Japanese encephalitis can trigger anti-N-methyl-D-aspartate receptor encephalitis

J Neurol. 2017 Jun;264(6):1127-1131. doi: 10.1007/s00415-017-8501-4. Epub 2017 May 3.


Japanese encephalitis (JE) is usually a monophasic disease; however, in rare cases, patients with JE may have an early relapse after a partial recovery, giving rise to a biphasic pattern for the disease. In this study, we report three pediatric cases in which post-JE relapse was characterized by movement disorder and/or behavioral problems, and was related to anti-N-methyl-D-aspartate receptor (NMDAR) immunoglobulin G (IgG). Serum and cerebrospinal fluid were examined for anti-NMDAR IgG in three patients who had confirmed JE and then developed relapsing symptoms which were similar to those of anti-NMDAR encephalitis. The main symptoms of the two young children were choreoathetosis, irritability, and sleep disorder; while for the teenager, agitation, mutism, rigidity, and sleep disorder were the main symptoms. Samples of cerebrospinal fluid from all patients were positive for anti-NMDAR IgG, and all patients gradually improved with immunotherapy. Testing for NMDAR antibodies is highly recommend in patients with JE, especially those with a relapsing syndrome involving movement disorder and/or behavioral problems, as these patients may benefit from immunotherapy.

Keywords: Anti-NMDAR encephalitis; Japanese encephalitis; Movement disorder; Relapsing.

MeSH terms

  • Adolescent
  • Anti-N-Methyl-D-Aspartate Receptor Encephalitis / diagnostic imaging
  • Anti-N-Methyl-D-Aspartate Receptor Encephalitis / etiology*
  • Anti-N-Methyl-D-Aspartate Receptor Encephalitis / virology
  • Child, Preschool
  • Encephalitis, Japanese / complications*
  • Encephalitis, Japanese / diagnostic imaging
  • Female
  • Follow-Up Studies
  • HEK293 Cells
  • Humans
  • Magnetic Resonance Imaging
  • Male
  • Receptors, N-Methyl-D-Aspartate / genetics
  • Receptors, N-Methyl-D-Aspartate / metabolism
  • Transfection


  • NR1A protein, human
  • Receptors, N-Methyl-D-Aspartate