Auto-immune anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis: three case reports

Paediatr Int Child Health. 2017 Aug;37(3):222-226. doi: 10.1080/20469047.2016.1191852. Epub 2016 Jun 22.

Abstract

Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is a recently identified auto-immune disorder characterised by severe memory deficit, a decreased level of consciousness, seizures, autonomic dysfunction and movement disorders. Three girls with the disorder are reported; they were aged 4 years, 5 years and 10 months. The 10-month-old infant who is one of the youngest patients reported with anti-NMDAR encephalitis worldwide, had MRI features suggestive of herpes simplex encephalitis (known to trigger anti-NMDAR encephalitis), but CSF PCR for herpes simplex was negative. All the patients presented with seizures, behavioural change, regression of speech, dystonia and choreo-athetosis. Anti-NMDAR antibodies were detected in all patients' sera and cerebrospinal fluid (CSF). Intravenous immunoglobulin, corticosteroids and rituximab were administered at different intervals. Cases 1 and 2 made a full recovery, but case 3 has mild motor and speech delay. Patients who present with encephalopathy, seizures and movement disorders should be tested for anti-NMDAR antibodies in serum and CSF in addition to being screened for herpes simplex encephalitis.

Keywords: Anti-NMDAR encephalitis; Auto-antibodies; Children; Immunomodulation therapy; Movement disorder.

Publication types

  • Case Reports

MeSH terms

  • Adrenal Cortex Hormones / therapeutic use
  • Anti-N-Methyl-D-Aspartate Receptor Encephalitis / diagnosis*
  • Anti-N-Methyl-D-Aspartate Receptor Encephalitis / drug therapy
  • Anti-N-Methyl-D-Aspartate Receptor Encephalitis / pathology*
  • Autoantibodies / blood*
  • Brain / diagnostic imaging*
  • Child
  • Child, Preschool
  • Diagnosis, Differential
  • Female
  • Humans
  • Immunoglobulins, Intravenous / therapeutic use
  • Immunologic Factors / therapeutic use
  • Infant
  • Magnetic Resonance Imaging
  • Receptors, N-Methyl-D-Aspartate / immunology*
  • Rituximab / therapeutic use
  • Treatment Outcome

Substances

  • Adrenal Cortex Hormones
  • Autoantibodies
  • Immunoglobulins, Intravenous
  • Immunologic Factors
  • Receptors, N-Methyl-D-Aspartate
  • Rituximab