[Anti-NMDA Receptor Encephalitis]

Brain Nerve. 2021 May;73(5):595-604. doi: 10.11477/mf.1416201803.
[Article in Japanese]


Anti-NMDA receptor (NMDAR) encephalitis is an autoimmune disease caused by autoantibodies against the extracellular conformational epitope of the NR1 subunit of the NMDAR (GluN1 antibodies). A series of autoantibodies directed against neuronal surface (NS) or synaptic proteins play an important role in the pathophysiological mechanisms of post-herpes simplex encephalitis (post-HSE), overlapping autoimmune encephalitis and demyelinating syndrome, epileptic seizures, psychosis, involuntary movements (orofacial and limb dyskinesias, catatonia, dystonia, chorea, myoclonus, psychogenic nonepileptic seizures, and faciobrachial dystonic seizures), postpartum psychosis, stiff-person spectrum disorder (including progressive encephalomyelitis with rigidity and myoclonus [PERM]), cerebellar ataxia, and sleep behavior disorders. These NS antibodies are identified with cell-based assays and immunohistochemistry using nonperfused paraformaldehyde-fixed rodent brain tissue. This paper presents an update on anti-NMDAR encephalitis, including the differential diagnosis of cryptogenic new-onset refractory status epilepticus (NORSE), and on the treatment strategy, including third-line therapy.

MeSH terms

  • Anti-N-Methyl-D-Aspartate Receptor Encephalitis* / diagnosis
  • Autoantibodies / metabolism
  • Female
  • Hashimoto Disease*
  • Humans
  • Receptors, N-Methyl-D-Aspartate
  • Seizures


  • Autoantibodies
  • Receptors, N-Methyl-D-Aspartate