John Libbey Eurotext

Epileptic Disorders

The Educational Journal of the

Sturge-Weber syndrome: a favourable surgical outcome in a case with contralateral seizure onset and myoclonic-astatic seizures Volume 13, numéro 1, Mars 2011

Vidéos

  • Sturge-Weber syndrome: a favourable surgical outcome in a case with contralateral seizure onset and myoclonic-astatic seizures
  • Sturge-Weber syndrome: a favourable surgical outcome in a case with contralateral seizure onset and myoclonic-astatic seizures

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Auteurs
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
  • Mots-clés : Sturge-Weber syndrome, myoclonic-astatic seizures, contralateral seizure onset, epilepsy surgery
  • DOI : 10.1684/epd.2011.0407
  • Page(s) : 76-81
  • Année de parution : 2011

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]