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Neurological response to early removal of ovarian teratoma in anti-NMDAR encephalitis
  1. M Seki1,
  2. S Suzuki1,
  3. T Iizuka2,
  4. T Shimizu1,
  5. Y Nihei1,
  6. N Suzuki1,
  7. J Dalmau3
  1. 1
    Department of Neurology, School of Medicine, Keio University, Tokyo, Japan
  2. 2
    Department of Neurology, School of Medicine, Kitasato University, Kanagawa, Japan
  3. 3
    Department of Neurology, Division of Neuro-oncology, University of Pennsylvania, Philadelphia, PA, USA
  1. Dr Shigeaki Suzuki, 35 Shinanomachi, Shinjuku-ku, Tokyo 160-8582, Japan; shigeaki{at}sc.itc.keio.ac.jp

Abstract

We report an 18-year-old woman with anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis, who developed psychiatric symptoms, progressive unresponsiveness, dyskinesias, hypoventilation, hypersalivation and seizures. Early removal of an ovarian teratoma followed by plasma exchange and corticosteroids resulted in a prompt neurological response and eventual full recovery. Serial analysis of antibodies to NR1/NR2B heteromers of the NMDAR showed an early decrease of serum titres, although the cerebrospinal fluid titres correlated better with clinical outcome. The patients’ antibodies reacted with areas of the tumour that contained NMDAR-expressing tissue. Search for and removal of a teratoma should be promptly considered after the diagnosis of anti-NMDAR encephalitis.

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Footnotes

  • Funding: This work was supported in part by grants RO1CA89054 and RO1CA1071892 (to JD).

  • Competing interests: None declared.