Investigators at King Abdulaziz University, Jeddah, Saudi Arabia, report their experience with IV pulse methylprednisolone in the treatment of children with severe drug-resistant epilepsy. Patients with infantile spasms, progressive degenerative, or metabolic disorders were excluded. Of 17 children aged 2-14 (mean 5.3) years, 88% had daily seizures and 13 (76%) had been admitted previously with status epilepticus. Cognitive and motor deficits were recognized in 82%. The epilepsy was cryptogenic in 47% and seizures were mixed in 41% (Lennox Gastaut in 4 (23%) and Doose syndrome in 2 (12%)). EEG showed focal or multifocal epileptiform discharges in 7 (41%) and generalized epileptiform discharges in 10 (59%). IV methylprednisolone 15 mg/kg/day, divided every 6 hours for 3 days was followed by oral prednisolone at 1-1.2 mg/kg/day once am for 1 week, then weaned slowly over 2 to 8 weeks (mean 3 wks). After follow-up for 6-24 months (mean 18), 6 (35%) became completely seizure free but 3 relapsed later, and 10 (59%) were improved. Those with mixed seizures were more likely to have a favorable response than those with one seizure type. No major side effects were noted, and 35% had improved alertness and appetite. [1]

COMMENTARY. A trial of add-on steroid therapy may be effective in children with intractable seizures of mixed type, apart from those with infantile spasms. Multiple antiepileptic medications were ineffective; the ketogenic diet was unavailable in this center and had not been tried.

Of 314 children enrolled in the Far-East Asia Catastrophic Epilepsy (FACE) study group, age of onset of epilepsy was <12 months in 239 cases (80%), epileptic spasms were the most frequent seizure type (in 42%), followed by generalized tonic seizures (in 20%) [2]. Epileptic syndromes included West syndrome (in 37%), unclassified (21%), Lennox-Gastaut (12%), Dravet (4%), and Rasmussen (2%). Cortical dysplasia and chromosomal anomalies were the two most frequent causes of epilepsy, in 16% and 6%, respectively; in almost one half of patients, the cause was unknown. Psychomotor development was retarded in 62% cases.