Ketamine Infusion for Super Refractory Status Epilepticus in Alternating Hemiplegia of Childhood

AbstractAlternating hemiplegia of childhood (AHC) is a rare neurodevelopmental disorder and characterized by infantile onset hemiplegia involving either side of the body and other paroxysmal spells, including epilepsy. The N-methyl-D-aspartate (NMDA) receptor noncompetitive antagonist ketamine has been proved to be effective terminating status epilepticus (SE) in animal models and human case studies. Less than 50 cases, those use ketamine, have been reported in the management of pediatric SE, but its effectiveness is unknown in patients with AHC. The author reported two patients with molecularly confirmed AHC who presented with super refractory status epilepticus. These two patients failed multiple antiepileptic drugs, including midazolam infusion and propofol but responded promptly to ketamine infusion. This is the first description of the effectiveness of ketamine as an effective third-line agent for super refractory SE associated with AHC. Increased glutamate-mediated neurotoxicity can be present in AHC, not only due to overexpression of glutamate receptors during status epilepticus but also due to unique characteristics of AHC-preferential inactivity of GABAergic interneuron and impaired action of glutamate transporters (EAAT-1). Moreover, as neuroregression is possible after SE in AHC, earlier termination of SE by using ketamine can be an effective option in this population. Further prospective, controlled studies are needed to confirm the finding of this study.