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Published on: October 11, 2018
Sleep in pediatric neuromuscular disorders
John E Pascoe1, Alexander Zygmunt2, Zarmina Ehsan3
1Division of Pulmonary and Sleep Medicine, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, United States; Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH, United States.
Insights
Sleep disordered breathing (SDB) affects many children with neuromuscular disorders (NMD). Early diagnosis and treatment, often with ventilation, are crucial to prevent serious health issues.
Area of Science:
- Pediatric Pulmonology
- Neurology
- Sleep Medicine
Background:
- Sleep disordered breathing (SDB) is common in children with neuromuscular disorders (NMD).
- Respiratory muscle weakness, altered respiratory drive, and scoliosis exacerbate sleep stress in this population.
- Untreated SDB can lead to cardiovascular, metabolic, and neurocognitive complications.
Conclusions:
- Clinicians should maintain a high suspicion for SDB in children with NMD.
- Individualized assessment is necessary due to varied muscle group involvement.
- Prompt diagnosis and management of SDB are vital for improving outcomes in pediatric NMD.
Abstract:
Sleep disordered breathing (SDB) is prevalent among children with neuromuscular disorders (NMD). The combination of respiratory muscle weakness, altered drive, and chest wall distortion due to scoliosis make sleep a stressful state in this population. Symptomatology can range from absent to snoring, nocturnal awakenings, morning headaches, and excessive daytime sleepiness. Sequelae of untreated SDB includes cardiovascular effects, metabolic derangements, and neurocognitive concerns which can be compounded by those innate to the NMD. The clinician should have a low threshold for obtaining polysomnography and recognize the nuances of individual disorders due to disproportionately impacted muscle groups such as hypoventilation in ambulating patients from diaphragm weakness. Non-invasive or invasive ventilation are the mainstay of treatment. In this review we explore the diagnosis and treatment of SDB in children with various NMD.
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