Neuropediatrics 2020; 51(03): 225-228
DOI: 10.1055/s-0039-3402005
Short Communication
Georg Thieme Verlag KG Stuttgart · New York

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

Debopam Samanta
1   Child Neurology Section, Department of Pediatrics, University of Arkansas for Medical Sciences, Little Rock, AR, United States
› Author Affiliations
Funding The author received no financial support for the research, authorship, and/or publication of this article.
Further Information

Publication History

03 July 2019

23 October 2019

Publication Date:
03 February 2020 (online)

Abstract

Alternating 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.

 
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