Therapeutic hypothermia for refractory status epilepticus in a child with malignant migrating partial seizures of infancy and SCN1A mutation: a case report.
Journal: 2014/July - Therapeutic hypothermia and temperature management
ISSN: 2153-7933
Abstract:
Status epilepticus (SE) is a common indication for neurocritical care and can be refractory to standard measures. Refractory SE (RSE) is associated with high morbidity and mortality. Unconventional therapies may be utilized in certain cases, including therapeutic hypothermia (TH), bumetanide, and the ketogenic diet. However, the literature describing the use of such therapies in RSE is limited. Details of a case of TH for RSE in an infant with malignant migrating partial seizures of infancy were obtained from the medical record. A 4-month-old child developed SE that was refractory to treatment with concurrent midazolam, phenobarbital, fosphenytoin, topiramate, levetiracetam, folinic acid, and pyridoxal-5-phosphate. This led to progressive implementation of three unconventional therapies: TH, bumetanide, and the ketogentic diet. Electrographic seizures ceased for the entirety of a 43-hour period of TH with a target rectal temperature of 33.0°C–34.0°C. No adverse effects of hypothermia were noted other than a single episode of asymptomatic hypokalemia. Seizures recurred 10 hours after rewarming was begun and did not abate with reinstitution of hypothermia. No effect was seen with administration of bumetanide. Seizures were controlled long-term within 48 hours of institution of the ketogenic diet. TH and the ketogenic diet may be effective for treating RSE in children.
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Ther Hypothermia Temp Manag 2(3): 144-149

Therapeutic Hypothermia for Refractory Status Epilepticus in a Child with Malignant Migrating Partial Seizures of Infancy and <em>SCN1A</em> Mutation: A Case Report

Department of Critical Care Medicine, University of Pittsburgh, Pittsburgh, Pennsylvania.
Safar Center for Resuscitation Research, University of Pittsburgh, Pittsburgh, Pennsylvania.
Department of Neurology, Children's Hospital of Pittsburgh of UPMC, Pittsburgh, Pennsylvania.
Department of Pediatrics, Children's Hospital of Pittsburgh of UPMC, Pittsburgh, Pennsylvania.
Department of Neurological Surgery, Children's Hospital of Pittsburgh of UPMC, Pittsburgh, Pennsylvania.
Corresponding author.
Address correspondence to: Michael J. Bell, M.D., Department of Critical Care Medicine, 4401 Penn Avenue, Faculty Pavilion - Second Floor, Pittsburgh, PA 15224. E-mail:ude.cmpu@4jmlleb
Address correspondence to: Michael J. Bell, M.D., Department of Critical Care Medicine, 4401 Penn Avenue, Faculty Pavilion - Second Floor, Pittsburgh, PA 15224. E-mail:ude.cmpu@4jmlleb

Abstract

Status epilepticus (SE) is a common indication for neurocritical care and can be refractory to standard measures. Refractory SE (RSE) is associated with high morbidity and mortality. Unconventional therapies may be utilized in certain cases, including therapeutic hypothermia (TH), bumetanide, and the ketogenic diet. However, the literature describing the use of such therapies in RSE is limited. Details of a case of TH for RSE in an infant with malignant migrating partial seizures of infancy were obtained from the medical record. A 4-month-old child developed SE that was refractory to treatment with concurrent midazolam, phenobarbital, fosphenytoin, topiramate, levetiracetam, folinic acid, and pyridoxal-5-phosphate. This led to progressive implementation of three unconventional therapies: TH, bumetanide, and the ketogentic diet. Electrographic seizures ceased for the entirety of a 43-hour period of TH with a target rectal temperature of 33.0°C–34.0°C. No adverse effects of hypothermia were noted other than a single episode of asymptomatic hypokalemia. Seizures recurred 10 hours after rewarming was begun and did not abate with reinstitution of hypothermia. No effect was seen with administration of bumetanide. Seizures were controlled long-term within 48 hours of institution of the ketogenic diet. TH and the ketogenic diet may be effective for treating RSE in children.

Abstract
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