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Anti-NMDA-Rezeptor-Enzephalitis

Ein interdisziplinäres Krankheitsbild

Anti-NMDA-receptor encephalitis

An interdisciplinary clinical picture

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Zusammenfassung

Die Anti-NMDA (N-Methyl-D-Aspartat)-Rezeptor-Enzephalitis ist eine seit kurzem bekannte, relativ häufige Form einer schweren Enzephalitis mit einem charakteristischen, in Phasen verlaufenden Krankheitsbild. Die Patienten werden meist durch ein schizophreniformes Syndrom mit formalen und inhaltlichen Denkstörungen (Wahn) und Halluzinationen auffällig, entwickeln aber zusätzlich Fieber, eine Bewusstseinsstörung, Hypoventilation, epileptische Anfälle, autonome Störungen und Dyskinesien. Ein großer Teil der Patienten gelangt daher zunächst in psychiatrische Behandlung, wobei in vielen Fällen die Verdachtsdiagnose einer drogeninduzierten Psychose gestellt wird. Die Anti-NMDA-Rezeptor-Enzephalitis wurde initial bei jungen Frauen mit Ovarialteratomen beschrieben, kommt aber auch häufig bei Frauen ohne Tumor sowie bei Männern und Kindern vor. Die Diagnosestellung der Erkrankung basiert auf der Kombination aus charakteristischem klinischem Bild, stützenden Befunden von Hirn-MRT, EEG und Liquoruntersuchung sowie dem Nachweis hochspezifischer Autoantikörper im Serum oder Liquor, die gegen die NR1-Untereinheit der Glutamatrezeptoren vom Typ NMDA gerichtet sind. Besonders bei der Differenzialdiagnostik einer „Enzephalitis ohne Erregernachweis“ muss an die Anti-NMDA-Rezeptor-Enzephalitis gedacht werden. Die Prognose der Erkrankung ist prinzipiell gut und die Symptome sind auch nach einer langen intensivmedizinischen Behandlung und Beatmungsdauer potenziell reversibel. Der Erfolg korreliert allerdings eng mit einer raschen Diagnosestellung, frühen immunmodulatorischen Therapie und – im Falle einer Neoplasie – besonders mit einer vollständigen Tumorentfernung. Die Versorgung der Patienten erfordert ein interdisziplinäres Vorgehen zwischen Neurologen, Psychiatern, Pädiatern, Onkologen und Gynäkologen.

Summary

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.

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Interessenkonflikt

Der korrespondierende Autor weist auf folgende Beziehung hin: K.P. Wandinger ist Angestellter der Euroimmun AG. Euroimmun entwickelt und vertreibt Testsysteme zum Nachweis von Autoantikörpern, u.a gegen NMDA-Rezeptoren. J. Dalmau hat ein Patent zum Nachweis von Antikörpern gegen NMDA-Rezeptoren angemeldet. Aktuelle Forschungsprojekte von J. Dalmau werden von Euroimmun unterstützt. Trotz des möglichen Interessenkonflikts ist der Beitrag unabhängig und produktneutral.

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Authors and Affiliations

  1. Experimentelle Neurologie und Klinik und Poliklinik für Neurologie, Charité – Universitätsmedizin Berlin, Charitéplatz 1, 10117, Berlin, Deutschland

    H. Prüß

  2. Department of Neurology, University of Pennsylvania, Philadelphia, PA, USA

    J. Dalmau

  3. Klinik und Poliklinik für Psychiatrie und Psychotherapie, Universitätsklinikum Münster, Münster, Deutschland

    V. Arolt

  4. Institut für Neuroimmunologie und Klinische MS-Forschung, Universitätsklinikum Eppendorf, Hamburg, Deutschland

    K.-P. Wandinger

  5. Institut für Experimentelle Immunologie, Euroimmun, Lübeck, Deutschland

    K.-P. Wandinger

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  2. J. Dalmau
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  4. K.-P. Wandinger
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Prüß, H., Dalmau, J., Arolt, V. et al. Anti-NMDA-Rezeptor-Enzephalitis. Nervenarzt 81, 396–408 (2010). https://doi.org/10.1007/s00115-009-2908-9

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  • DOI: https://doi.org/10.1007/s00115-009-2908-9

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