Gabriela Carrera BARRIGA, Margaret FREE
The Journal of Pediatric Academy - 2026;7(3):144-148
Kawasaki disease (KD) is an acute medium-vessel vasculitis that predominantly affects young children but poses significant diagnostic challenges in infants, who often present with atypical or incomplete features. Neurologic involvement, including aseptic meningitis, is an uncommon but recognized manifestation that may delay diagnosis. We report a 7-week-old full-term male who presented with fever, irritability, respiratory distress, and cerebrospinal fluid (CSF) pleocytosis, concerning for infectious meningitis. Despite broad-spectrum antimicrobial therapy, blood, urine, and CSF cultures, as well as multiplex polymerase chain reaction (PCR) testing, were negative. Brain magnetic resonance imaging (MRI) demonstrated diffuse leptomeningeal enhancement, reinforcing the suspicion of central nervous system infection. Although he initially improved clinically with declining inflammatory markers, fever and systemic inflammation recurred during the second week of illness. With no infectious source identified, the patient underwent echocardiography, which revealed coronary artery ectasia with aneurysms, confirming atypical KD. Treatment with intravenous immunoglobulin, corticosteroids, and low-dose aspirin resulted in rapid defervescence and improvement in laboratory markers, with partial regression of coronary abnormalities. This case highlights critical diagnostic pitfalls in infantile KD, including misleading early improvement, aseptic meningitis as a presenting feature, and physiologic vascular enhancement on MRI mimicking leptomeningeal disease. Early consideration of KD is essential in infants with persistent or recurrent fever, even in the absence of classic criteria.
