxia; prevalence; incidence; gluten-free diet celiac disease; gluten neuropathy; gluten ataxia; prevalence; incidence; gluten-free diet

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Mearns, E.S.; Taylor, A.; Thomas Craig, K.J.; Puglielli, S.; Cichewicz, A.B.; Leffler, D.A.; Sanders, D.S.; Lebwohl, B.; Hadjivassiliou, M. Neurological Manifestations of Neuropathy and Ataxia in Celiac Disease: A Systematic Review. Nutrients 2019, 11, 380.

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