Anti NMDA receptor encephalitis: a potentially treatable encephalitis

Authors

  • Archana K. M. Department of Pediatrics, T. N. Medical College and B. Y. L. Ch. Nair Hospital, Mumbai, Maharashtra, India
  • Sushma Save Department of Pediatrics, T. N. Medical College and B. Y. L. Ch. Nair Hospital, Mumbai, Maharashtra, India
  • Koppikar Rahul Gautam Department of Pediatrics, T. N. Medical College and B. Y. L. Ch. Nair Hospital, Mumbai, Maharashtra, India

DOI:

https://doi.org/10.18203/2349-3291.ijcp20202628

Keywords:

Anti NMDA receptor encephalitis, IVIG, NMDAR antibodies, Neuropsychiatric manifestations

Abstract

Anti NMDA receptor encephalitis is autoimmune encephalitis where antibodies are directed against NMDA receptor subunit. It represents a new category of immune-mediated disorder that is often paraneoplastic, presenting with neuropsychiatric symptoms, which is treatable and can be diagnosed serologically affecting both children and adults. Patient can have variable clinical presentation ranging from prodromal illness, neuropsychiatric symptoms, seizures, autonomic instability, hyperkinesias, catatonia, hypoventilation and with or without an associated teratoma. A positive serum or CSF sample screening for antibodies to the NMDA receptor subunit is confirmative. Supportive findings include CSF study, EEG and MRI Brain. The first-line therapies includes IVIG, corticosteroids or plasma exchange. Second line immunotherapy is rituximab or cyclophosphamide or both. Given the high mortality rate (up to 25%), the likelihood of presentation across the age range and the potential for treatment, a high index of suspicion is warranted by clinicians. Authors report a case of a 5 year old child with anti NMDA receptor encephalitis who responded well to IVIG therapy.

References

Dalmau J, Lancaster E, Martinez-Hernandez E, Rosenfeld MR, Balice-Gordon R. Clinical experience and laboratory investigations in patients with anti-NMDAR encephalitis. Lancet Neurol. 2011;10(1):63-74.

Remy KE, Custer JW, Cappell J, Foster CB, Garber NA, Walker LK, et al. Pediatric anti-N-methyl-D-aspartate receptor encephalitis: A review with pooled analysis and critical care emphasis. Front Pediatr. 2017;5:250.

Barry H, Byrne S, Barrett E, Murphy KC, Cotter DR. Anti-N-methyl-d-aspartate receptor encephalitis: review of clinical presentation, diagnosis and treatment. BJP Bull. 2015;39(1):19-23.

Dalmau J, Gleichman AJ, Hughes EG, Rossi JE, Peng X, Lai M, et al. Anti-NMDA-receptor encephalitis: case series and analysis of the effects of antibodies. Lancet Neurol. 2008 Dec 1;7(12):1091-8.

Sansing LH, Tüzün E, Ko MW, Baccon J, Lynch DR, Dalmau J. A patient with encephalitis associated with NMDA receptor antibodies. Nat Clin Pract Neurol. 2007 May;3(5):291.

Carroll BT. The universal field hypothesis of catatonia and neuroleptic malignant syndrome. CNS Spectrums. 2000 Jul;5(7):26-33.

Finke C, Kopp UA, Prüss H, Dalmau J, Wandinger KP, Ploner CJ. Cognitive deficits following anti-NMDA receptor encephalitis. J Neurol Neurosurg Psychiatr. 2012 Feb 1;83(2):195-8.

Vitaliani R, Mason W, Ances B, Zwerdling T, Jiang Z, Dalmau J. Paraneoplastic encephalitis, psychiatric symptoms, and hypoventilation in ovarian teratoma. Ann Neurol. 2005 Oct;58(4):594-604.

Armangue T, Titulaer MJ, Málaga I, Bataller L, Gabilondo I, Graus F, et al. Pediatric anti-N-methyl-D-aspartate receptor encephalitis-clinical analysis and novel findings in a series of 20 patients. J Pediatr. 2013 Apr 1;162(4):850-6.

Irani SR, Bera K, Waters P, Zuliani L, Maxwell S, Zandi MS, 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 May 26;133(6):1655-67.

Florance NR, Davis RL, Lam C, Szperka C, Zhou L, Ahmad S, et al. Anti-N‐methyl‐D‐aspartate receptor (NMDAR) encephalitis in children and adolescents. Ann Neurol. 2009 Jul;66(1):11-8.

Downloads

Published

2020-06-24

Issue

Section

Case Reports