• D J Pedrosa, C Geyer, J Klosterkötter, G R Fink, and L Burghaus.
• Klinik und Poliklinik für Neurologie, Universitätsklinik Köln. david.pedrosa@uk-koeln.de
• Fortschr Neurol Psychiatr. 2012 Jan 1; 80 (1): 29-35.

AbstractAnti-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.© Georg Thieme Verlag KG Stuttgart · New York.

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