Elsevier

Pediatric Neurology

Volume 31, Issue 2, August 2004, Pages 139-142
Pediatric Neurology

Case report
Celiac disease and childhood stroke

https://doi.org/10.1016/j.pediatrneurol.2004.02.014Get rights and content

Abstract

Celiac disease is associated with a diversity of central nervous system manifestations although an association with stroke has not been documented. This case report describes a child who presented with a recurrent transient hemiplegia. Magnetic resonance imaging of the brain confirmed infarction; transcranial Doppler studies and magnetic resonance angiography were abnormal. Although there were virtually no gastrointestinal symptoms and the child was thriving, celiac disease serology was strongly positive and a duodenal biopsy confirmed the disease. Tissue transglutaminase is the major autoantigen in celiac disease and is thought to maintain vascular endothelial integrity. Antiendomysial immunoglobulin A antibodies, demonstrated to be the same autoantibody as antitransglutaminase, react with cerebral vasculature, suggesting an autoimmune mechanism for celiac disease associated vasculopathy. Because celiac disease is a potentially treatable cause of cerebral vasculopathy, serology—specifically antitissue transglutaminase antibodies—should be included in the evaluation for cryptogenic stroke in childhood, even in the absence of typical gut symptoms.

Introduction

Stroke in childhood often has no identifiable underlying cause. In those children with idiopathic stroke, the risk of recurrence is difficult to predict and no intervention is of proven benefit in reducing that risk. Celiac disease is a common disorder in childhood, caused by an inappropriate immune response to dietary gluten. An association with cutaneous vasculitis, other autoimmune disorders, malignancy, and a diversity of neurologic disorders are widely reported in adults, but, in contrast to both Crohn's disease and ulcerative colitis, a link with childhood stroke has not previously been described. This study presents a child with stroke in whom celiac disease was also diagnosed, although no typical symptoms or signs of the disease were evident.

Tissue transglutaminase has recently been identified as the major autoantigen in celiac disease. We postulate a mechanism by which a disease-specific vasculopathy could arise in association with celiac disease. This association, if confirmed, is of particular significance as celiac disease is common, treatable, and not always associated with gastrointestinal symptoms.

Section snippets

Case report

A 3-year-old female presented with recurrent episodes of transient weakness of the left arm and unsteady gait, each episode lasting about 5 minutes. There was no systemic disturbance or change in conscious level. Five episodes occurred in 24 hours, and they continued to occur less frequently over the following 48 hours. Interim neurologic examinations were entirely normal. A further transient episode, lasting 4 hours, occurred 2 months later.

At the age of 2, the child had had recurrent

Discussion

Celiac disease is an autoimmune disease associated with intolerance to the gliadin component in dietary gluten [1]. Ongoing dietary exposure to gluten perpetuates an enteropathy and may give rise to malabsorption, with chronic diarrhea, weight loss, and abdominal distention. Misery and irritability are characteristic features in children. Antiendomysial, antitransglutaminase, antigliadin, and antireticulin immunoglobulin A antibodies are associated with celiac disease and are used both for

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