Pediatric State of the Art Symposium

Abstract

1 Ajay Gupta, and 2 Mohamad Mikati ( 1 Department of Neurology, Cleveland Clinic Foundation, Cleveland, OH ; and 2 Department of Neurology, American University of Beirut, Beirut, Lebanon )There is a unique, apparently heterogeneous, group of epilepsy syndromes in children that is characterized by sleep activation of the epileptiform abnormalities with nearly continuous sharp waves seen on the scalp EEG during sleep. These include commonly recognized childhood conditions such as Landau‐Kleffner syndrome (LKS), electrical status epilepticus in slow wave sleep (ESES), and benign rolandic epilepsy of childhood (BREC). Most of these children have a period of normal development and healthy childhood. Besides presenting with seizures that are often not too difficult to treat, these children have other major cognitive, behavioral and academic difficulties that are disproportionate to the burden of epilepsy and medications used to treat them. It is commonly believed that abnormal electrical activity in sleep per se leads to cognitive, behavioral, and academic difficulties in these children. However, the pathophysiological understanding of this phenomenon of sleep activation and its clinical implications are poorly understood. Moreover, there are also controversies regarding terminology, classification, and management of these disorders. Some experts believe that these conditions are distinct disease entities while others hypothesize that they may represent a spectrum of one condition. Similar sleep activation of epileptic discharges has also been reported in some other children with neuroimaging abnormalities in the deep gray nuclei. Some experts believe that the treatment with antiepileptic medications in these children should be titrated to the EEG response in sleep and not to the clinical response of seizures. It is unclear as to how often a sleep EEG should be done, and to what extent aggressive medical treatment and antiepileptic polytherapy be pursued. Use of steroids, recent success with high dose nightly benzodiazepine therapy, and surgical procedure like multiple subpial transactions in the insular regions continue to be controversial due to risk of serious side effects and modest benefits in reported anecdotal case reports and series. The focus of this symposium is to revisit these syndromes to: a) define the clinical spectrum of these conditions; b) formulate the best practices for management in the light of controversies in medical and surgical options; and c) discuss possible pathophysiological mechanisms underlying these disorders and probe future strategies for better treatment. Margret Shouse will show similarities between kindled epileptic kittens and sleep activation of EEG in children with these conditions. She will review the potential mechanisms and generators of EEG activity during sleep, discuss how the cognitive and behavioral sequelae may occur regardless of severe seizure burden. Edouard Hirsch will discuss the available clinical evidence to suggest that these “disorders may be a clinical spectrum of one condition” and not distinct disease entities. He will also discuss diagnostic criteria and role of neuroimaging. Jim Rivello will review the available evidence, and emphasize the best clinical practices for management in these children. First, he will discuss the goals of treatment i.e. seizure control, behavior improvement, cognition monitoring, and sleep EEG improvement. Second, he will formulate a treatment and follow‐up plan with indications for various medical (anti epileptic agents, steroids, nightly benzodiazepenes etc.) and surgical (multiple subpial transactions) options. Each speaker will also formulate an important research question for the future that will improve our understanding of these conditions for devising better treatment methods. Live interaction with the diverse group of audience will also provide a forum to discuss where and how we should move forward with a collaborative effort for advancing our knowledge and treatment of these conditions.