[Anti-NMDA-receptor encephalitis. An interdisciplinary clinical picture]

Nervenarzt. 2010 Apr;81(4):396, 398, 400, passim. doi: 10.1007/s00115-009-2908-9.
[Article in German]

Abstract

Anti-NMDA-receptor encephalitis is a severe and considerably underdiagnosed form of encephalitis with characteristic clinical features including psychiatric symptoms, decreased levels of consciousness, hypoventilation, epileptic seizures, autonomic dysfunction and dyskinesias. Most patients are primarily seen by psychiatrists, often on the assumption of a drug-induced psychosis. Anti-NMDA-receptor encephalitis had initially been described in young women with ovarian teratoma, but is also common in women without tumour, in men and in children. The diagnosis is based on the characteristic clinical picture, supporting findings of brain MRI, electroencephalogram and cerebrospinal fluid (CSF), and the presence of highly specific autoantibodies directed against the NR1 subunit of NMDA-type glutamate receptors in the serum or CSF. In particular, anti-NMDA-receptor encephalitis must be excluded in patients with 'encephalitis of unknown cause'. In principle, the prognosis is favourable and recovery from symptoms can be expected even after prolonged intensive care treatment and mechanical ventilation. However, improvement correlates with prompt identification of the disorder, early immunotherapy and - in the case of a malignancy - with complete tumour removal. Patient care requires an interdisciplinary approach including neurologists, psychiatrists, paediatricians, oncologists and gynaecologists.

Publication types

  • Case Reports
  • English Abstract
  • Review

MeSH terms

  • Autoantibodies / blood
  • Autoimmune Diseases / diagnosis*
  • Autoimmune Diseases / drug therapy
  • Autoimmune Diseases / immunology*
  • Child
  • Cooperative Behavior
  • Diagnosis, Differential
  • Electroencephalography
  • Encephalitis / diagnosis*
  • Encephalitis / drug therapy
  • Encephalitis / immunology*
  • Female
  • Fluorescent Antibody Technique, Indirect
  • Humans
  • Immunization, Passive
  • Immunosuppressive Agents / therapeutic use
  • Interdisciplinary Communication
  • Limbic Encephalitis / diagnosis
  • Limbic Encephalitis / immunology
  • Limbic Encephalitis / therapy
  • Limbic System / pathology
  • Magnetic Resonance Imaging
  • Male
  • Paraneoplastic Syndromes / diagnosis
  • Paraneoplastic Syndromes / drug therapy
  • Paraneoplastic Syndromes / immunology
  • Patient Care Team*
  • Plasmapheresis
  • Psychotic Disorders / diagnosis*
  • Psychotic Disorders / drug therapy
  • Psychotic Disorders / immunology*
  • Receptors, N-Methyl-D-Aspartate / immunology*
  • Young Adult

Substances

  • Autoantibodies
  • Immunosuppressive Agents
  • NR1 NMDA receptor
  • Receptors, N-Methyl-D-Aspartate