Kantonsspital St.Gallen

Anti-N-methyl-D-aspartate receptor (anti-NMDA-R) encephalitis with ovarial teratoma - case report

Philip Siebel, Dominique Flügel & Gian-Reto Kleger

abstract Introduction: Anti-NMDA-receptor associated encephalitis is a recently described new entity of encephalitis with a very characteristic and nearly pathognomonic disease progression and movement disorder most often affecting young females with ovarian teratoma. The prodromal phase with flu-like symptoms (stage 1) is followed by the psychiatric stage 2 followed by stage 3 with unresponsiveness (catatonia), central hypoventilation and autonomic instability In stage 4 a hyperkinetic movement disorder with facial predominance additionally occurs before recreation takes place after several weeks to months (stage 5). In most of the patients an ovarian teratoma with neural elements can be found.
We report an illustrative video-case of a young woman with ovarian teratoma-associated encephalitis.
Methods/Results: Our 38yrs old previously healthy female patient was hospitalized on a psychiatric ward because of acute schizophrenia and progressed within 3 days to a catatonic state. She had antipsychotic treatment with Haloperidol without much success. Thereafter central hypoventilation and hypersalivation occurred leading to intubation on day 6 and referral to our intensive care unit. Two days after intubation orofacial movements started (orofacial automatisms, jaw opening and closing with severe tongue biting) with intermittent synchronized spread to the extremities and diaphragm. CSF showed lymphocytic pleocytosis (33/ul), normal MRI of the brain and a generalized delta activity in EEG. Because of this characteristic movement disorder in combination with a catatonic state a CT scan of the abdomen has been requested which showed a 17mm ovarian teratoma on the right side. 12 days after first hospitalization the tumor was removed and two circles of intravenous immunoglobulin (IVIG) were applied. During the first 4 weeks after operation and IVIG treatment the clinical picture remained the same although we have been able to further reduce the sedation with Propofol. Overall we expect a favorable prognosis after early removal of the teratoma. The result of anti-NMDA-antibodies was positive.
Conclusion: This case is very illustrative regarding the typical disease course and also from the movement disorders perspective. It is very important to recognize this entity because early causal therapy with removal of the disease causing ovarian teratoma in IVIG therapy is essential for a good outcome.
keywords NMDA, encephalitis, teratoma, antibodies
citation siebel p, Flügel D, Kleger G R. Anti-N-methyl-D-aspartate receptor (anti-NMDA-R) encephalitis with ovarial teratoma - case report. Schweizerisch Archiv für Neurologie und Psychiatrie 2009; 160:168-168.
type journal paper/review (English)
date of publishing 24-10-2009
journal title Schweizerisch Archiv für Neurologie und Psychiatrie (160/4/2009)
pages 168-168