Successful surgical management of New Onset Refractory Status Epilepticus (NORSE) presenting with gelastic seizures in a 3year old girl

Publication date: Available online 22 May 2017 Source:Epilepsy & Behavior Case Reports Author(s): Ahmad Marashly, Sean Lew, Jennifer Koop Gelastic seizures (GS) are typically associated with hypothalamic hamartomas and present in childhood. However it is now known that GS can be found in focal epilepsies arising from other regions in the brain, including mesial and neocortical frontal, temporal and parietal regions. GS have rarely been described as the presenting manifestation of New Onset Refractory Status Epilepticus (NORSE). In this article we describe a previously healthy 3year old girl who presented with an explosive onset of GS that were refractory to multiple anti-epileptic medications. These seizures arose from the right frontal region. An extensive metabolic and immunological workup was negative. Her brain magnetic resonance imaging (MRI) was negative, however the Positron Emission Tomography (PET) scan showed a hypermetabolic region in the right frontal inferior gyrus. She underwent a depth electrode evaluation that revealed a widespread irritative zone involving the PET “lesion” as well as mesial and neocortical regions in the right frontal lobe. The seizure onset zone was also widespread and non-localizable, however the GS were associated with a clear ictal epileptiform activity arising from the depth of the superior frontal gyrus, which was not overlapping with PET hypermetabolic region. She underwent a right frontal lobectomy sparing the primary...
Source: Epilepsy and Behavior Case Reports - Category: Neurology Source Type: research