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Treatment responsive opsoclonus–ataxia associated with ovarian teratoma
  1. I Kawachi1,
  2. E Saji1,
  3. Y Toyoshima2,
  4. J Dalmau3,
  5. M Nishizawa1
  1. 1Department of Neurology, Brain Research Institute, Niigata University, Niigata, Japan
  2. 2Department of Pathology, Brain Research Institute, Niigata University, Niigata, Japan
  3. 3Division of Neuro-oncology, Department of Neurology, University of Pennsylvania, Philadelphia, Pennsylvania, USA
  1. Correspondence to Dr M Nishizawa, Department of Neurology, Brain Research Institute, Niigata University, Asahimachi 1, Niigata 951-8585, Japan; nishi{at}bri.niigata-u.ac.jp

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Opsoclonus–ataxia syndrome (OAS) is characterised by the subacute onset of ataxia and involuntary chaotic saccades occurring in all directions of gaze. OAS is often accompanied by myoclonus and may result from paraneoplastic, parainfectious and idiopathic aetiologies. Paraneoplastic OAS may be caused by neuroblastoma in children, and small cell lung carcinoma or breast cancer with anti-Ri antibodies in adults.1 We report here a patient with ovarian teratoma and a treatment responsive paraneoplastic OAS with subclinical supratentorial hypoperfusion on single photon emission computed tomography (SPECT).

Case report

A 24-year-old woman developed abdominal pain and vertigo with unstable gait in the absence of any preceding infection. Findings on CT of the abdomen indicated a left ovarian tumour, 13 cm in diameter. Routine laboratory studies showed no elevations in levels of carcinoembryonic antigen, α-fetoprotein or carbohydrate antigen 125. The patient underwent unilateral salpingo-oophorectomy and removal of a teratoma that contained tissues derived from the three embryological layers without immature features, including a dense meshwork of astrocytes, small mature neurons, numerous axons positive for phosphorylated neurofilament and catecholaminergic neurons (figure 1A, B). Following tumour resection she continued to have severe …

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