Intracranial hemorrhage as the initial presentation of Sturge–Weber syndrome: a case report and literature review

Abstract

Background Sturge–Weber syndrome (SWS) is a rare congenital neurocutaneous disorder. Timely diagnosis and management remain challenging, particularly when intracranial hemorrhage (IH) occurs as a rare but serious complication. Case presentation A young child developed IH after minor head trauma and presented with irritability, vomiting, gait instability, and lethargy. His symptoms progressed to recurrent focal motor seizures, status epilepticus, and hemiplegia. Initial CT showed a hematoma in the left frontal lobe, but SWS was not recognized immediately. Gadolinium-enhanced magnetic resonance imaging showed multiple gyriform enhancing linear shadows and leptomeningeal enhancement along the cortex extending into the sulci, consistent with pial angiomatosis. The diagnosis of SWS was established on the basis of the extensive port-wine stain (PWS), seizures, and neuroimaging findings. Intravenous midazolam relieved the seizures and restored consciousness, followed by sequential oral levetiracetam and valproic acid therapy. During follow-up, he remained seizure-free for more than two years while receiving anticonvulsants and low-dose aspirin, with only mild right hemiparesis. Conclusion IH may be the first recognized neurological manifestation of previously undiagnosed SWS. In children with facial PWS and acute neurological symptoms, prompt neuroimaging may facilitate diagnosis and guide individualized management.