Anti-N-Methy-D-Aspartate receptor encephalitis in pediatric age

  • Mariana Amorim Branco Neuropediatrics Department, Centro Materno-Infantil do Norte, Centro Hospitalar Universitário do Porto
  • Luísa Sousa Neurology Department, Centro Hospitalar Universitário do Porto
  • Cristina Garrido Neuropediatrics Department, Centro Materno-Infantil do Norte, Centro Hospitalar Universitário do Porto
  • Inês Carrilho Neuropediatrics Department, Centro Materno-Infantil do Norte, Centro Hospitalar Universitário do Porto
  • Manuela Santos Neuropediatrics Department, Centro Materno-Infantil do Norte, Centro Hospitalar Universitário do Porto
  • Teresa Temudo Neuropediatrics Department, Centro Materno-Infantil do Norte, Centro Hospitalar Universitário do Porto
  • Ana Martins Silva Neurology Department, Centro Hospitalar Universitário do Porto
  • Sónia Figueiroa Neuropediatrics Department, Centro Materno-Infantil do Norte, Centro Hospitalar Universitário do Porto
Keywords: antibodies, encephalitis, neoplasm

Abstract

Introduction: Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is an immune-mediated syndrome characterized by psychiatric symptoms, movement disorders, insomnia, seizures, altered level of consciousness and autonomic dysfunction. The aim of this study was described the cases of anti-NMDAR encephalitis admitted to a Neuropediatrics Department, in order to alert for the importance of early recognition of this clinical entity due to its implications in prognosis.
Material and Methods: A retrospective review of the clinical processes of patients with anti-NMDAR encephalitis admitted to the Neuropediatrics Department of a University Hospital in the period between May 2009 and May 2016 was conducted.
Results: Diagnosis of anti-NMDAR encephalitis was established in nine patients. Mean age at diagnosis was 9.4 years and five patients were male. Most cases (88.9%) presented with neuropsychiatric symptomatology. Movement disorders were another frequent finding (88.9%) and include chorea, dystonia and dyskinesias. Altered level of consciousness occurred in 77.8% of cases, insomnia and speech disturbance in 66.7%, seizures in 55.6% and autonomic dysfunction in 22.2%. Anti-NMDAR antibodies were identified in the cerebrospinal fluid of all patients. One patient had an underlying neoplasm. All patients underwent immunosuppressive therapy and seven patients fully recovered. In the remaining patients, sequelae included refractory epilepsy and cognitive impairment.
Discussion: The diagnosis of anti-NMDAR encephalitis should be considered in children and adolescents presenting with psychiatric symptoms associated and movement disorders. In this sample, although most patients responded very favorably to treatment, severe sequelae were also observed. This highlights the importance of an early diagnosis to initiate treatment as soon as possible.

References

Brito MJ, Luís C, Silva R, Gouveia C, Vieira JP. Enfalites – Recomendações da Sociedade de Infecciologia Pediátrica e da Sociedade Portuguesa de Neuropediatria. Acta Pediatr Port 2011: 42: 284-91.

Armangue T, Pedrol MP, Dalmau J. Autoimmune Encephalitis in Children. J Child Neurol. 2012; 27: 1460-1469.

Ferdinand P, Mitchell L. Anti-NMDA Receptor Encephalitis. J Clin cell Immunol 2012; S 10:007.

Florance-Ryan N, Dalmau J. Update on anti-N-methyl-D-aspartate receptor encephalitis in children and adolescents. Current Opinion in Pediatrics 2010; 22: 739-744.

Dalmau J, Lancaster E, Hernandez EM, Rosenfeld MR, Gordon RB. Clinical experience and laboratory investigations in patients with anti-NMDAR encephalitis. Lancet Neurol 2011; 10: 63-74.

Borlot F, Santos MLF, Bandeira M, Liberalesso PB, Kok F, Lohr A, Reed UC. Anti-N-methyl D-aspartate receptor encephalitis in childhood. J Pediatr (Rio J) 2012; 88: 275-8.

Dalmau J, Rosenfeld MR. Paraneoplastic and autoimmune encephalitis. UpToDate 2017

Armangue T, Titulaer MJ, Málaga I, Bataller L, Gabilondo I, Graus F, Dalmau J. Pediatric Anti-NMDA encephalitis – Clinical analysis and novel findings in a series of 20 patients. J Pediatr. 2013; 162: 850-856.

Baker J, Jeziorkowski C, Siebe C, Osborn MB. Two Cases of Anti-NMDA Receptor Encephalitis. West J Emerg Med.2016; 17: 623-626.

Matoq AA, Rappoport AS, Yang Y, O` Babatunde J, Bakerywala R, Sheth RD. Anti-NMDA- receptor antibody encephalitis in infants. Epilepsy and Behavior Case Reports 4 (2015): 99-101.

Graus F, Titulaer M, Balu R et al. A clinical approach to diagnosis of autoimmune encephalitis. www.thelancet.com/neurology 2016; 15: 391-404.

Kubota S, Fuchigami T, Momoki E, Hoshi R et al. Anti-N-Methyl-D-Aspartate Receptor Encephalitis and Ovarian Teratoma: A Case Report. Int J Clin Pediatr. 2017; 6:20-23.

Bartolini L. How do you treat anti-NMDA receptor encephalitis? Neurol Clin Pract 2016; 6: 69-72.

Published
2019-07-18
Section
Original Articles