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Background: Opsoclonus-myoclonus syndrome (OMS) is an immune-mediated disorder that affects the central nervous system. It is recognized as a paraneoplastic syndrome highly associated with neuroblastic tumors. In Mexico, accurate epidemiological data are lacking and there are no well-structured protocols aimed at standardizing a treatment regimen in patients with OMS. Aim: This study aims to describe the clinical features, etiological factors, and treatment related to OMS in Mexican children. Patients and Methods: A retrospective study was performed over a 10-year period (2006-2016), using the database of the "Hospital Infantil de México Federico Gómez". The patients were divided into two groups (tumor vs. no tumor) to determine if there were significant demographic differences between the subgroups. The demographic variables were compared between groups using the Student's t-test. Results: Eleven patients were identified. The median age at the onset of the first clinical manifestation was 23 ± 14 months and the median age at diagnosis was 26 ± 15 months. The time delay between the onset of symptoms and diagnosis was approximately 3 months. Conclusion: In Mexico, there is a paucity of experience regarding the clinical presentation of OMS, and due to the infrequency of this condition, it is rarely diagnosed in our population since most pediatricians are unaware of the existence of the syndrome. The findings of this paper are consistent with the previous studies in terms of a closely equal sex ratio, predominance of Stage I tumors, a greater number of female patients with a paraneoplastic etiology, high relapse rates, and frequent neuropsychiatric morbidity. [ABSTRACT FROM AUTHOR]

Kinsbourne syndrome, also known as “Opsoclonus-myoclonus syndrome” or “Dancing eyes syndrome” is a rare condition characterized by rapid, irregular, multi-directional eye movements (opsoclonus), myoclonic movements in the trunk, face and/or limbs and ataxia. It occurs in children aged between 6 and 36 months. Its etiology may be paraneoplasic (neuroblastoma), non-paraneoplasic (infectious processes) or idiopathic. Regardless of its etiology, immunosuppressive drugs have been used in order to reduce the formation of antibodies possibly involved in the pathophysiology. We report the case of a 21 month-old girl with this syndrome secondary to an infectious respiratory illness. She had ataxia, opsoclonus, upper limbs myoclonus, irritability and altered sleep pattern. Neuroblastoma was ruled out. Initial management was done with methylprednisolone pulses, followed by oral prednisolone. She had progressive clinical improvement, and is currently asymptomatic with no sequelae. In these patients a paraneoplasic syndrome should always be ruled out.

Opsoclonus-myoclonus syndrome (OMS) is a rare, autoimmune neurological disorder that is poorly recognized and undertreated. Neuroblastoma is found in one half of the cases. Because of the high incidence of spontaneous regression of neuroblastoma, it is unknown whether not finding a tumor means there was none. To define demographic trends and the standard of care in the first large series of OMS, 105 children were recruited over a 13-year period in a retrospective questionnaire survey. Children with and without a tumor differed little in viral-like prodrome and neurological symptoms. Earliest neurological symptoms were staggering and falling, leading to a misdiagnosis of acute cerebellitis. Later symptoms included body jerks, drooling, refusal to walk or sit, speech problems, decreased muscle tone, opsoclonus, and inability to sleep. Tumor resection alone did not provide adequate therapy for most. Adrenocorticotropic hormone (ACTH), prednisone, and intravenous immunoglobulin were used with equal frequency, but ACTH was associated with the best early response. More than one half of the children had relapses. Residual behavioral, language, and cognitive problems occurred in the majority. The delay in diagnosis (11 weeks) and initiation of treatment (17 weeks) is unacceptably long. [ABSTRACT FROM PUBLISHER]