Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2023 Mar 24;87(4):e2022-0024.
doi: 10.5935/0004-2749.2022-0024. Online ahead of print.

Adult-onset idiopathic opsoclonus-myoclonus syndrome

Xiaohan Zhang  1 , Wenjing Yan  1 , Yuqiang Song  1 , Haifang Zhu  1 , Yanping Sun  1
Affiliations

Adult-onset idiopathic opsoclonus-myoclonus syndrome

Xiaohan Zhang et al. Arq Bras Oftalmol. .

Abstract
in English, Portuguese

Purpose: Opsoclonus-myoclonus syndrome is extremely uncommon in adults with an autoimmune pathophysiology. Because of the rarity of the syndrome, international recognition of opsoclonus-myoclonus-ataxia syndrome needs to be improved urgently. Therefore, the goal of this study was to raise the awareness of the opsoclonus-myoclonus-ataxia syndrome and help doctors in better diagnosing and using immunotherapy.

Methods: We present a case study of an adult-onset case of idiopathic opsoclonus-myoclonus syndrome characterized by spontaneous arrhythmic multidirectional conjugate eye movements, myoclonus, ataxia, sleep disorders, and intense fear. Additionally, we conduct a literature search and summarize the pathophysiology, clinical presentation, diagnosis, and treatment of opsoclonus-myoclonus-ataxia syndrome.

Results: Immunotherapies successfully treated the patient's opsoclonus, myoclonus, and ataxia. Further, the article also includes an update summary of the opsoclonus-myoclonus-ataxia syndrome.

Conclusion: The prevalence of residual sequela in adults with opsoclonus-myoclonus-ataxia syndrome is low. Early diagnosis and treatment may result in a better prognosis. Furthermore, combined immunotherapy is expected to reduce the incidence of refractory and reoccurring opsoclonus-myoclonus-ataxia syndrome.

Objetivo: A síndrome de opsoclonia-mioclonia é extremamente rara em adultos e tem uma fisiopatologia autoimune. Devido à raridade dessa síndrome, o reconhecimento da síndrome de opsoclonia-mioclonia-ataxia precisa melhorar urgentemente em todo o mundo. Assim sendo, este estudo visou aumentar a conscientização sobre a síndrome de opsoclonia-mioclonia-ataxia e ajudar os médicos para um melhor diagnóstico e o uso correto da imunoterapia.

Métodos: Este é o relato de um caso adulto de síndrome de opsoclonia-mioclonia idiopática com movimentos oculares conjugados, multidirecionais, arrítmicos e espontâneos, mioclonia, ataxia, distúrbios do sono e medo intenso. Além disso, foram pesquisadas as publicações recentes relevantes e resumiu-se a fisiopatologia, a apresentação clínica, o diagnóstico e o tratamento da síndrome de opsoclonia-mioclonia-ataxia.

Resultados: A paciente recuperou-se totalmente da opsoclonia, da mioclonia e da ataxia através de imunoterapia. O artigo também fornece um resumo atualizado sobre a síndrome de opsoclonia-mioclonia-ataxia.

Conclusão: Adultos com síndrome de opsoclonia-mioclonia-ataxia têm uma baixa frequência de sequelas residuais. O diagnóstico e o tratamento precoces podem levar a melhores prognósticos. Espera-se que a imunoterapia combinada reduza a incidência da síndrome de opsoclonia-mioclonia-ataxia refratária e recorrente.

PubMed Disclaimer

References

    1. Bhatia P, Heim J, Cornejo P, Kane L, Santiago J, Kruer MC. Opso-clonus-myoclonus-ataxia syndrome in children. J Neurol. 2022;260(2):750–757. - PubMed
    1. Pranzatelli MR. The immunopharmacology of the opsoclonus-myoclonus syndrome. Clin Neuropharmacol. 1996;19(1):1–47. - PubMed
    1. Khadilkar S, Benny R. Opsoclonus myoclonus ataxia syndrome. Neurol India. 2018;66(5):1293–1294. - PubMed
    1. Kang BH, Kim JI. Opsoclonus-myoclonus syndrome associated with mumps virus infection. J Clin Neurol. 2014;10(3):272–275. - PMC - PubMed
    1. Desai SD, Gandhi FR, Vaishnav A. Opsoclonus Myoclonus Syndrome: a rare manifestation of dengue infection in a child. [2020 July 21];J Pediatr Neurosci [Internet] 2018 13(4):455–458. Available from: Opsoclonus Myoclonus Syndrome: A Rare Manifestation of Dengue Infection in a Child - PMC (nih.gov) - PMC - PubMed

LinkOut - more resources