Sturge-Weber syndrome Type I: a rare case report.

Introduction and importance: To document a rare case of Sturge-Weber syndrome (SWS) Type I with acute neurological symptoms. Case presentation: An 11-year-old boy, previously diagnosed with Sturge-Weber syndrome (SWS) Type I, presented to the emergency department with acute neurological symptoms that included vomiting, headaches, left-sided hemiparesis, and right-sided deviation of the labial commissure. Clinical discussion: Sturge-Weber syndrome (SWS) is a rare neurocutaneous disorder characterized by facial port-wine stains, leptomeningeal angiomas, and ocular involvement. Our case presented with preserved cognition despite extensive temporal lobe angiomatosis, contrasting the typical presentation of seizures and developmental delay. The patient then experienced stroke-like episodes from fragile leptomeningeal vasculature. With anticonvulsants and low-dose aspirin therapy, the patient achieved full neurological recovery within the first 3 months and maintained stability during 2 years of follow-up. Conclusion: This case underscores the diverse clinical spectrum of Sturge-Weber syndrome and emphasizes the crucial role of imaging in achieving an accurate diagnosis. Early identification and treatment are essential to prevent further complications and optimize patient outcomes.