Review of Opsoclonus-Myoclonus Ataxia Syndrome in Pediatric Patients
Mandy Hsu1, Isbaah Tejani2, Nidhi Shah3
1University Park Program, Penn State College of Medicine, State College, PA 16803, USA.
Insights
Opsoclonus-myoclonus ataxia syndrome (OMAS) in a 6-month-old infant was linked to neuroblastoma. Early diagnosis and treatment, including surgery and immunotherapy, are crucial for managing this rare neurological disorder.
Area of Science:
- Pediatric Neurology
- Neuro-oncology
- Immunology
Background:
- Opsoclonus-myoclonus ataxia syndrome (OMAS), or Kinsbourne syndrome, is a rare neurological disorder characterized by myoclonus, ataxia, and abnormal eye movements.
- OMAS typically affects young children and can be associated with neuroblastoma, a pediatric cancer.
- Early identification and intervention are critical for improving outcomes in OMAS patients.
Purpose of the Study:
- To report a rare case of OMAS in a 6-month-old infant.
- To highlight the association between OMAS and neuroblastoma in early childhood.
- To review the clinical presentation, diagnosis, and management strategies for OMAS.
Main Methods:
- A case report of a 6-month-old infant presenting with OMAS symptoms.
- Diagnostic workup including identification of neuroblastoma.
- Systematic literature review using PubMed on OMAS clinical presentation, diagnosis, and management.
Main Results:
- The infant was diagnosed with neuroblastoma, which was surgically removed.
- Symptoms recurred post-surgery and were treated with dexamethasone, IVIG, and rituximab.
- The patient experienced mild global developmental delays but showed overall improvement.
Conclusions:
- OMAS can occur in very young infants and is strongly associated with neuroblastoma.
- Multimodal treatment including surgery and immunotherapy is effective in managing OMAS.
- Long-term monitoring for developmental outcomes is essential in affected children.
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 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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