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A pediatric case of gluten sensitivity with severe neurological presentation
R Jorge1, C Aguiar, C Espinheira
1Department of Pediatrics, São João Hospital Centre, Alameda Prof. Hernâni Monteiro, 4200 - 319, Porto, Portugal, ritamilheiro@hotmail.com.
Insights
Gluten sensitivity can cause severe neurological issues in children, even without digestive symptoms. A gluten-free diet effectively resolved symptoms and halted brain lesion progression in a young girl.
Area of Science:
- Pediatric Neurology
- Autoimmune Disorders
- Gastroenterology
Background:
- Neurologic manifestations are recognized in adult celiac disease patients, but are considered rare in children.
- Gluten sensitivity can present with neurological symptoms, sometimes preceding gastrointestinal issues.
Observation:
- A 3-year-old girl presented with severe neurological symptoms and white matter lesions on MRI, mimicking acute disseminated encephalomyelitis.
- The patient had no gastrointestinal symptoms, but elevated IgA anti-transglutaminase (TG) 2 antibodies and duodenal biopsy confirmed celiac disease.
- Recurrent neurological symptoms and lesions prompted consideration of an autoimmune/inflammatory systemic disease.
Findings:
- A gluten-free diet led to complete resolution of the child's neurological manifestations.
- The gluten-free diet also halted the progression of white matter lesions observed on cerebral MRI.
Implications:
- Testing for anti-TG2 autoantibodies is recommended for children with unexplained neurological issues of probable autoimmune origin to identify potential gluten sensitivity.
- A gluten-free diet is the primary and effective treatment for gluten sensitivity, regardless of symptom presentation.
- Further research is needed on pediatric neurological manifestations of gluten sensitivity and their response to dietary intervention.
Unlabelled:
Neurologic manifestations are present in a reasonable proportion of adult celiac patients and can be the first sign of gluten sensitivity. In children, the risk of neurological complications is lower, and gluten sensitivity with neurological presentation seems to be rare. We report a case of gluten sensitivity with severe neurological presentation in a 3-year-old girl. In the absence of gastrointestinal symptoms, the presence of white matter lesions in cerebral magnetic resonance imaging suggested an acute disseminated encephalomyelitis. Recurrence of neurologic symptoms and cerebral lesions over a long period of time made the authors consider an autoimmune/inflammatory systemic disease. IgA anti-transglutaminase (TG) 2 was elevated and duodenal biopsy confirmed the presence of enteropathy. A gluten-free diet led to complete resolution of neurological manifestations and arrest of white matter lesions progression.
Conclusion:
In children with unclear neurologic manifestations with probable autoimmune etiology, anti-TG2 autoantibody titers should be determined considering the possibility of gluten sensitivity. Gluten-free diet remains the only effective treatment reported to date and, therefore, should be recommended to all patients with gluten sensitivity despite the type of manifestations. More studies, focusing on neurological manifestations and its response to gluten-free diet, are needed.
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