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SummaryThe aim of this retrospective study of children affected by epileptic encephalopathy was to evaluate seizure frequency, electroencephalographic pattern and neuropsychological status, before and after intravenous methylprednisolone therapy. Eleven children with epileptic encephalopathy were administered one cycle of intravenous methylprednisolone (15-30 mg/kg/day for three consecutive days, once a month for four months) in addition to constant dosages of their regular antiepileptic drugs. The treatment resulted in statistically significant reductions of generalized slow spike-and-wave discharges (p<0.0028) and seizure frequency (p<0.013), which persisted even after methylprednisolone pulse therapy was stopped. A globally positive outcome was noted in 9/11 patients (81.8%). This methylprednisolone treatment regimen did not cause significant or persistent adverse effects. We suggest that children with epileptic encephalopathy without an underlying structural lesion could be the best candidates for intravenous methylprednisolone pulse therapy.

Epileptic encephalopathies are age-dependent conditions, typically drug resistant, characterized by severe cognitive impairment due to the epileptic activity itself. Corticosteroid therapy with adrenocorticotrophic hormone, hydrocortisone, prednisone or methylprednisolone is one of the therapeutic approaches tried in these children. Although the first use of steroids as antiepileptic drugs dates back to 1950, there is no wide agreement about the ideal treatment regimen in epileptic encephalopathies other than West syndrome; no evidence from class I and II studies are available at the moment. In this study, we present a literature review on steroid therapy in childhood epilepsy with special regard to effectiveness, tolerability and possible mechanisms of action.

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