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

Authors

  • 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

DOI:

https://doi.org/10.25753/BirthGrowthMJ.v28.i2.14322

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.

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References

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Published

2019-07-18

How to Cite

1.
Branco MA, Sousa L, Garrido C, Carrilho I, Santos M, Temudo T, Martins Silva A, Figueiroa S. Anti-N-Methy-D-Aspartate receptor encephalitis in pediatric age. REVNEC [Internet]. 2019Jul.18 [cited 2024Sep.19];28(2):63-9. Available from: https://revistas.rcaap.pt/nascercrescer/article/view/14322

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