NMDA R Encephalitis Management; To be Aggressive or Cautious, Investigating a Standardized Treatment Regimen

Background: NMDA Encephalitis does not have a standardized treatment protocol and it varies from provider to provider.

Objectives: We present two cases of NMDA encephalitis that received two different treatments and their response and outcomes.

Methods: Patients are presented; Patient A: 20 year old right-handed woman admitted for progressive encephalitis and myelopathy. Workup revealed + NMDA consistent with encephalitis, treated with 5 days IV methylprednisolone, aborted IVIG after 400 mg due to the development of angioedema, plasmapheresis for 5 exchanges every other day, then initiation of rituximab 2 g.

Patient B: 39 year old left-handed woman with subacute presentation of fatigue and progressive encephalopathy, discovered to have NMDA R encephalitis, s/p 5 days IV methylpred, 2 g/kg IVIG, rituximab 2g and cyclophosphamide 1g.

Results: Patient A had a mRS of 5 one month after disease onset. Patient B had a mRS of 2 one month after disease onset. The patient that received the combination therapy of Rituximab and cyclophosphamide significantly improved while the patient that received solely Rituximab, albeit with addition of PLEX after aborted IVIG had a more severe outcome and a protracted recovery period.

Conclusions: Although it is important to be cautious when using immunosuppression as there are many potential adverse effects, NMDA R encephalitis is an aggressive disease entity and aggressive therapy is warranted.