You are here

Article Text

Article menu
Download PDFPDF
Paediatrics
Benign paraspinal ganglioneuroma with paraneoplastic opsoclonus myoclonus syndrome
  1. Haritha Prabaharan1,
  2. Suryakala Chandrasekaran1,
  3. Niharika Shetty2 and
  4. Praveen Nayak K2
  1. 1Pediatrics, NITTE (Deemed to be University), Mangalore, Karnataka, India
  2. 2Pediatrics, KS Hegde Medical Academy, Mangalore, Karnataka, India
  1. Correspondence to Dr Niharika Shetty; shetty.niharika{at}gmail.com

Abstract

Opsoclonus-myoclonus-ataxia syndrome (OMAS) is a rare immune-mediated movement disorder occurring as a paraneoplastic manifestation of neuroblastic tumours (NTs), especially neuroblastoma in infancy. Ganglioneuroma (GN), the benign tumour in the spectrum, is rarely associated with OMAS. We report the case of a child in her second year of life presenting with acute onset of progressive paraplegia and OMAS. MRI showed diffuse and infiltrating left paraspinal mass from T3–T9 levels with differentials of neuroblastoma or ganglioneuroblastoma. Histopathological and immunohistochemistry examination of the excised tumour showed maturing GN. The OMAS was managed with intravenous immunoglobulin and steroids. In the 6-month follow-up, the child has a residual motor weakness with myelomalacia in neuroimaging. The case report substantiates the occurrence of OMAS as paraneoplastic manifestation in NTs, including benign, in children younger than 2 years with a female predilection.

  • Movement disorders (other than Parkinson's)
  • Neurooncology
  • Paediatric oncology

https://doi.org/10.1136/bcr-2023-256846

Statistics from Altmetric.com

    Request Permissions

    If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.

    View Full Text

    Footnotes

    • Contributors The following authors were responsible for drafting of the text, sourcing and editing of clinical images, investigation results, drawing original diagrams and algorithms, and critical revision for important intellectual content: NS, PNK, HP and SC. The following authors gave final approval of the manuscript: NS, PNK, HP and SC.

    • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

    • Case reports provide a valuable learning resource for the scientific community and can indicate areas of interest for future research. They should not be used in isolation to guide treatment choices or public health policy.

    • Competing interests None declared.

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