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Investigating neuroblastoma in childhood opsoclonus-myoclonus syndrome
  1. Andreas Brunklaus1,2,3,
  2. Keith Pohl4,
  3. Sameer M Zuberi1,
  4. Carlos de Sousa3
  1. 1Fraser of Allander Neurosciences Unit, Royal Hospital for Sick Children, Glasgow, UK
  2. 2School of Medicine, University of Glasgow, Glasgow, UK
  3. 3Great Ormond Street Hospital, London, UK
  4. 4Evelina Children's Hospital, Guy's and St Thomas' NHS Foundation Trust, London, UK
  1. Correspondence to Andreas Brunklaus, Fraser of Allander Neurosciences Unit, Royal Hospital for Sick Children, Yorkhill, Glasgow G3 8SJ, UK; andreas.brunklaus{at}nhs.net

Abstract

Objective Opsoclonus-myoclonus syndrome (OMS) is a serious, often disabling neurological illness of early childhood which is frequently associated with occult neuroblastoma. As investigation methods vary significantly, the authors assessed the usefulness of imaging and metabolic studies in tumour detection.

Methods Retrospective case note review of 101 OMS patients from two paediatric neurology centres over 53 years.

Results The prevalence of neuroblastoma in OMS was 8% in the 1970s, 16% in the 1980s, 38% in the 1990s and 43% in the 2000s, with tumours being mainly low grade. CT/MR imaging of the chest and abdomen was the most accurate test to detect occult neuroblastoma. Poorer sensitivities were noted for metaiodobenzylguanidine scintigraphy and urine catecholamines, reflecting the low metabolic activity of these tumours.

Conclusion CT/MR imaging has the highest detection rate of neuroblastoma and this should be reflected in investigation protocols to achieve the best possible outcome for children with OMS.

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Footnotes

  • Competing interests None.

  • Ethics approval This study was approved by the research and development departments at Great Ormond Street Hospital in London and the Royal Hospital for Sick Children in Glasgow (Reference: 08NS12).

  • Provenance and peer review Not commissioned; externally peer reviewed.

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