Celiac Disease, Beyond the Bowel: A Review of Its Neurological Manifestations.

Saawan C Patel,Mridul Soni,Ibrahim Sange,Pranay K Joshi,Idan Grossmann,Diana I Zamora,Gautami S Patel,Devarashetty Shreya,Kevin Rodriguez

doi:10.7759/cureus.20112

Abstract

Celiac disease (CD) is a multi-systemic autoimmune condition that causes a hyperinflammatory response when gluten is ingested. There has been a shift in the clinical presentation of CD from a mere malabsorption disorder to an autoimmune condition that affects multiple organ systems, which could increase the rate of hospitalizations and a decreased quality of life. This article has compiled various studies that have explored the neurological manifestations of celiac disease, their epidemiology, possible pathogenic mechanisms, diagnosis, and treatment. The most common neurological conditions include gluten ataxia (GA), gluten neuropathy, gluten encephalopathy, and epilepsy which usually present as sporadic diseases which are difficult to diagnose in the absence of gastrointestinal (GI) symptoms. The treatment for most of these conditions is a gluten-free diet (GFD) regardless of GI involvement.

Highlights

BackgroundCeliac disease (CD), previously known as celiac sprue, is an autoimmune condition in which genetically predisposed individuals develop an immunologic reaction to ingested gluten, a protein found in barley, wheat, and rye, destroying the intestinal villi [1]
The results demonstrate the effectiveness of a gluten-free diet (GFD) for the treatment of gluten ataxia (GA) even in the absence of detectable enteropathy (Table 1) [22]
It was found that six patients with CD showed signs of chronic axonal neuropathy compared to only one patient with reflux disease

Summary

Introduction

Celiac disease (CD), previously known as celiac sprue, is an autoimmune condition in which genetically predisposed individuals develop an immunologic reaction to ingested gluten, a protein found in barley, wheat, and rye, destroying the intestinal villi [1]. It is a condition that is quite commonly underdiagnosed due to its variable clinical presentations, wide age group, and unclear pathogenesis. A protein found in gluten, is the major pathogenic component in CD [6]. It is deamidated by tissue transglutaminase (tTG), making it available for consumption by antigen-presenting cells (APCs). This, in turn, leads to T-cell mediated hypersensitivity reaction (type 4) and a humoural response resulting in histologic changes in the small intestine, such as lymphocytes in the lamina propria, crypt hyperplasia, and blunting of the intestinal villi (Figure 1) [2]

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