Opsoclonus-myoclonus-ataxia syndrome

Authors

  • Darlis Yisel Carcasés-Hernández Hospital Clínico Quirúrgico "Hermanos Ameijeiras". Servicio de Neurología. La Habana.
  • Rolando Zamora-Fung Hospital Clínico Quirúrgico "Hermanos Ameijeiras". Servicio de Neurología. La Habana.
  • Grisel Pérez-Rosales Hospital Clínico Quirúrgico "Hermanos Ameijeiras". Servicio de Neurología. La Habana.
  • Leda Fernández-Cué Hospital Clínico Quirúrgico "Hermanos Ameijeiras". Servicio de Neurología. La Habana.
  • Irma Regla Olivera-Leal Hospital Clínico Quirúrgico "Hermanos Ameijeiras". Servicio de Neurología. La Habana.

Keywords:

ataxia, esteroides, inmunoglobulina intravenosa, síndrome de Opsoclonía-Mioclonía

Abstract

Introduction: opsoclonus-Myoclonus-Ataxia Syndrome, also known as "Kinsbourne syndrome" or "Dancing Eye Syndrome," is a rare neurological condition characterized by the acute or subacute onset of a disorder involving saccadic eye movements, called opsoclonus, limb myoclonus, and gait ataxia.

Objective: to present an uncommon case in neurological clinical practice and its evolution with the treatment.

Case Report: a 52-year-old female patient with a one-month history of symptoms, including fever, arthralgia, myalgia, holocranial headache, and retroocular pain, initially diagnosed as arbovirosis. Two weeks later, she suddenly developed generalized tremors at rest and during action, gait incoordination, falls, and abnormal eye movements. Progressively, hand myoclonus, photophobia, drowsiness alternating with periods of irritability, and difficulty articulating words appeared. A clinical diagnosis of Kinsbourne syndrome of post-infectious origin was made, and she received immunomodulatory and immunosuppressive treatments at different evolutionary stages, with satisfactory progression and total remission.

Conclusions: kinsbourne syndrome requires early initiation of immunomodulatory and immunosuppressive treatments and identification of the etiological factor to determine a satisfactory outcome and prevent long-term complications and relapses.

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References

1. Zhang X, Yan W, Song Y, Zhu H, Sun Y. Adult-onset idiopathic opsoclonus-myoclonus syndrome. Arq Bras Oftalmol [Internet]. 2023 [citado 23 de febrero de 2025];87(4):e2022-0024. Disponible en: https://doi.org/10.5935/0004-2749.2022-0024

2. Balint B. Autoimmune Movement Disorders. Continuum (N Y) [Internet]. 2024 [citado 20 de febrero de 2025];30(4):1088-109. Disponible en: https://doi.org/10.1212/CON.0000000000001455

3. Marsili L, Marcucci S, La Porta J, Chirra M, Espay AJ, Colosimo C. Paraneoplastic Neurological Syndromes of the Central Nervous System: Pathophysiology, Diagnosis, and Treatment. Biomedicines [Internet]. 2023 [citado 21 de febrero de 2025];11(5):1406. Disponible en: https://doi.org/10.3390/biomedicines11051406

4. Pohl KR, Pritchard J, Wilson J. Neurological sequelae of the dancing eye syndrome. Eur J Pediatr [Internet]. 1996 [citado 24 de febrero de 2025];155(3):237-44. Disponible en: https://doi.org/10.1007/BF01953945

5. Almudhry M, Wagner MW, Longoni G, Yea C, Vidarsson L, Ertl-Wagner B, et al. Brain Volumes in Opsoclonus-Myoclonus Ataxia Syndrome: A Longitudinal Study. J Child Neurol [Internet]. 2024 [citado 20 de febrero de 2025];39(3-4):129-34. Disponible en: https://doi.org/10.1177/08830738241240181

6. Yıldırım M, Öncel İ, Bektaş Ö, Tanalı G, Şahin S, Kutluk T, et al. Clinical features and outcomes of opsoclonus myoclonus ataxia syndrome. Eur J Paediatr Neurol [Internet]. 2022 [citado 19 de febrero de 2025];41:19-26. Disponible en: https://doi.org/10.1016/j.ejpn.2022.09.002

7. Costa PA, Costa BMLA, Rozenbaum G, Barreto-Coelho P. Anti-Ma paraneoplastic opsoclonus-myoclonus syndrome. BMJ Case Rep [Internet]. 2021 [citado 21 de febrero de 2025];14(5):e243136. Disponible en: https://doi.org/10.1136/bcr-2021-243136

8. Fedoseeva IF, Goncharenko A V, Poponnikova T V, Glebova I V, Pinevich OS, Goncharenko VA. Paraneoplastic opsoclonus-myoclonus syndrome. Zhurnal Nevrol i psikhiatrii Im SS Korsakova [Internet]. 2024 [citado 23 de febrero de 2025];124(12):165-70. Disponible en: https://doi.org/10.17116/jnevro2024124121165

9. Finsterer J, Scorza FA. Opsoclonus Myoclonus Ataxia Syndrome Due to SARS-CoV-2. Neuroophthalmology [Internet]. 2023 [citado 27 de febrero de 2025];47(1):1-6. Disponible en: https://doi.org/10.1080/01658107.2022.2128378

10. Haridas A, Ravi P. Opsoclonus-myoclonus syndrome caused by organophosphate poisoning. Pract Neurol [Internet]. 2023 [citado 20 de febrero de 2025];23(3):243-5. Disponible en: https://doi.org/10.1136/pn-2022-003612

11. Alburaiky S, Dale RC, Crow YJ, Jones HF, Wassmer E, Melki I, et al. Opsoclonus-myoclonus in Aicardi-Goutières syndrome. Dev Med Child Neurol [Internet]. 2021 [citado 4 de febrero de 2025];63(12):1483-6. Disponible en: https://doi.org/10.1111/dmcn.14969

12. Gupta A, Kundal R, Pandey M. Opsoclonus-myoclonus Syndrome with Neuroblastoma in Children and their Anaesthetic Management. J Coll Physicians Surg Pak [Internet]. 2022 [citado 26 de febrero de 2025];32(8):1086-8. Disponible en: https://doi.org/10.29271/jcpsp.2022.08.1086

13. Zarco L, Gil L, Millán SP, Pretelt F. Síndrome de opsoclonus-mioclonus-ataxia (OMA) parainfeccioso secundario a infección por citomegalovirus. Acta Neurológica Colomb [Internet]. 2011 [citado 27 de febrero de 2025];27(4):237-42. Disponible en: http://www.scielo.org.co/scielo.php?script=sci_arttext&pid=S0120-87482011000400007&lng=en.

Published

2026-01-21

How to Cite

1.
Carcasés-Hernández DY, Zamora-Fung R, Pérez-Rosales G, Fernández-Cué L, Olivera-Leal IR. Opsoclonus-myoclonus-ataxia syndrome. Rev Cubana Neurol Neurocir [Internet]. 2026 Jan. 21 [cited 2026 Mar. 6];14:e642. Available from: https://revneuro.sld.cu/index.php/neu/article/view/642

Issue

Section

Case presentation