Opsoclonus-myoclonus syndrome (Kinsbourne syndrome): narrative review and diagnostic-therapeutic update
DOI:
https://doi.org/10.70577/j729d392Keywords:
Kinsbourne Syndrome, Opsoclonus-Myoclonus, Neuroblastoma, Immunotherapy, Neuroimmunological Disorders.Abstract
Opsoclonus-myoclonus syndrome (OMS), also known as Kinsbourne syndrome, is a rare neurological disorder characterized by opsoclonus, myoclonus, ataxia, and neurobehavioral disturbances of predominantly immune-mediated origin. Due to its low prevalence and clinical heterogeneity, it remains a significant diagnostic and therapeutic challenge for healthcare professionals. The aim of this literature review was to analyze current scientific evidence regarding the clinical features, pathophysiology, diagnostic approaches, associated etiologies, and therapeutic options for opsoclonus-myoclonus syndrome. A narrative review was conducted through the consultation of major scientific databases, including PubMed, Scopus, Web of Science, ScienceDirect, and Google Scholar, selecting relevant studies on immunological mechanisms, paraneoplastic associations, diagnostic criteria, and immunomodulatory treatments. The findings indicate that neuroblastoma is the most common associated condition in pediatric patients, whereas paraneoplastic, autoimmune, and idiopathic etiologies predominate in adults. Furthermore, available evidence supports the involvement of B-cell-mediated immune mechanisms and inflammatory processes affecting the central nervous system. Regarding treatment, corticosteroids, adrenocorticotropic hormone, intravenous immunoglobulin, and rituximab have shown favorable outcomes in symptom control and relapse prevention. It is concluded that early diagnosis and prompt initiation of immunotherapy are essential to improve neurological outcomes and reduce long-term cognitive and functional sequelae in affected patients.
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Copyright (c) 2026 Jimmy Fernando Yaguana Torres,Jorge Abel Anoceto Díaz, Pamela Nicol Castillo Macias, Bryan Kenneth Lema Masache, Katheryn Estefanía Paguay Amaguaya (Autor/a)
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