Hostname: page-component-7bb8b95d7b-s9k8s Total loading time: 0 Render date: 2024-09-13T19:21:27.030Z Has data issue: false hasContentIssue false

Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis presenting to the emergency department with status epilepticus

Published online by Cambridge University Press:  04 March 2015

Brodie Nolan
Affiliation:
Department of Medicine, University of Toronto, Toronto, ON
Katharina Plenk
Affiliation:
Department of Internal Medicine, York Central Hospital, Richmond Hill, ON
David Carr*
Affiliation:
Department of Family and Community Medicine, University of Toronto, University Health Network, Toronto, ON
*
Toronto General Hospital, R. Fraser Elliott Building, Ground -480, 200 Elizabeth Street, Toronto, ON M5G 2C4; [email protected]

Abstract

Core share and HTML view are not available for this content. However, as you have access to this content, a full PDF is available via the ‘Save PDF’ action button.

Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is a recently described and underdiagnosed entity that typically affects young, previously healthy individuals. Patients usually present in phases, which may include refractory seizures, psychosis, unresponsiveness, and autonomic instability. The diagnosis of anti-NMDAR encephalitis is challenging; however, prompt diagnosis and early treatment can lead to complete recovery. The incidence of anti-NMDAR encephalitis may be as high as four times that of encephalitis from herpes simplex, varicella-zoster, and West Nile viruses; however, it remains an underrecognized disorder. Early initiation of immunotherapy in anti-NMDAR encephalitis has been found to improve patient outcomes. Because of this, emergency physicians must be vigilant and consider this diagnosis in patients with altered mental status in whom a toxicologic or other etiology is not suspected. Early consideration of this diagnosis can facilitate urgent neurology consultation and prevent diagnostic delays arising from psychiatric referrals. It is essential to consider this diagnosis in suspicious emergency department presentations, particularly young patients who present with altered mental status, psychosis, or new-onset seizure activity when other obvious causes are ruled out. Emergency physicians should discuss the possibility of empirical intravenous immunoglobulin administration with neurology consultants if anti-NMDAR encephalitis is suspected. We describe the case of a 20-year-old man with anti-NMDAR encephalitis who presented to the emergency department with status epilepticus.

Type
Case Report • Rapport de cas
Copyright
Copyright © Canadian Association of Emergency Physicians 2014

References

REFERENCES

1. Dalmau, J, Tüzün, E, Wu, H-Y, et al. Paraneoplastic anti-Nmethyl-D-aspartate receptor encephalitis associated withovarian teratoma. Ann Neurol 2007;61:2536, doi:10.1002/ana.21050.Google Scholar
2. Hughes, EG, Peng, X, Gleichman, AJ, et al. Cellular and synaptic mechanisms of anti-NMDA receptor encephalitis. J Neurosci 2010;30:5866–75, doi:10.1523/JNEUROSCI.0167-10.2010.Google Scholar
3. Peery, HE, Day, GS, Dunn, S, et al. Anti-NMDA receptor encephalitis.The disorder, the diagnosis and the immunobiology. Autoimmun Rev 2012;11:863–72, doi:10.1016/j.autrev.2012.03.001.Google Scholar
4. Day, GS, High, SM, Cot, B, Tang-Wai, DF. Anti-NMDAreceptor encephalitis: case report and literature review of an under-recognized condition. J Gen Intern Med 2011;26:811–6, doi:10.1007/s11606-011-1641-9.Google Scholar
5. Irani, SR, Bera, K, Waters, P, et al. N-methyl-D-aspartate antibody encephalitis: temporal progression of clinical and paraclinical observations in a predominantly non-paraneoplastic disorder of both sexes. Brain 2010;133(Pt 6):1655–67, doi:10.1093/brain/awq113.Google Scholar
6. Gable, MS, Gavali, S, Radner, A, et al. Anti-NMDA receptor encephalitis: report of ten cases and comparison with viral encephalitis. Eur J Clin Microbiol Infect Dis 2009;28:1421–9, doi:10.1007/s10096-009-0799-0.Google Scholar
7. Gable, MS, Sheriff, H, Dalmau, J, et al. The frequency of autoimmune N-methyl-D-aspartate receptor encephalitis surpasses that of individual viral etiologies in young individuals enrolled in the California Encephalitis Project. Clin Infect Dis 2012;54:899904, doi:10.1093/cid/cir1038.Google Scholar
8. Dalmau, J, Lancaster, E, Martinez-Hernandez, E, et al. Clinical experience and laboratory investigations in patients with anti-NMDAR encephalitis. Lancet Neurol 2011;10:6374, doi:10.1016/S1474-4422(10)70253-2.Google Scholar
9. Prüss, H, Dalmau, J, Harms, L, et al. Retrospective analysis of NMDA receptor antibodies in encephalitis of unknown origin. Neurology 2010;75:1735–9, doi:10.1212/WNL.0b013e3181fc2a06.Google Scholar
10. Dalmau, J, Gleichman, AJ, Hughes, EG, et al. Anti-NMDAreceptor encephalitis: case series and analysis of the effects of antibodies. Lancet Neurol 2008;7:1091–8, doi:10.1016/S1474-4422(08)70224-2.Google Scholar
11. Titulaer, MJ, McCracken, L, Gabilondo, I, et al. Treatment and prognostic factors for long-term outcome in patients with anti-NMDA receptor encephalitis: an observational cohort study. Lancet Neurol 2013;12:157–65, doi:10.1016/S1474-4422(12)70310-1.Google Scholar