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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MDPI AG
2024-03-01
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Series: | Children |
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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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format | Article |
id | doaj.art-5cd491e3898b44dab42d78965d474821 |
institution | Directory Open Access Journal |
issn | 2227-9067 |
language | English |
last_indexed | 2024-04-24T18:25:36Z |
publishDate | 2024-03-01 |
publisher | MDPI AG |
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series | Children |
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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