Sturge-Weber syndrome (SWS) is a rare neurocutaneous syndrome characterized by facial port-wine birthmarks and associated eye and meningeal angiomatosis. Its neurological manifestations include intellectual disability, seizures, and stroke-like episodes (SLEs), often misdiagnosed as stroke. We report a case of SWS presenting with left-sided weakness. Cranial computerized tomography revealed SWS-related venous anomalies, while digital cerebral angiogram confirmed proliferative angiopathy of the distal middle cerebral artery (MCA) with proximal MCA spasm, resolved by intra-arterial verapamil. Despite initial symptom resolution, electroencephalography (EEG) revealed electroclinical seizures. The patient was treated with anti-seizure medications (ASMs) and aspirin, achieving full recovery. SLEs in SWS can result from vascular malformations or postictal phenomena, with ischemia-related events often complicated by vasospasm and altered hemodynamics. Our case highlights the importance of multimodal imaging and video EEG in distinguishing SLE etiologies in SWS, which can guide timely intervention with ASMs and aspirin to reduce recurrence and improve prognosis.
Nazish et al. (Sun,) studied this question.