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Sleep in infants with congenital myasthenic syndromes

Serena Caggiano1, Sonia Khirani2, Elisabetta Verrillo3

  • 1Respiratory Unit, Academic Pediatric Department, Bambino Gesù Children's Hospital, Rome, Italy; AP-HP, Hôpital Necker-Enfants Malades, Pediatric Noninvasive Ventilation and Sleep Unit, Paris, France.

European Journal of Paediatric Neurology : EJPN : Official Journal of the European Paediatric Neurology Society
|July 31, 2017
PubMed

Insights

Infants with congenital myasthenic syndrome (CMS) often have abnormal breathing during sleep, requiring intervention. Heart rate (HR) variability was not significantly altered in these infants during sleep studies.

Area of Science:

  • Pediatric Neurology
  • Sleep Medicine
  • Genetics

Background:

  • Congenital myasthenic syndrome (CMS) infants are susceptible to brief resolved unexplained events (BRUE) and sleep-disordered breathing.
  • Understanding sleep patterns and cardiorespiratory events in CMS is crucial for management.

Purpose of the Study:

  • To investigate sleep characteristics in infants with CMS.
  • To specifically analyze heart rate (HR) variability in relation to respiratory events.

Main Methods:

  • Overnight polygraphy was conducted on 5 infants with CMS.
  • Heart rate (HR) variations (bradycardia/tachycardia) associated with respiratory events were analyzed.
  • Apnea-hypopnea index (AHI) and transcutaneous gas exchange were assessed.

Main Results:

  • All infants exhibited abnormal AHI, with higher values in younger infants.
  • Three infants required ventilatory support post-study due to high AHI or hypoventilation.
  • No significant abnormalities in HR variations were detected during sleep studies.

Conclusions:

  • Sleep polygraphy is clinically valuable for identifying respiratory abnormalities in CMS infants.
  • Early identification of sleep-disordered breathing and hypoventilation can guide timely intervention.
  • While respiratory events are common, significant HR variability abnormalities were not observed in this cohort.
Abstract

Keywords:
Brief resolved unexplained eventCongenital myasthenic syndromeHeart ratePoly(somno)graphySleepVentilatory support

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