From Gut to Gray Matter: A Case Report of Posterior Reversible Encephalopathy Syndrome in a Pediatric Patient With Celiac Disease.

Abstract

Posterior reversible encephalopathy syndrome (PRES) is a rare neurological condition characterized by vasogenic edema, primarily affecting the posterior cerebral hemispheres. Although typically reversible with prompt treatment, PRES can lead to severe complications if not recognized early. This report presents an unusual and fatal case of PRES in a pediatric patient with celiac disease, a combination that is rarely documented in medical literature. A 9-year-old child with a history of celiac disease and dermatitis herpetiformis presented to the Emergency Room with a two-month history of vomiting and loose stools, accompanied by a recent 20-min seizure. Initial examination revealed pallor, emaciation, drowsiness, and a generalized rash. The patient was tachycardic, hypertensive (blood pressure 150/100 mmHg), and had an oxygen saturation of 65%. Neurological examination showed a glasgow coma scale (GCS) score of 10/15, increased muscle tone, and heightened reflexes. CT and MRI scans revealed intraparenchymal and subarachnoid hemorrhages. Despite aggressive management including intubation, antihypertensive therapy, anticonvulsants, and broad-spectrum antibiotics, the patient's condition deteriorated rapidly. Complications included pneumoperitoneum and eventual cardiac arrest, leading to the patient's death. This case highlights the potential for severe, life-threatening complications of PRES in pediatric patients with underlying autoimmune conditions such as celiac disease. It underscores the importance of considering PRES in the differential diagnosis for children with celiac disease presenting with neurological symptoms, even in the absence of typical radiological findings. The case also illustrates the need for further research into the relationship between celiac disease and PRES to improve outcomes in similar cases.