J Korean Child Neurol Soc.  2006 Nov;14(2):342-347.

A Case of The Successful Treatment of Pentobarbital for Posthypoxic Action Myoclonus(Lance-Adams Syndrome) with Refractory Status Epilepticus

• Kim DH
• Kim SJ
• Oh KI
• Kwon YS
• Son BK

Abstract

Lance-Adams syndrome is a rare complications of cardiorespiratory arrest. We report a 15-month-old child with Lance-Adams syndrome who fell into a refractory status epilepticus after a successful resuscitation after the cardiopulmonary arrest from upper airway obstruction due to the croup. On the day of the admission, he went through a generalized tonic-clonic seizure with poor mental status. His condition became aggravated on 18th day of admission when he developed focal myoclonus in his left arm and leg. The EEG findings before the pentobarbital treatment show partial electrical seizure. The seizures were intractable despite the administration of midazolam, phenobarbital, phenytoin, and valproic acid. Therefore, a pentobarbital(PTB) therapy was required. PTB administered by a continuous infusion pump at a loading dose of 5 mg/kg was sufficient to produce a burst suppression pattern and a seizure control, followed by a maintenance infusion of 0.5-1.5 mg/kg/hour. At that time, the brain MRI showed a diffuse distribution of high signal intensity and swelling in both basal ganglia, thalamus and the temporo-occipital area. The therapy continued for 10 days. Thereafter, PTB was gradually tapered after a minimum 48-hours of a seizure-free status. So we report a case with a brief review of related literature.

Keyword

Pentobarbital therapy; Lance-Adams syndrome; Refractory status epilepticus

MeSH Terms

Airway Obstruction
Arm
Basal Ganglia
Brain
Child
Croup
Electroencephalography
Heart Arrest
Humans
Infant
Infusion Pumps
Leg
Magnetic Resonance Imaging
Midazolam
Myoclonus
Pentobarbital*
Phenobarbital
Phenytoin
Resuscitation
Seizures
Status Epilepticus*
Thalamus
Valproic Acid
Midazolam
Pentobarbital
Phenobarbital
Phenytoin
Valproic Acid