Efficacy of add on corticosteroids in the management of pharmaco resistant epilepsy in Lennox Gastaut syndrome (LGS), multi center pilot study

Introduction: Lennox–Gastaut syndrome (LGS) is a childhood epileptic encephalopathy, which is notoriously difficult to treat. Children often end up on poly-pharmacy with numerous anticonvulsants, causing hazardous adversities. The aim was to investigate efficacy of corticosteroids in the management of pharmaco-resistant epilepsy in LGS.

Method: Single armed, open-labeled, efficacy trial with add on corticosteroids was performed over 24-weeks. Primary outcome parameter was seizure freedom, secondary outcome parameters were safety, improvement in behaviour and quality of life. Intravenous methyl-prednisolone was given 30 mg/kg over five days, followed by oral prednisolone 2 mg/kg for 9 days. Steroid was weaned gradually over six weeks. Twice weekly pulses of prednisolone (2 mg/kg) were commenced thereafter, 30 mg/kg intravenous methyl-prednisolone once in 6-weeks. Patients were monitored closely at regular intervals.

Results: Twenty one children, mean age 5.7 years (2.3–16.3 years), males – 11, fulfilled the inclusion criteria. All had daily seizures; axial/axio-rhizomelic tonic seizures (21), myoclonic (16), drop attacks (11), atypical-absences (5). Four dropped out on days 5, 8, 12 and 13 due to hospital-acquired-infection, maternal concerns, uncontrollable hypertension and severe urinary tract infection respectively. All who completed up to day-14 had >50% seizure reduction (seizure freedom in 12). Two relapsed between 18 and 24 weeks. Three had positive urine sugar, all had significant rise in BMI. Height-velocity and electrolytes remained unchanged. The quality of life and childhood behavior scores improved significantly (P < 0.05) by 24 weeks.

Conclusion: Majority achieved significant seizure control through steroids, with minimum adverse effects. The quality of life and behavior scores improved significantly. Suggest randomized-blinded-placebo-controlled study to confirm the findings.