Treatment of absence status in the Lennox-Gastaut syndrome with propofol

Absence status epilepticus is the most common form of nonconvulsive status, and occurs with some frequency in Lennox-Gastaut syndrome.¹ Clinical features include a fluctuating confusional state associated with a continuous generalized electroencephalographic epileptiform abnormality.² Therapeutic options currently are limited to the administration of intravenous benzodiazepine; some reports have noted effectiveness of long-acting agents such as valproic acid in the routine treatment of absence seizures.³ We report a patient with Lennox-Gastaut syndrome and prolonged atypical absence status epilepticus refractory to administration of parenteral benzodiazepine who responded clinically and electroencephalographically to a bolus and subsequent brief continuous infusion of intravenous propofol.

Case report. The patient is an 11-year-old boy with mental retardation and Lennox-Gastaut syndrome. He was born at 37 weeks' gestation following a pregnancy complicated by intrauterine growth retardation. Developmental delay and a congenital left hemiparesis were noted early in life and he developed seizures at the age of 10 months. Over time he experienced a multiplicity of seizure types including atypical absences, atonic seizures, generalized tonic clonic seizures, and myoclonic attacks. Medications used in attempts to control his seizures have included …