Use and Safety of Immunotherapeutic Management of N-Methyl-d-Aspartate Receptor Antibody Encephalitis

Authors:
  • IGInternational NMDAR Antibody Encephalitis Consensus Group
    International NMDAR Antibody Encephalitis Consensus Group

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Abstract
N-methyl-d-aspartate receptor (NMDAR) antibody encephalitis (NMDARE) is the most common autoimmune encephalitis, predominantly affecting children and young adults. Management is challenging owing to the frequently severe disease in the acute phase, often requiring prolonged hospitalization, the variable and unpredictable outcomes, and the possibility of relapses.1 Some patients recover fully, but many experience long-term cognitive and psychiatric problems, with significant effects on education, employment, and quality of life.2 Overall, immunotherapy improves functional outcome1,3-5 and reduces relapses,1,6,7 but many therapeutic questions remain incompletely answered, including the effects of specific immunotherapies on outcome and risk of relapses and the role of second-line and maintenance long-term immunotherapies.