An Emerging Cause of Treatable Encephalitis: A Case of Anti-NMDA receptor (NMDAR) Encephalitis

Abstract

Anti-NMDA Receptor encephalitis is the most common type of autoimmune limbic encephalitis known. It is characterised by sub-acute onset of psychiatric symptoms along with refractory epilepsy, orofacial and limb dyskinesia and dysautonomia. High clinical suspicion and appropriate antibody testing in cerebrospinal fluid (CSF) and serum is required for early diagnosis and initiation of immunotherapy. Delay in treatment may lead to significant permanent neuro-deficits as well as mortality. Here, we present a case of 20-year-old female who presented with hallmark clinical features and high titre NMDAR antibody positivity in serum and CSF. She failed to respond with first line immunosuppressant (combination of immunoglobulin and steroids) and had to be started on weekly Rituximab for a month with dramatic clinical response. She was continued on oral steroids for 6 months and Azathioprine for 1 year. USS Pelvis and MRI Pelvis did not reveal any ovarian teratoma even after two and half years of regular follow up with 6 monthly ultrasound scan of the pelvis. This case highlights that we should diagnose the illness early and initiates second line treatment like Rituximab in case of failure to respond to first line agents in initial ten days.

Keywords: NMDAR, Encephalitis, Rituximab, Teratoma, Seizures

Cite this Article
Praveen Kumar Yadav, Dipanwita Sen. An Emerging Cause of Treatable Encephalitis: A Case of Anti-NMDA Receptor (NMDAR) Encephalitis. Research and Reviews: Journal of Neuroscience. 2017; 7(3): 8–11p.