title: Opsoclonus-myoclonus syndrome associated with a nasopharyngeal tumor in an adult: a case report.
abstract: BACKGROUND
  Opsoclonus-myoclonus syndrome is a rare autoimmune syndrome usually seen in children and very rarely in adults. It typically presents with a triad of opsoclonus, myoclonus and ataxia, and is most often associated with a tumor or after an infection or vaccination. Around half of all adult cases are paraneoplastic in origin, and isolated case reports include associations with lung, breast and ovarian cancers. To the best of our knowledge, this is the first-ever reported case of paraneoplastic opsoclonus-myoclonus syndrome occurring in association with a nasopharyngeal carcinoma.
  
  
  METHODS
  A 50-year-old British Caucasian woman presented with left-sided otalgia and subjective hearing loss. Over the coming weeks she developed subacute confusion and dizziness, leading to recurrent falls. Her clinical examination revealed opsoclonus, myoclonus and signs of cerebellar dysfunction. Subsequent magnetic resonance imaging revealed a left-sided nasopharyngeal carcinoma, which was confirmed on biopsy. A tapering dose of steroids and a five-day course of intravenous immunoglobulins, followed by a combination of chemo-radiotherapy for the nasopharyngeal carcinoma, led to a significant clinical improvement. At six months follow-up she had no signs of focal neurological deficit, apart from the inability to tandem walk. We believe that the typical clinical features, presence of a tumor and response to treatment support a paraneoplastic aetiology.
  
  
  CONCLUSIONS
  We show that a nasopharyngeal carcinoma can be associated with adult onset opsoclonus-myoclonus syndrome. Both neurologists and otorhinolaryngologists must be aware of such a presentation. Prognosis of the syndrome depends on early and adequate management of the tumor, therefore prompt identification of the syndrome and the underlying tumor is essential.
authors.affiliation: Royal Liverpool Hospital University Hospital, Postgraduate Office, Prescot Street, Liverpool, L7 8XP, UK. bgani56@gmail.com.
authors.forename: Bilal Gani
authors.initials: BG
authors.lastname: Taib
authors.affiliation: Department of Otolaryngology, Head and Neck Surgery, University Hospital Aintree, Longmoor Lane, Liverpool, L9 7AL, UK. akinshuck@gmail.com.
authors.forename: Andrew J
authors.initials: AJ
authors.lastname: Kinshuck
authors.affiliation: Department of Neurology, Walton Centre NHS Foundation Trust, Lower Lane, Liverpool, L9 7LJ, UK. philip.milburn-mcnulty@thewaltoncentre.nhs.uk.
authors.forename: Philip
authors.initials: P
authors.lastname: Milburn-McNulty
authors.affiliation: Department of Neurology, Walton Centre NHS Foundation Trust, Lower Lane, Liverpool, L9 7LJ, UK. lauren.fratalia@thewaltoncentre.nhs.uk.
authors.forename: Lauren
authors.initials: L
authors.lastname: Fratalia
authors.affiliation: Department of Cellular Pathology, St Helens & Knowsley NHS Trust, Marshalls Cross Road, Merseyside, WA9 3DA, UK. Leigh.Forsyth@sthk.nhs.uk.
authors.forename: Leigh
authors.initials: L
authors.lastname: Forsyth
authors.affiliation: Clatterbridge Cancer Centre NHS Foundation Trust, Clatterbridge Road, Wirral, Merseyside, CH63 4JY, UK. david.husband@clatterbridgecc.nhs.uk.
authors.forename: David
authors.initials: D
authors.lastname: Husband
authors.affiliation: Department of Otolaryngology, Head and Neck Surgery, University Hospital Aintree, Longmoor Lane, Liverpool, L9 7AL, UK. T.M.Jones@liverpool.ac.uk.|Liverpool CR-UK Centre, University of Liverpool, Daulby Street, Liverpool, L69 3GA, UK. T.M.Jones@liverpool.ac.uk.
authors.forename: Terry M
authors.initials: TM
authors.lastname: Jones
authors.affiliation: Department of Neurology, Walton Centre NHS Foundation Trust, Lower Lane, Liverpool, L9 7LJ, UK. anu.jacob@thewaltoncentre.nhs.uk.
authors.forename: Anu
authors.initials: A
authors.lastname: Jacob
mesh_terms: D002277:Carcinoma; D003131:Combined Modality Therapy; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D008875:Middle Aged; D000077274:Nasopharyngeal Carcinoma; D009303:Nasopharyngeal Neoplasms / Q000150:complications* / Q000175:diagnosis / Q000628:therapy; D053578:Opsoclonus-Myoclonus Syndrome / Q000188:drug therapy / Q000209:etiology*
chemical_list: D005938:Glucocorticoids; D016756:Immunoglobulins, Intravenous