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Nabbout and FIREs

P Kurbatova, F Wendling, A Kaminska, A Rosati, R Nabbout, R Guerrini, O Dulac, G Pons, C Cornu, P Nony, C Chiron, P Benquet
Abnormal reemergence of depolarizing GABAA current during postnatal brain maturation may play a major role in paediatric epilepsies, Dravet syndrome (DS) being among the most severe. To study the impact of depolarizing GABA onto distinct patterns of EEG activity, we extended a neural mass model as follows: one sub-population of pyramidal cells was added as well as two sub-populations of interacting interneurons, perisomatic-projecting interneurons (basket-like) with fast synaptic kinetics GABAA (fast, I1) and dendritic-projecting interneurons with slow synaptic kinetics GABAA (slow, I2)...
September 2016: Experimental Neurology
Carlos A Pardo, Rima Nabbout, Aristea S Galanopoulou
The mechanisms of epileptogenesis in pediatric epileptic syndromes are diverse, and may involve disturbances of neurodevelopmental trajectories, synaptic homeostasis, and cortical connectivity, which may occur during brain development, early infancy, or childhood. Although genetic or structural/metabolic factors are frequently associated with age-specific epileptic syndromes, such as infantile spasms and West syndrome, other syndromes may be determined by the effect of immunopathogenic mechanisms or energy-dependent processes in response to environmental challenges, such as infections or fever in normally-developed children during early or late childhood...
April 2014: Neurotherapeutics: the Journal of the American Society for Experimental NeuroTherapeutics
Rima Nabbout
Idiopathic hemiconvulsion hemiplegia and epilepsy syndrome (IHHE) and febrile infection-related epilepsy syndrome (FIRES) are rare epileptic syndromes characterized by the occurrence of status epilepticus in a previously healthy child during or closely after a febrile episode. In both syndromes, there is no evidence of central nervous system infection (encephalitis) and the etiology remains unclear. Treatment is disappointing, particularly in FIRES, except for a response to ketogenic diet (KD) in half of patients...
September 2013: Epilepsia
Rima Nabbout
The role of immunity and inflammation in epilepsy have long been suggested by the anticonvulsant activity of steroids in some infancy and childhood epilepsies. The role of fever and infection in exacerbating seizures due to possible proinflammatory molecules, the increased frequency of seizures in systemic autoimmune diseases like systemic lupus erythematous, and, recently, the detection of autoantibodies in some unexplained epilepsies reinforced the causal place of immunity and inflammation in epilepsies with unknown etiology...
September 2012: Epilepsia
Uri Kramer, Ching-Shiang Chi, Kuang-Lin Lin, Nicola Specchio, Mustafa Sahin, Heather Olson, Rima Nabbout, Gerhard Kluger, Jainn-Jim Lin, Andreas van Baalen
PURPOSE: To explore the correlations between treatment modalities and selected disease parameters with outcome in febrile infection-related epilepsy syndrome (FIRES), a catastrophic epileptic encephalopathy with a yet undefined etiology. METHODS: We conducted a retrospective multicenter study on children who had been included in eight studies published between November 2001 and July 2010. Additional data were retrieved from six of the eight participating centers...
November 2011: Epilepsia
Rima Nabbout, Annamaria Vezzani, Olivier Dulac, Catherine Chiron
Fever-induced refractory epileptic encephalopathy in school-aged children (FIRES), and idiopathic hemiconvulsion-hemiplegia syndrome (IHHS) are both triggered by fever, although evidence for a causal microorganism or an autoimmune phenomenon is lacking. FIRES begins in school age with status epilepticus lasting several weeks, involves perisylvian areas including mesial temporal structures, and is followed by pharmacoresistant epilepsy with major cognitive deterioration. IHHS begins in infancy with unilateral clonic status epilepticus and is followed by hemiplegia with pharmacoresistant epilepsy...
January 2011: Lancet Neurology
Rima Nabbout, Michel Mazzuca, Philippe Hubert, Sylviane Peudennier, Catherine Allaire, Vincent Flurin, Marina Aberastury, Walter Silva, Olivier Dulac
PURPOSE: Fever induced refractory epileptic encephalopathy in school age children (FIRES) is a devastating condition initiated by prolonged perisylvian refractory status epilepticus (SE) triggered by fever of unknown cause. SE may last more than 1 month, and this condition may evolve into pharmacoresistant epilepsy associated with severe cognitive impairment. We aimed to report the effect of ketogenic diet (KD) in this condition. METHODS: Over the last 12 years we collected data of nine patients with FIRES who received a 4:1 ratio of fat to combined protein and carbohydrate KD...
October 2010: Epilepsia
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