Rapid onset of altered mental status with progressive autonomic instability and hypoventilation in a 2-year-old male

Anti N-methyl-D-aspartate receptor (NMDAR) encephalitis should besuspected in children who report with acute behavioral change, seizures,and non-specific movement disorders. While historically associated withadolescent females with ovarian teratoma, anti-NMDAR encephalitis hasnow been described in the pediatric population of both sexes. These patientsdisplay the neurological and psychiatric manifestations similar to femalesbut lack a tumor. We report a two-year-old previously healthy young malewho presented with alteration in speech, seizures, and extra pyramidalmovement disorders. He was diagnosed with anti-NMDAR encephalitis withpositive cerebrospinal fluid (CSF) serology for anti-NMDAR antibodies.In this case report, we describe the clinical presentation, course of illness,investigation, and management of our patient. We conclude by emphasizingthree observations and insights from our case compared to what is mentionedin the literature.

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1. Florance-Ryan N, Dalmau J. Update on anti-Nmethyl-D-aspartate receptor encephalitis in children and adolescents. Curr Opin Pediatr 2010; 22: 739- 744.

2. Dalmau J, Gleichman AJ, Hughes EG, et al. AntiNMDA-receptor encephalitis: case series and analysis of the effects of antibodies. Lancet Neurol 2008; 7: 1091-1098.

3. Kashyape P, Taylor E, Ng J, Krishnakuma D, Kirkham F, Whitney A. Successful treatment of two paediatric cases of anti-NMDA receptor encephalitis with cyclophosphamide: the need for early aggressive immunotherapy in tumour negative paediatric patients. Eur J Paediatr Neurol 2012; 16: 74-78.

4. Mirza MK, Pogoriler J, Paral K, et al. Adjunct therapeutic plasma exchange for anti-N-methylD-aspartate receptor antibody encephalitis: a case report and review of literature. J Clin Apher 2011; 26: 362-365.

5. Dalmau J, Tǖzǖn E, Wu HY, et al. Paraneoplastic anti-N-methyl-D-aspartate receptor encephalitis associated with ovarian teratoma. Ann Neurol 2007; 61: 25-36.

6. Hughes EG, Peng X, Gleichman AJ, et al. Cellular and synaptic mechanisms of anti-NMDA receptor encephalitis. J Neurosci 2010; 30: 5866-5875.

7. Perry HE, Day GS, Dunn S et al. Anti-NMDA receptor encephalitis. The disorder, the diagnosis and the immunobiology. Autoimmun Rev 2012; 11: 863-872.

8. Iizuka T, Sakai F, Ide T, et al. Anti-NMDA receptor encephalitis in Japan: long-term outcome without tumor removal. Neurology 2008; 70: 504-511.

9. Wandinger KP, Saschenbrecker S, Stoecker W, Dalmau J. Anti-NMDA-receptor encephalitis: a severe, multistage, treatable disorder presenting with psychosis. J Neuroimmunol 2011; 231: 86-91.

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

11. 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: 1421-1429.

12. Matoq AA, Rappoport AS, Yang Y, O’Babatunde J, Bakerywala R, Sheth RD. Anti-NMDA-receptor antibody encephalitis in infants. Epilepsy Behav Case Rep 2015; 4: 99-101.
Turkish Journal of Pediatrics-Cover
  • ISSN: 0041-4301
  • Yayın Aralığı: 6
  • Başlangıç: 1958
  • Yayıncı: Hacettepe Üniversitesi Çocuk Sağlığı Enstitüsü Müdürlüğü
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