Fatal acute fulminant cerebral edema complicating Kawasaki disease in an infant: a case report

Background: The neurological complications of Kawasaki disease (KD) are rare and typically benign. However, recent reports have described severe presentations including acute fulminant cerebral edema (AFCE), which may be associated with cerebral vasculitis. Case presentation: A previously healthy 23-month-old girl was diagnosed with KD on day five of illness. Initial treatment with intravenous immunoglobulin (IVIG) and aspirin led to temporary resolution of fever. On day 14, symptoms recurred, and a second dose of IVIG was administered. On day 16, the patient developed generalized seizures refractory to midazolam that required fosphenytoin administration. Brain MRI performed 2 h after seizure onset revealed hyperintensity in the deep white matter on T2-weighted and fluid-attenuated inversion recovery imaging, isointensity on diffusion-weighted imaging (DWI), and high signal intensity on apparent diffusion coefficient (ADC) maps. The bilateral temporal lobes showed DWI hyperintensity and low ADC signals. Cerebrospinal fluid (CSF) analysis revealed markedly elevated protein levels (445 mg/dL) without pleocytosis or hypoglycorrhachia. The patient received intensive care with mechanical ventilation, continuous sedation, and supportive therapy. Approximately 12 h after seizure onset, the patient developed sudden bradycardia and bilateral fixed dilated pupils. Brain CT revealed extensive cerebral edema. Despite intensive care, she developed brain herniation and died two months after the onset of KD. Conclusion: AFCE is a rare but life-threatening complication of KD. MRI findings suggesting vasogenic edema and markedly elevated CSF protein levels may serve as early warnings. Recognition of these findings may allow timely diagnosis and appropriate management of this devastating condition.