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

Overview

Journal Nutrients

Date 2019 Feb 15

PMID 30759885

Citations 29

Authors

Elizabeth S Mearns

Aliki Taylor

Kelly J Thomas Craig

Stefanie Puglielli

Daniel A Leffler

David S Sanders

Benjamin Lebwohl

Marios Hadjivassiliou

Affiliations

Soon will be listed here.

Abstract

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.

Citing Articles

Paediatric gluten ataxia: a rare neurological presentation of gluten sensitivity in a toddler.

Rasool A, Power C BMJ Case Rep. 2025; 18(2).

PMID: 39947735 PMC: 11831055. DOI: 10.1136/bcr-2024-262288.

Coeliac disease: complications and comorbidities.

Ludvigsson J, Yao J, Lebwohl B, Green P, Yuan S, Leffler D Nat Rev Gastroenterol Hepatol. 2025; .

PMID: 39875649 DOI: 10.1038/s41575-024-01032-w.

Exploring the association between atrial fibrillation and celiac disease: a comprehensive review.

Subedi R, Soulat A, Rauf Butt S, Mohan A, Danish Butt M, Arwani S Ann Med Surg (Lond). 2024; 86(12):7155-7163.

PMID: 39649916 PMC: 11623827. DOI: 10.1097/MS9.0000000000002259.

Case Report: Ocular paroxysmal non-epileptic events as the presenting sign of celiac disease in children: a case series.

Monzani A, Genoni G, Papa A, Paggi N, Capra E, Brustia F Front Pediatr. 2024; 12:1450563.

PMID: 39539767 PMC: 11557330. DOI: 10.3389/fped.2024.1450563.

Associations between Celiac Disease, Extra-Gastrointestinal Manifestations, and Gluten-Free Diet: A Narrative Overview.

Santonicola A, Wieser H, Gizzi C, Soldaini C, Ciacci C Nutrients. 2024; 16(12).

PMID: 38931169 PMC: 11206979. DOI: 10.3390/nu16121814.

References

Rampertab S, Pooran N, Brar P, Singh P, Green P . Trends in the presentation of celiac disease. Am J Med. 2006; 119(4):355.e9-14. DOI: 10.1016/j.amjmed.2005.08.044. View

Muzaimi M, Thomas J, Palmer-Smith S, Rosser L, Harper P, Wiles C . Population based study of late onset cerebellar ataxia in south east Wales. J Neurol Neurosurg Psychiatry. 2004; 75(8):1129-34. PMC: 1739172. DOI: 10.1136/jnnp.2003.014662. View

Ludvigsson J, Leffler D, Bai J, Biagi F, Fasano A, Green P . The Oslo definitions for coeliac disease and related terms. Gut. 2012; 62(1):43-52. PMC: 3440559. DOI: 10.1136/gutjnl-2011-301346. View

Ihara M, Makino F, Sawada H, Mezaki T, Mizutani K, Nakase H . Gluten sensitivity in Japanese patients with adult-onset cerebellar ataxia. Intern Med. 2006; 45(3):135-40. DOI: 10.2169/internalmedicine.45.1351. View

Jericho H, Sansotta N, Guandalini S . Extraintestinal Manifestations of Celiac Disease: Effectiveness of the Gluten-Free Diet. J Pediatr Gastroenterol Nutr. 2017; 65(1):75-79. DOI: 10.1097/MPG.0000000000001420. View

Leffler D, Green P, Fasano A . Extraintestinal manifestations of coeliac disease. Nat Rev Gastroenterol Hepatol. 2015; 12(10):561-71. DOI: 10.1038/nrgastro.2015.131. View

Luostarinen L, Himanen S, Luostarinen M, Collin P, Pirttila T . Neuromuscular and sensory disturbances in patients with well treated coeliac disease. J Neurol Neurosurg Psychiatry. 2003; 74(4):490-4. PMC: 1738407. DOI: 10.1136/jnnp.74.4.490. View

Reilly N, Green P . Epidemiology and clinical presentations of celiac disease. Semin Immunopathol. 2012; 34(4):473-8. DOI: 10.1007/s00281-012-0311-2. View

Diaconu G, Burlea M, Grigore I, Anton D, Trandafir L . Celiac disease with neurologic manifestations in children. Rev Med Chir Soc Med Nat Iasi. 2014; 117(1):88-94. View

10.

Lionetti E, Catassi C . The Role of Environmental Factors in the Development of Celiac Disease: What Is New?. Diseases. 2017; 3(4):282-293. PMC: 5548256. DOI: 10.3390/diseases3040282. View

11.

Bushara K, Goebel S, Shill H, Goldfarb L, Hallett M . Gluten sensitivity in sporadic and hereditary cerebellar ataxia. Ann Neurol. 2001; 49(4):540-3. View

12.

Abele M, Burk K, Schols L, Schwartz S, Besenthal I, Dichgans J . The aetiology of sporadic adult-onset ataxia. Brain. 2002; 125(5):961-8. DOI: 10.1093/brain/awf107. View

13.

Hadjivassiliou M, Boscolo S, Tongiorgi E, Grunewald R, Sharrack B, Sanders D . Cerebellar ataxia as a possible organ-specific autoimmune disease. Mov Disord. 2008; 23(10):1370-7. DOI: 10.1002/mds.22129. View

14.

Moher D, Liberati A, Tetzlaff J, Altman D . Preferred reporting items for systematic reviews and meta-analyses: the PRISMA statement. Ann Intern Med. 2009; 151(4):264-9, W64. DOI: 10.7326/0003-4819-151-4-200908180-00135. View

15.

Tonutti E, Bizzaro N . Diagnosis and classification of celiac disease and gluten sensitivity. Autoimmun Rev. 2014; 13(4-5):472-6. DOI: 10.1016/j.autrev.2014.01.043. View

16.

Hadjivassiliou M . Immune-mediated acquired ataxias. Handb Clin Neurol. 2011; 103:189-99. DOI: 10.1016/B978-0-444-51892-7.00011-5. View

17.

Hadjivassiliou M, Rao D, Grinewald R, Aeschlimann D, Sarrigiannis P, Hoggard N . Neurological Dysfunction in Coeliac Disease and Non-Coeliac Gluten Sensitivity. Am J Gastroenterol. 2016; 111(4):561-7. PMC: 4854981. DOI: 10.1038/ajg.2015.434. View

18.

Green P, Lebwohl B, Greywoode R . Celiac disease. J Allergy Clin Immunol. 2015; 135(5):1099-106. DOI: 10.1016/j.jaci.2015.01.044. View

19.

Thawani S, Brannagan 3rd T, Lebwohl B, Mollazadegan K, Green P, Ludvigsson J . Type 1 Diabetes, Celiac Disease, and Neuropathy-A Nationwide Cohort Study. J Clin Neuromuscul Dis. 2017; 19(1):12-18. DOI: 10.1097/CND.0000000000000174. View

20.

Hadjivassiliou M, Gibson A, DAVIES-JONES G, Lobo A, Stephenson T . Does cryptic gluten sensitivity play a part in neurological illness?. Lancet. 1996; 347(8998):369-71. DOI: 10.1016/s0140-6736(96)90540-1. View