Opsoclonus-ataxia syndrome associated with ovarian mature teratoma

J Obstet Gynaecol Res. 2015 Jul;41(7):1149-53. doi: 10.1111/jog.12675. Epub 2015 Mar 15.

Abstract

A 16-year-old girl with no prior medical history developed vertigo and nausea following alimentary infection. Neurological examination showed limb and truncal ataxia, opsoclonus, myoclonus, and hyperreflexia. Brain magnetic resonance imaging and cerebrospinal fluid analysis showed no abnormalities. Treatment with i.v. high-dose methylprednisolone and immunoglobulin was started, but this proved ineffective. The clinical course was unusual, so whole-body computed tomography was done to evaluate other differential diagnoses. Imaging identified right ovarian mature teratoma. Paraneoplastic opsoclonus-ataxia syndrome was suspected, therefore single-incision laparoscopic ovarian cystectomy was done 10 days after admission. Two months after therapy, the patient had complete recovery and remained asymptomatic at 1 year after onset. Serum testing for anti-neuronal antibodies was negative, including for anti-N-methyl-d-aspartate-receptor antibody. Young women with ataxia and opsoclonus of unclear etiology should be examined for the presence of ovarian teratoma, then intensive immunotherapy and prompt tumor resection can lead to good clinical outcome.

Keywords: anti-N-methyl-d-aspartate receptor; laparoscopic surgery; opsoclonus-ataxia syndrome; ovarian teratoma; paraneoplastic neurological syndrome.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Combined Modality Therapy
  • Diagnosis, Differential
  • Female
  • Humans
  • Immunotherapy
  • Japan
  • Laparoscopy
  • Nausea / etiology
  • Nausea / prevention & control
  • Opsoclonus-Myoclonus Syndrome / etiology*
  • Opsoclonus-Myoclonus Syndrome / prevention & control
  • Organ Sparing Treatments
  • Ovarian Neoplasms / diagnosis
  • Ovarian Neoplasms / physiopathology*
  • Ovarian Neoplasms / surgery
  • Ovarian Neoplasms / therapy
  • Ovariectomy
  • Teratoma / diagnosis
  • Teratoma / physiopathology*
  • Teratoma / surgery
  • Teratoma / therapy
  • Treatment Outcome
  • Vertigo / etiology
  • Vertigo / prevention & control

Supplementary concepts

  • Teratoma, Ovarian