Erstmanifestation eines organischen manisch-schizophreniformen Syndroms mit nachfolgender Katatonie bei NMDA-Rezeptor-Antikörper-positiver Encephalitis

Zusammenfassung

Wir berichten über eine 39-jährige Patientin mit ausgeprägter Katatonie nach zuvor über sechs Monate progredientem schizomanischen Syndrom. Klinisch zeigten sich bei Aufnahme eine Katatonie, ein tetraspastisches Syndrom und fokale epileptische Anfälle. Kernspintomografisch kamen kontrastmittelaffine Signalsteigerungen im beidseitigen subkortikalen Marklager zur Darstellung. Im Liquor zeigte sich eine lymphozytäre Pleozytose mit autochthonen oligoklonalen Banden. Im Serum waren reproduzierbar Antikörper gegen den NMDA-NR-1-Rezeptor nachweisbar. Eine ausführliche Tumorsuche erbrachte ein negatives Ergebnis, auch hinsichtlich des Vorliegens einer Ovarialneoplasie, welche bei der paraneoplastischen Form der NMDA-Rezeptor-Antikörper-positiven Enzephalitis häufig anzutreffen ist. Wir gehen daher von einer autoimmunen, nicht paraneoplastischen Enzephalitis aus. Unter immunsuppressiver Therapie initial mit Glukokortikoiden und im Verlauf mit Rituximab kam es zu einer schrittweisen deutlichen Besserung der Symptomatik. Dieser Fall zeigt, dass bei erstmaliger psychotischer Symptomatik nach einer NMDA-Rezeptor-Antikörper-positiven Enzephalitis gefahndet werden sollte.

Abstract

We report on a 39-year-old female patient who developed catatonia after there had been schizomanic symptoms in the six months before. At admission the patient exhibited catatonia, a tetraspastic syndrome and focal epileptic seizures. The cranial MRI revealed bilateral subcortical hyperintense lesions which took up contrast agent. Examination of the cerebrospinal fluid disclosed a lymphocytic pleocytosis and autochthone oligoclonal bands. In the serum autoantibodies against the NMDA-NR-1 receptor were reproducibly detected. A detailed search for a tumour was negative. In detail, we could exclude a neoplasm of the ovaries which is often present in the paraneoplastic type of anti-NMDA-receptor encephalitis. Therefore we assume an autoimmune, not paraneoplastic, encephalitis in our patient. The symptoms improved significantly after an immunosuppressive therapy – initially with glucocorticoids followed by rituximab – had been initiated. This case illustrates that an autoimmune encephalitis should be looked for when first psychotic symptoms occur.

Schlüsselwörter

NMDA-Rezeptor-Antikörper - Enzephalitis - autoimmun - Psychose - Rituximab

Key words

anti-NMDA receptor - encephalitis - autoimmune process - psychosis - rituximab

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