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Keywords = Kinsbourne syndrome

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12 pages, 633 KiB  
Review
Review of Opsoclonus-Myoclonus Ataxia Syndrome in Pediatric Patients
by Mandy Hsu, Isbaah Tejani, Nidhi Shah, Rasaq Olaosebikan, Ashutosh Kumar and Sunil Naik
Children 2024, 11(3), 367; https://doi.org/10.3390/children11030367 - 19 Mar 2024
Cited by 3 | Viewed by 5566
Abstract
Opsoclonus-myoclonus ataxia syndrome (OMAS), also known as Kinsbourne syndrome, is a rare disorder that presents with myoclonus, ataxia, abnormal eye movements, irritability, and sleep disruptions, often in young children. We report a case of an infant barely 6 months old, with no significant [...] Read more.
Opsoclonus-myoclonus ataxia syndrome (OMAS), also known as Kinsbourne syndrome, is a rare disorder that presents with myoclonus, ataxia, abnormal eye movements, irritability, and sleep disruptions, often in young children. We report a case of an infant barely 6 months old, with no significant past medical history, who presented to the emergency department with tremors, jerking motions of the head and arms, and rapid eye movements. After an extensive workup, she was found to have a neuroblastoma, which was subsequently surgically removed via thoracotomy. Despite an initial improvement in symptoms post-resection, the patient’s symptoms recurred. She was subsequently treated with dexamethasone, intravenous immunoglobulin (IVIG), and rituximab. After treatment, the patient was noted to have mild global developmental delays but was otherwise well. This case report highlights the rare occurrence of OMAS in an infant barely 6 months old at diagnosis. Using the PubMed database, a systematic review was conducted to highlight the clinical presentation, diagnosis, and management of OMAS. Full article
(This article belongs to the Section Pediatric Neonatology)
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