Review of Opsoclonus-Myoclonus Ataxia Syndrome in Pediatric Patients

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 m...

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Main Authors: Mandy Hsu, Isbaah Tejani, Nidhi Shah, Rasaq Olaosebikan, Ashutosh Kumar, Sunil Naik
Format: Article
Language:English
Published: MDPI AG 2024-03-01
Series:Children
Subjects:
Online Access:https://www.mdpi.com/2227-9067/11/3/367
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author Mandy Hsu
Isbaah Tejani
Nidhi Shah
Rasaq Olaosebikan
Ashutosh Kumar
Sunil Naik
author_facet Mandy Hsu
Isbaah Tejani
Nidhi Shah
Rasaq Olaosebikan
Ashutosh Kumar
Sunil Naik
author_sort Mandy Hsu
collection DOAJ
description 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.
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spelling doaj.art-5cd491e3898b44dab42d78965d4748212024-03-27T13:31:15ZengMDPI AGChildren2227-90672024-03-0111336710.3390/children11030367Review of Opsoclonus-Myoclonus Ataxia Syndrome in Pediatric PatientsMandy Hsu0Isbaah Tejani1Nidhi Shah2Rasaq Olaosebikan3Ashutosh Kumar4Sunil Naik5University Park Program, Penn State College of Medicine, State College, PA 16803, USAMedical College, Aga Khan University, Karachi P.O. Box 8842, PakistanDepartment of Pediatric Hematology/Oncology, Penn State Health Milton S. Hershey Medical Center, Hershey, PA 17033, USADepartment of Pediatric Hematology/Oncology, Penn State Health Milton S. Hershey Medical Center, Hershey, PA 17033, USADepartment of Pediatric Neurology, Penn State Health Milton S. Hershey Medical Center, Hershey, PA 17033, USADepartment of Pediatric Neurology, Penn State Health Milton S. Hershey Medical Center, Hershey, PA 17033, USAOpsoclonus-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.https://www.mdpi.com/2227-9067/11/3/367opsoclonus-myoclonus ataxia syndromeKinsbourne syndromedancing eye dancing feet syndromepediatricneuroblastomaparaneoplastic syndrome
spellingShingle Mandy Hsu
Isbaah Tejani
Nidhi Shah
Rasaq Olaosebikan
Ashutosh Kumar
Sunil Naik
Review of Opsoclonus-Myoclonus Ataxia Syndrome in Pediatric Patients
Children
opsoclonus-myoclonus ataxia syndrome
Kinsbourne syndrome
dancing eye dancing feet syndrome
pediatric
neuroblastoma
paraneoplastic syndrome
title Review of Opsoclonus-Myoclonus Ataxia Syndrome in Pediatric Patients
title_full Review of Opsoclonus-Myoclonus Ataxia Syndrome in Pediatric Patients
title_fullStr Review of Opsoclonus-Myoclonus Ataxia Syndrome in Pediatric Patients
title_full_unstemmed Review of Opsoclonus-Myoclonus Ataxia Syndrome in Pediatric Patients
title_short Review of Opsoclonus-Myoclonus Ataxia Syndrome in Pediatric Patients
title_sort review of opsoclonus myoclonus ataxia syndrome in pediatric patients
topic opsoclonus-myoclonus ataxia syndrome
Kinsbourne syndrome
dancing eye dancing feet syndrome
pediatric
neuroblastoma
paraneoplastic syndrome
url https://www.mdpi.com/2227-9067/11/3/367
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