Behavioral problem, Acute Confusional state and Mutism as Presentation of NMDA Encephalitis
Keywords:Encephalitis, NORSE, NMDA
Anti-N-methyl D-aspartate (NMDA) receptor (anti-NMDAR) encephalitis is caused by immunoreactivity against the NR1 subunit of the NMDA receptor. We present the case of an 18-year-old young girl with that presented to us with behavioral problem, acute confusional state and mutism. After a detailed history and a thorough clinical examination the autoimmune encephalitis antibodies profile were sent. This came positive for NMDA receptor and negative for CASPR-2, Type AMPA1/2, LGI-1, DPPX, and GABAB receptor antibodies. The patient was started on corticosteroids, azathioprine and procyclidine. She was sent home on immune suppressive therapy and called after 4 weeks for follow up. Upon follow up her mutism was gone but the behavioral issue was not significantly improved. Steroids induced psychosis was also observed. She was started on Rituximab with improvement in her behavior. Acute confusional state and behavioral illness preceded the diagnosis of NMDA Encephalitis. The diagnosis of autoimmune encephalitis should be considered a top differential in relapsing patients as it has a very wide range of presentation. Prompt diagnosis and early treatment are the need of the hour as the ramifications of a mis or missed diagnosis could be detrimental
Dalmau J, Tüzün E, Wu HY, et al. Paraneoplastic anti-N-methyl-D-aspartate receptor encephalitis associated with ovarian teratoma. Ann Neurol. 2007;61(1):25–36. https://doi.org/10.1002/ana.21050.
Tsutsui K, Kanbayashi T, Tanaka K, et al. Anti-NMDA-receptor antibody detected in encephalitis, schizophrenia, and narcolepsy with psychotic features. BMC Psychiatry. 2012;12:37. Published 2012 May 8. https://doi.org/10.1186/1471-244X-12-37.
Mann A, Machado NM, Liu N, et al. A multidisciplinary approach to the treatment of anti-NMDA-receptor antibody encephalitis: a case and review of the literature. J Neuropsychiatry Clin Neurosci. 2012;24(2):247–254. https://doi.org/10.1176/appi.neuropsych.11070151.
Kuppuswamy P, Takala C, Sola, C. Management of psychiatric symptoms in NMDAR encephalitis: a case series, literature review and future directions. Gen Hosp Psychiatry. 2014;36(4):388–391. https://doi.org/10.1016/j.genhosppsych.2014.02.010.
Dalmau J, Gleichman AJ, Hughes EG, et al. Anti-NMDA-receptor encephalitis: case series and analysis of the effects of antibodies. Lancet Neurol 2008;7:1091-98
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