A prospective study of the presentation and management of dancing eye syndrome/opsoclonus-myoclonus syndrome in the United Kingdom

Eur J Paediatr Neurol. 2010 Mar;14(2):156-61. doi: 10.1016/j.ejpn.2009.03.002. Epub 2009 May 6.

Abstract

The incidence, mode of presentation and management of Dancing Eye Syndrome/Opsoclonus-Myoclonus Syndrome (DES/OMS) was prospectively evaluated in 20 United Kingdom (UK) paediatric neurology centres by questionnaire over a 24-month period between 2003 and 2005. Nineteen children were notified, giving an incidence of 0.18 cases per million total population per year. Mean age at presentation was 18 months (range 3-42 months). Fifteen families consented to participate in the study. Atypical features were present in 6/15 cases including very delayed presentation of opsoclonus, dysphagia, and rapid spontaneous improvement without treatment. Only 4/15 cases were associated with neuroblastoma (NB) but current practice in excluding this is diverse and a standardised approach is suggested.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Abdominal Neoplasms / epidemiology
  • Abdominal Neoplasms / pathology
  • Brain Neoplasms / epidemiology
  • Brain Neoplasms / pathology
  • Catecholamines / urine
  • Child, Preschool
  • Deglutition Disorders / epidemiology
  • Female
  • Humans
  • Incidence
  • Infant
  • Magnetic Resonance Imaging
  • Male
  • Neuroblastoma / epidemiology
  • Neuroblastoma / pathology
  • Opsoclonus-Myoclonus Syndrome / diagnosis
  • Opsoclonus-Myoclonus Syndrome / epidemiology*
  • Opsoclonus-Myoclonus Syndrome / therapy*
  • Prevalence
  • Prospective Studies
  • Remission, Spontaneous

Substances

  • Catecholamines