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Updated: Oct 9, 2026

Drug-Induced Sleep Endoscopy (DISE) with Target Controlled Infusion (TCI) and Bispectral Analysis in Obstructive Sleep Apnea
07:54

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[Effect of diazepam on apnea attacks in a 6-year-old girl with Rett syndrome: a polysomnographic study]

M Kurihara1, K Kumagai, Y Nakae

  • 1Department of Pediatrics, Kanagawa Rehabilitation Center, Atsugi, Kanagawa.

No to Hattatsu = Brain and Development
|February 24, 2001
PubMed

Insights

Oral diazepam effectively reduced severe apnea attacks in a child with Rett syndrome. Polysomnography confirmed a significant decrease in breathing pauses, improving respiratory function.

Area of Science:

  • Neurology
  • Pediatrics
  • Sleep Medicine

Background:

  • Rett syndrome is a rare neurodevelopmental disorder primarily affecting girls, often associated with significant respiratory abnormalities.
  • Apnea attacks, particularly during wakefulness following hyperventilation, represent a serious clinical challenge in Rett syndrome management.

Observation:

  • A 6-year-old girl with Rett syndrome presented with severe apnea attacks triggered by hyperventilation while awake.
  • Initial treatment with low-dose oral diazepam (1.5 mg/day) demonstrated a notable positive effect on breathing disturbances.

Findings:

  • Polysomnography objectively quantified the efficacy of diazepam, revealing a substantial reduction in apnea attacks from 15.7 to 0.6 per hour.
  • The duration of the longest apnea episodes decreased significantly from 40 seconds to 18 seconds with diazepam treatment.

Implications:

  • These findings underscore the potential therapeutic role of diazepam in managing hyperventilation-induced apnea in Rett syndrome.
  • The results support existing hypotheses implicating the cerebral cortex and reticular formation in the pathophysiology of respiratory dysfunction in Rett syndrome.

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