Neuronal Networks Group, Neuropharmacology and Neurobiology, School of Clinical and Experimental Medicine, University of Birmingham, Birmingham, United Kingdom, Department of Pediatric Neurology, 2
nd Faculty of Medicine, Charles University in Prague, University Hospital Motol, Prague, Department of Neurology, 2
nd Faculty of Medicine, Charles University in Prague, University Hospital Motol, Prague, Department of Circuit Theory, Faculty of Electrical Engineering, Czech Technical University, Prague, Czech Republic
Sturge-Weber syndrome is a neurocutaneous disorder classically characterized by the presence of facial port-wine stain and ipsilateral leptomeningeal angiomatosis. It is often associated with refractory epilepsy which requires surgical treatment. We present a case of a patient who initially presented with partial seizures of temporo-occipital origin, ipsilateral to the pial angiomatosis. During the course of the disease, the patient developed medically refractory epilepsy with partial seizures originating predominantly from the contralateral temporo-occipital area as well as myoclonic and myoclonic-astatic seizures. Resection of the occipital and temporal lobe affected by the pial angioma resulted in favourable outcome. Bilateral dysfunction observed in Sturge-Weber syndrome may result in an increased capability of focal discharges to generate synchronous epileptiform activity leading to an increased incidence of generalised seizures, most probably via a mechanism of secondary bilateral synchrony.
[Published with video sequences]