Atypical Sturge–Weber syndrome Type III without facial nevus presenting as acute focal neurological deficit in a child: a case report

Abstract We report the case of a 4-year-old female who presented with high-grade fever and acute left-sided weakness. Initial non-contrast CT suggested acute encephalitis, leading to a provisional diagnosis of partially treated meningoencephalitis and illustrating the potential for diagnostic misinterpretation. However, contrast-enhanced MRI revealed leptomeningeal enhancement, cortical atrophy, and dilated deep medullary veins, consistent with atypical Sturge–Weber syndrome Type III. The patient improved with supportive management. This case highlights the diagnostic challenge of atypical SWS and underscores the crucial role of contrast-enhanced MRI when CT findings are nonspecific or misleading.