Critical Care Management of Anti-N-Methyl-D-Asparate Receptor Encephalitis

Objectives: Anti-N-methyl-D-asparate receptor encephalitis is considered an immune-mediated form of encephalitis with paraneoplastic and nonparaneoplastic forms. Delay in recognition is common and patients typically present to the ICU without a diagnosis or with complications following a delayed diagnosis. The aim of this review is to provide a focused overview for the ICU clinician regarding presentation, diagnosis, and critical care management. Data Sources, Study Selection, and Data Extraction: PubMed database search with manual review of articles involving anti-N-methyl-D-asparate receptor encephalitis. Data Synthesis: Anti-N-methyl-D-asparate receptor encephalitis is increasingly encountered in the ICU. The cascade of events initiating anti-N-methyl-D-asparate receptor antibody formation may involve an infectious trigger particularly in the setting of teratoma. Following a prodrome, most patients develop psychiatric symptoms followed by movement disorder. Classical, psychiatric, and catatonic phenotypes may be distinguished based on the presence and severity of symptoms. Early immunotherapy and low initial cerebrospinal fluid inflammation are independent predictors of positive outcomes in ICU patients. Concomitant organ failure, status epilepticus, and the identification of a tumor did not influence outcome in critically ill patients. Supportive care in the ICU includes management of various manifestations of dyskinesia, status epilepticus, autonomic disorders, and the need for general sedation. Common treatment strategies and limitations are discussed including the emerging role of bortezomib. Conclusions: Intensivists should be familiar with the presentation and management of anti-N-methyl-D-asparate receptor encephalitis. Early diagnosis and immediate implementation of steroids, immunoglobulins, and/or plasmapheresis and immune therapy are associated with a good neurologic outcome although response may be delayed. The selection and timing of second-line immune therapy requires further study. Dr. Neyens disclosed off-label product use of IV immune globulin, rituximab, and bortezomib. Dr. Gaskill disclosed off-label product use of rituximab and bortezomib. Dr. Chalela has disclosed that he does not have any potential conflicts of interest. For information regarding this article, E-mail: Chalela@musc.edu Copyright © by 2018 by the Society of Critical Care Medicine and Wolters Kluwer Health, Inc. All Rights Reserved.

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