Severe Multi-organ dysfunction secondary to Shigella flexneri encephalopathy (Ekiri Syndrome): a case report and review of the literature.

Shigella infection is a major global cause of diarrhea, particularly among children in low- and middle-income countries. While most cases are self-limited, certain strains such as Shigella flexneri can lead to severe systemic complications, including encephalopathy. Among the rarest and most fatal forms is Ekiri syndrome, primarily reported in East Asian literature, characterized by fulminant neurotoxicity, high fever, seizures, and rapid progression to coma or death. In this study, we report the case of a previously healthy 14-year-old girl who presented with vomiting, diarrhea, and low-grade fever, initially treated conservatively. Her condition worsened with progressive lethargy and a generalized seizure, leading to ICU admission. Brain imaging showed diffuse cerebral edema, and her temperature intermittently spiked to 44-45 °C. Laboratory findings revealed marked leukopenia, and stool culture confirmed Shigella flexneri. Despite supportive management including anticonvulsants, empiric antibiotics, and antivirals for suspected CNS infection, her neurologic status continued to deteriorate. As per family request, she was referred to a tertiary center, where her condition rapidly worsened. She developed multi-organ failure with acute kidney injury requiring dialysis, elevated liver enzymes, cardiac dysfunction, and pneumothorax. Despite intensive care, she succumbed to complications after nearly ten days of hospitalization. This case illustrates a fulminant presentation of Shigella-associated encephalopathy consistent with Ekiri syndrome. Clinicians should maintain a high index of suspicion in pediatric patients with gastrointestinal and early neurological symptoms. Early recognition and aggressive supportive care are essential to improving outcomes in this rare but devastating condition.