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Fortschr Neurol Psychiatr 2012; 80(1): 29-35
DOI: 10.1055/s-0031-1281961
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© Georg Thieme Verlag KG Stuttgart · New York

Anti-NMDA-Rezeptor-Enzephalitis: Ein neurologisch-psychiatrischer Notfall

Anti-NMDA Receptor Encephalitis: A Neurological and Psychiatric Emergency
D. J. Pedrosa
1   Klinik und Poliklinik für Neurologie, Universitätsklinik Köln
,
C. Geyer
2   Klinik für Psychiatrie und Psychotherapie, Universitätsklinik Köln
,
J. Klosterkötter
2   Klinik für Psychiatrie und Psychotherapie, Universitätsklinik Köln
,
G. R. Fink
1   Klinik und Poliklinik für Neurologie, Universitätsklinik Köln
,
L. Burghaus
1   Klinik und Poliklinik für Neurologie, Universitätsklinik Köln
› Author Affiliations
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Publication History

Publication Date:
15 December 2011 (online)

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Zusammenfassung

Die Anti-NMDA-Rezeptor-Enzephalitis ist eine in den letzten Jahren zunehmend Aufmerksamkeit findende Enzephalitis mit charakteristischen und in verschiedenen Phasen verlaufenden Symptomen. Initial stehen vor allem psychiatrische Symptome im Vordergrund mit progredienten schizoiden Verhaltensauffälligkeiten einschließlich formaler und inhaltlicher Denkstörungen und Persönlichkeitsveränderungen, sodass ein beträchtlicher Teil der – häufig jungen – Erkrankten zunächst psychiatrisch betreut wird. Im weiteren Verlauf treten neurologische Symptome wie epileptische Anfälle, Bewegungsstörungen und autonome Störungen hinzu. Die Anti-NMDA-Rezeptor-Enzephalitis wurde erstmals bei jungen Frauen im Zusammenhang mit Teratomen beschrieben, tritt jedoch auch nicht tumorassoziiert sowie bei Männern und Kindern auf. Die Diagnosestellung basiert dabei neben einem charakteristischen klinischen Bild auf den Befunden von MRT, EEG und Liquor inkl. dem Nachweis der hochspezifischen Antikörper, die sich gegen die NR1-Untereinheit der Glutamatrezeptoren vom Typ NMDA richten. Die Prognose der Erkrankung ist prinzipiell gut und eine vollständige Remission möglich. Dabei sind allerdings eine rasche Diagnosestellung und ein rechtzeitiger Beginn der immunmodulatorischen Therapie notwendig. Die radikale Tumorentfernung im Falle einer Neoplasie-assoziierten Genese stellt ebenfalls einen entscheidenden kurativen Behandlungsschritt dar.

Abstract

Anti-NMDA receptor encephalitis is a severe autoimmune disease, first described in 2007. Since then a number of cases have been published, suggesting that to date the disease is a considerably underdiagnosed entity. The clinical picture develops over a relatively long period of time and is initially characterised by psychiatric symptoms such as decreased levels of consciousness and hallucinations as well as paranoid behaviour. In the course of the disease neurological symptoms occur, in particular, seizures, autonomic dysfunction and dyskinesias. Due to the young age of many patients, the symptoms are often mistaken as to result from drug-induced psychosis. Anti-NMDA receptor Encephalitis was first described in young women with teratomas. In the past few years the disorder has also been reported in men and children and without any detectable neoplasia. The diagnosis is based on the characteristic clinical picture and supportive findings in MRI, EEG and the cerebrospinal fluid. Hereby, highly specific autoantibodies directed against the NR1 subunit of the NMDA-type glutamate receptors in the CSF (or serum) play an important role and should be sought specifically in any case of an “encephalitis of unknown cause”. The prognosis of the disease is favourable, even when autonomic disorders entail ventilation and/or prolonged intensive care treatment is necessary. Nonetheless, the clinical outcome is highly dependent on an early diagnosis and immunotherapy without delay. In the case of a malignancy, tumour removal is also crucial. Taken together, an interdisciplinary approach including neurologists, psychiatrists, oncologists and gynaecologists is essential in order to detect and effectively treat this disorder.

Schlüsselwörter

Anti-NMDA-Rezeptor-Enzephalitis - Epilepsie - akute Psychose - affektive Störung

Key words

anti-NMDA receptor encephalitis - epilepsy - acute psychosis - affective disorder
 

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