Seizures, severe encephalopathy and brain edema caused by shigella sonnei infection in cystic fibrosis

Seizures and encephalopathy in children with cystic fibrosis (CF) may be secondary to an electrolyte imbalance such as hyponatremia or hypocalcemia, liver failure, intracranial hemorrhage due to vitamin K malabsorption, diabetic ketoacidosis, cyclosporin A toxicity after lung transplantation and other causes not related directly to the CF. We describe a 2.5-year-old boy with CF and acute Shigella sonnei gastroenteritis accompanied by seizures and severe encephalopathy. Lumbar puncture revealed an elevated pressure of 290 mmH₂O, and computed tomography demonstrated severe brain edema. After antimicrobial and supportive therapy, the child showed complete recovery. The diagnosis was Ekiri syndrome. A review of the literature did not disclose a high incidence of shigellosis in patients with CF, and our report is the first to describe Ekiri syndrome encephalopathy in a child with CF.