OBJECTIVES – To analyse outcome of second line immunomodulatory treatment in anti-NMDAR encephalitis.

METHODS - A retrospective analysis of 13 children treated for anti-NMDAR encephalitis (August 2016 to April 2018). Clinical course, treatment regimens and outcomes were analysed.

RESULTS - Thirteen patients (9 females) were treated for anti-NMDAR encephalitis. Mean age at presentation was 5.8 years (range: 8 months to 12 years, SD 4.3 years). All but two patients had seizures; two had epilepsia partialis continua. Three had refractory status epilepticus (one super refractory). All had severe insomnia and classical involuntary movements.

All but three received IVIG (2g/kg) and IV methyl prednisolone pulse for five days concurrently as primary immune therapy. Three received only IV methyl prednisolone pulse and not IVIG. All patients received IV rituximab as second line immunotherapy. Three patients received plasmapheresis after IV Rituximab. Nine patients received IV pulse Cyclophosphomide monthly (two to four doses) after having partial recovery with IV Rituximab. Twelve patients recovered completely without any residual neurological or cognitive deficits. Four patients recovered completely with IV Rituximab alone as second line therapy; two with only IV methyl prednisolone and IV Rituximab alone (cost effective regimen). One patient improved substantially but still has verbal memory and cognitive deficits along with drug resistant focal epilepsy.

Conclusion: IV Rituximab is effective and safe second line therapy for severe anti-NMDAR encephalitis. Monthly pulse Cyclophosphomide is safe (in short term) and provides excellent outcome in severe patients who had not responded to other immune therapy options.