Clinical Neuroscience

NMDA-receptor associated encephalitis in a woman with mature cystic ovarian teratoma

VANYA Melinda1, FÜVESI Judit2, KOVÁCS A. Zoltán3, GORGORAPTIS Nikos4, SALEK-HADDADI Afram4, KOVÁCS LÁSZLÓ5, BÁRTFAI György1

NOVEMBER 30, 2016

Clinical Neuroscience - 2016;69(11-12)

DOI: https://doi.org/10.18071/isz.69.0427

Introduction - N-methyl-D-aspartate receptor (NMDA-R) antibody-associated encephalitis has been reported in the international neurological literature to be associated with mature or immature ovarian teratomas (OTs). However, few cases of encephalitis were diagnosed in Hungary. In 2011 Hollody et al. described the first case of anti-NMDA receptor associated encephalitis in Hungary. Objective - Our aim was to present a case of NMDA-R antibody-mediated encephalitis in a woman with OT thereby providing information facilitating diagnosis of OT in women, who present with symptoms of encephalitis. Case - We report the case of a 25 year-old women, who developed NMDA-R -antibody associated autoimmune encephalitis and who displayed confusion, disorientation, a behavioural disturbance with agitation and features of paranoia and at least one reported generalized tonic clonic seizure and orofacial dyskinesia. Magnetic resonance imaging revealed a functional ovarian cyst measuring 3.3 cm, which was removed surgically and demonstrated histologically to be a mature cystic OT. The serum was positive for antibodies to NMDA receptors. Following intravenous immunoglobulin treatment, oophorectomy and a 5-day course of plasma exchange, followed by corticosteroid and azathioprine immunosuppressive therapy, the patient displayed a significant clinical improvement. Conclusion - Cystic teratomas are common benign ovarian lesions in women of reproductive age. Although the association of OTs and NMDA-R antibody-associated encephalitis has been described in the international neurological literature, this relationship needs to be considered from on the interdisciplinary aspect by the health care providers.

AFFILIATIONS

  1. Department of Obstetrics and Gynaecology, Faculty of General Medicine, Albert Szent-Györgyi Health Centre, University of Szeged, Szeged, Hungary
  2. Department of Neurology, Faculty of General Medicine, Albert Szent-Györgyi Health Centre, University of Szeged, Szeged, Hungary
  3. Department of Psychiatry, Faculty of General Medicine, Albert Szent-Györgyi Health Centre, University of Szeged, Szeged, Hungary
  4. Centre for Neurosciences, Department of Neurology, The Royal London Hospital, London, United Kingdom
  5. Department of Rheumatology, Faculty of General Medicine, Albert Szent-Györgyi Health Centre, University of Szeged, Szeged, Hungary

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