Article ; Online: Benign paraspinal ganglioneuroma with paraneoplastic opsoclonus myoclonus syndrome.
BMJ case reports
2024 Volume 17, Issue 2
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, ...
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. |
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MeSH term(s) | Child ; Humans ; Female ; Opsoclonus-Myoclonus Syndrome/complications ; Opsoclonus-Myoclonus Syndrome/diagnosis ; Ganglioneuroma/complications ; Ganglioneuroma/diagnosis ; Neuroblastoma/diagnosis ; Ataxia/complications ; Movement |
Language | English |
Publishing date | 2024-02-27 |
Publishing country | England |
Document type | Case Reports ; Journal Article |
ISSN | 1757-790X |
ISSN (online) | 1757-790X |
DOI | 10.1136/bcr-2023-256846 |
Database | MEDical Literature Analysis and Retrieval System OnLINE |
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