Pediatric anti-NMDA receptor encephalitis with catatonia: treatment with electroconvulsive therapy

Taha Moussa,Karyn Gerstle,Joseph Cooper,Khalid Afzal,Linda Wagner-Weiner,Ryan Rosenberger

doi:10.1186/s12969-019-0310-0

Abstract

BackgroundAnti-NMDA receptor encephalitis, an autoimmune disease associated with antibodies against N-methyl-D-aspartate (NMDA) receptors, is being diagnosed more frequently, especially in children and young adults. Acute neurological and psychiatric manifestations are the common presenting symptoms. Diagnosing anti-NMDA receptor encephalitis is often challenging given the wide range of clinical presentation, and may be further complicated by its overlap of symptoms, brain MRI changes, and CSF findings with other entities affecting the brain. Even though diagnosis can be made by identifying antibodies in immune-mediated encephalitis, the diagnosis may be delayed by weeks to months. Delay in initiation of treatment with immune suppressive therapies is shown to be associated with adverse outcomes. Malignant catatonia is a severe and life-threatening state associated with anti-NMDA receptor encephalitis. It is often inadequately assessed and may not respond to immunosuppressive treatment.Case presentationWe present a confirmed case of anti-NMDA receptor encephalitis in a 16 year old girl who had severe critical neurological and psychiatric manifestations, including malignant catatonia and autonomic instability. Our patient continued to manifest malignant catatonia despite the initiation of prompt, aggressive immune suppressive therapies, including corticosteroids, plasmapheresis, intravenous gammaglobulin and rituximab, as well as treatment with high-dose benzodiazepines. Once electroconvulsive therapy (ECT) began, she had a robust response with resolution of her catatonia. Six weeks after treatment with eight ECT cycles, she had returned to her normal baseline cognitive and motor function.ConclusionsECT was an effective and well-tolerated therapy in our patient, and should be considered for the treatment of children with anti-NMDA receptor encephalitis whose catatonia does not respond to immunosuppression and benzodiazepines.

Highlights

electroconvulsive therapy (ECT) was an effective and well-tolerated therapy in our patient, and should be considered for the treatment of children with anti-NMDA receptor encephalitis whose catatonia does not respond to immunosuppression and benzodiazepines
In 2007, antibodies against N-methyl-D-aspartate (NM DA) receptor were identified in the hippocampus and forebrain of 12 cases of paraneoplastic encephalitis leading to the discovery of anti-NMDA receptor encephalitis [1]
Over the course of her ECT treatments, her Bush-Francis Catatonia Rating Scale (BFCRS) improved from 27 to 2 (Fig. 1). We evaluated her mental status using the Clock Drawing Test (CDT) prior to ECT #3, ECT #5, and the day after ECT #8 (Fig. 2), showing marked improvement in Discussion Since its discovery in 2007, anti-NMDA receptor encephalitis is being recognized more frequently, especially in adolescents and young adults [3]

Summary

Conclusions

ECT was an effective and well-tolerated therapy in our patient, and should be considered for the treatment of children with anti-NMDA receptor encephalitis whose catatonia does not respond to immunosuppression and benzodiazepines.

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Funding None