Neuroblastoma-related severe hypoperfusion in the cerebellum of an infant: A case of opsoclonus-myoclonus syndrome

Document Type : Case report


1 Department of Diagnostic Imaging, Graduate School of Medicine, Hokkaido University, Sapporo, Japan

2 Department of Nuclear Medicine, Hokkaido University Hospital, Sapporo, Japan

3 Global Center for Biomedical Science and Engineering, Faculty of Medicine, Hokkaido University, Sapporo, Japan

4 Department of Pediatrics, Graduate School of Medicine, Hokkaido University, Sapporo, Japan

5 Department of Diagnostic and Interventional Radiology, Hokkaido University Hospital, Sapporo, Japan


A 2-year-old girl started to wobble without any specific triggers, so the patient was admitted to our hospital's pediatric department. The entire cerebellum showed severe atrophy on MRI and much lower uptake than that in the cerebral cortex on perfusion SPECT. The diagnosis of opsoclonus-myoclonus syndrome (OMS) was suspected. MRI visualized a small mass behind the inferior vena cava. Although its uptake on I-123 MIBG scintigraphy was inconclusive, the mass was surgically removed, and the diagnosis of neuroblastoma was pathologically confirmed. OMS is one of the paraneoplastic neurological syndromes with cerebellar ataxia, myoclonus of the trunk and extremities, and opsoclonus as its main symptoms. Approximately 50% of children cases with OMS are associated with neuroblastoma. The prognosis for neuroblastoma itself with OMS is relatively good, but the neurological prognosis is very poor. If there is decreased blood flow in the cerebellum of an infant, it may be necessary to search for neuroblastoma.


  1. Rossor T, Yeh EA, Khakoo Y, Angelini P, Hemingway C, Irani SR, et al. Diagnosis and Management of Opsoclonus-Myoclonus-Ataxia Syndrome in Children: An International Perspective. Neurology-Neuroimmunology Neuroinflammation. 2022; 9(3): e1153.
  2. Pranzatelli MR, Tate ED, McGee NR. Demographic, clinical, and immunologic features of 389 children with opsoclonus-myoclonus syndrome: a cross-sectional study. Frontiers in Neurology. 2017; 8: 468.
  3. Matthay KK, Blaes F, Hero B, Plantaz D, De Alarcon P, Mitchell WG, Pike M, Pistoia V. Opsoclonus myoclonus syndrome in neuroblastoma a report from a workshop on the dancing eyes syndrome at the advances in neuroblastoma meeting in Genoa, Italy, 2004. Cancer letters. 2005; 228(1-2):275-82.
  4. Pang KK, de Sousa C, Lang B, Pike MG. A prospective study of the presentation and management of dancing eye syndrome/ opsoclonus–myoclonus syndrome in the United Kingdom. european journal of paediatric neurology. 2010; 14(2):156-61.
  5. Pranzatelli MR. The neurobiology of the opsoclonus-myoclonus syndrome. Clin Neuropharmacol. 1992; 15(3):186-228.
  6. Whittle SB, Smith V, Doherty E, Zhao S, Mc Carty S, Zage PE. Overview and recent advances in the treatment of neuroblastoma. Expert review of anticancer therapy. 2017; 17(4):369-86.
  7. Rudnick E, Khakoo Y, Antunes NL, Seeger RC, Brodeur GM, Shimada H, et al. Opsoclonus‐myoclonus‐ataxia syndrome in neuroblastoma: Clinical outcome and antineuronal antibodies—a report from the children's cancer group study. Medical and Pediatric Oncology: The Official Journal of SIOP—International Society of Pediatric Oncology (Societé Internationale d'Oncologie Pédiatrique. 2001; 36(6):612-22.
  8. Hayward K, Jeremy RJ, Jenkins S, Barkovich AJ, Gultekin SH, Kramer J, et al. Long-term neurobehavioral outcomes in children with neuroblastoma and opsoclonus-myoclonus-ataxia syndrome: relationship to MRI findings and anti-neuronal antibodies. The Journal of pediatrics. 2001; 139(4):552-9.
  9. Oguro K, Kobayashi JI, Aiba H, Hojo H. Opsoclonus-myoclonus syndrome with abnormal single photon emission computed tomography imaging. Pediatric neurology. 1997; 16(4):334-6.