International Journal of Epilepsy 2016; 03(01): 42-62
DOI: 10.1016/j.ijep.2015.12.037
Thieme Medical and Scientific Publishers Private Ltd. 2017

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

MS S Fernando
1   Department of Paediatric Neurology, Teaching Hospital Anuradhapura, Sri Lanka
GID KS Dharmaratne
2   Institute of Neurology, National Hospital, Colombo, Sri Lanka
R Gamage
2   Institute of Neurology, National Hospital, Colombo, Sri Lanka
› Author Affiliations

Subject Editor:
Further Information

Publication History

Publication Date:
12 May 2018 (online)

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.