Anti-NMDA-receptor encephalitis: a severe, multistage, treatable disorder presenting with psychosis.

Anti-NMDA-receptor encephalitis is a severe, treatable and potentially reversible disorder presenting with memory deficits, psychiatric symptoms and seizures. Initially described in young patients with ovarian teratoma, the disease is meanwhile increasingly recognized also in women without tumours, in men and in children. The presence of anti-glutamate receptor (type NMDA) autoantibodies in serum or cerebrospinal fluid is specific for this novel and widely underdiagnosed disorder. Early recognition is crucial since prognosis largely depends on adequate immunotherapy and, in paraneoplastic cases, complete tumour removal. Indirect immunofluorescence using NMDA-type glutamate receptors recombinantly expressed in human cells is a highly competent method for diagnosing anti-NMDA-receptor encephalitis.

Wandinger KP ，Saschenbrecker S ，Stoecker W ，Dalmau J ... -  《-》

[Anti-nMDA receptor encephalitis--clinical manifestations and pathophysiology].

Iizuka T ，Sakai F 《-》

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

Prüss H ，Dalmau J ，Arolt V ，Wandinger KP ... -  《-》

[Anti-NMDA receptor encephalitis: a neurological and psychiatric emergency].

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.

Pedrosa DJ ，Geyer C ，Klosterkötter J ，Fink GR ，Burghaus L ... -  《-》

[Case report of anti-NMDA receptor encephalitis suspected of schizophrenia].

Suzuki Y ，Kurita T ，Sakurai K ，Takeda Y ，Koyama T ... -  《-》