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Persistent seropositivity in oophorectomy-resistant anti-NMDA receptor encephalitis
  1. Susmit Tripathi1,2,
  2. Nara M Michaelson1,2 and
  3. Alan Segal1
  1. 1Neurology, NewYork-Presbyterian Hospital/Weill Cornell Medical Center, New York, New York, USA
  2. 2Neurology, Memorial Sloan Kettering Cancer Center, New York, New York, USA
  1. Correspondence to Dr Susmit Tripathi; sut9038{at}nyp.org

Abstract

To discuss (1) the significance of seropositivity in anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis and (2) clinical decision making in oophorectomy resistant disease. Patient A (a 35-year-old woman) had high CSF and serum anti-NMDA antibody titres, a complicated hospital course, little improvement with first and second-line therapies, and remained with high CSF and serum antibody titres despite unilateral oophorectomy, requiring a nearly 13-month long hospitalisation. Conversely, patient B (a 29-year-old woman) had low CSF titres, seronegative disease and quickly recovered to her baseline with first line therapies and oophorectomy. Anti-NMDAR antibodies are themselves pathological, causing signalling dysfunction and internalisation of the NMDAR. Seropositivity with anti-NMDAR antibodies likely reflects leakage from the blood–brain barrier, with high serum titres being a downstream effect of high CSF titres. Empiric bilateral oophorectomies is controversial but appropriate on a case-by-case basis in extremely treatment-resistant NMDAR encephalitis given the possibility of antigenic microteratomas, which may not be detected on imaging or even bilateral ovarian biopsies.

  • obstetrics and gynaecology
  • neurology
  • pathology

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Footnotes

  • Twitter @nystrokedoc

  • ST and NMM contributed equally.

  • Contributors ST conceptualised and created all figures. ST, NMM and AS drafted the original manuscript. ST and NMM are cofirst authors. All authors were directly involved in the patient’s care and management, provided intellectual content for the manuscript and edited the manuscript.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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