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Autonomic dysfunction in childhood hypersomnia disorders.

Spoorthi Jagadish1, Wolfgang Singer1, Suresh Kotagal1

  • 1Department of Neurology, Mayo Clinic, Rochester, MN, USA.

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Summary

Orthostatic intolerance (OI) affects one-third of children with hypersomnia disorders, predominantly females. Early screening for OI is crucial as it is a treatable comorbidity impacting well-being.

Keywords:
Autonomic reflex screenComposite autonomic symptom score (CASS)Idiopathic hypersomniaNarcolepsyOrthostatic intolerance (OI)Postural tachycardia syndrome (POTS)

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Area of Science:

  • Neurology
  • Sleep Medicine
  • Autonomic Neuroscience

Background:

  • Orthostatic intolerance (OI) is a common autonomic dysfunction characterized by symptoms like light-headedness and palpitations upon standing.
  • OI can significantly impact a child's quality of life, with associated symptoms including headache, fatigue, and nausea.
  • The prevalence of autonomic symptoms, particularly OI, in pediatric hypersomnia disorders remains largely unexamined.

Purpose of the Study:

  • To investigate the prevalence and characteristics of orthostatic intolerance (OI) in children and adolescents diagnosed with hypersomnia disorders.
  • To determine if OI is associated with specific types of hypersomnia or demographic factors.
  • To assess the utility of autonomic reflex screen (ARS) testing in this patient population.

Main Methods:

  • Retrospective review of medical records for 89 children and adolescents diagnosed with narcolepsy types 1 or 2, idiopathic hypersomnia, KLS, or medical-condition-related hypersomnia.
  • Identification of patients presenting with OI symptoms (light-headedness, palpitations, fatigue, headache, nausea, abdominal pain) and those who underwent an autonomic reflex screen (ARS) battery.
  • Analysis of ARS results by an autonomic neurology specialist, excluding patients with comorbidities like sleep apnea or depression.

Main Results:

  • 37% (33/89) of pediatric patients with hypersomnia disorders presented with OI symptoms and underwent ARS testing.
  • OI was not linked to the severity of sleepiness or specific hypersomnia diagnoses but showed a significant female predominance (2:1 ratio).
  • OI symptoms were reproduced on tilt table testing in 17/33 subjects, with 5 meeting criteria for postural orthostatic tachycardia syndrome (POTS).

Conclusions:

  • Orthostatic intolerance (OI) is a common presenting symptom in approximately one-third of children with hypersomnia disorders, with a notable predilection for females.
  • A subset of these patients may meet criteria for postural orthostatic tachycardia syndrome (POTS).
  • Screening for autonomic dysfunction, including OI, is recommended in pediatric patients with hypersomnia as it represents a potentially treatable comorbidity affecting overall well-being.