Abstract
A six-year-old girl with mental retardation and a history of West syndrome had recurrent episodes of complex partial seizure status epilepticus (CPSE), characterized by decreased consciousness, vacant eyes and head nodding since early childhood. Electroencephalograms during CPSE revealed bursts of rhythmic high-voltage θ waves with fronto-central predominance, and Tc-99m-ECD single-photon emission computed tomography showed increased perfusion in the left frontoparietal areas and the left basal ganglia. Continuous infusion of midazolam was required to abolish CPSE on several occasions. However, the seizures became midazolam dependent, and many antiepileptic drugs did not succeed in allowing midazolam discontinuation. Benzodiazepines such as clonazepam and clobazam also had no benefit. However, clorazepate showed dramatic effectiveness, and she could be weaned off from three months of midazolam infusion with seizure freedom. Clorazepate is known for its efficacy in partial seizures and in epilepsy cases with psychic disturbances. Clorazepate may be an alternative drug for CPSE that is resistant to other antiepileptic agents.
