1441 Severe Sleep Onset Central Apneas in a Young Female with Seizure Disorder and Arnold Chiari Malformation Post-Decompression

Daniel Rongo; Janey Dudley; Likhita Shaik; Michael Ibarra; Won Lee; Venjata Mukkavilli

doi:10.1093/sleep/zsaf090.1441

Introduction This case highlights the challenges towards treating a patient with isolated sleep-onset central apnea. I present a challenging case of a young female with a sleep-onset central sleep apnea treated with a conservative management approach leading to significant improvement. Report of case A 30-year-old female with a medical history of cognitive delay, Lennox-Gastaut with intractable seizures, and Arnold Chiari malformation status post-decompression (10 years prior) was referred for evaluation due to episodes of apnea observed by her mother. She notably had her clonazepam discontinued a few months prior to this presentation. During these events, she experienced oxygen desaturations to the 60s lasting up to 2–3 minutes. Labs suggest a chronic hypocapnic state at baseline. Polysomnography revealed isolated severe sleep-onset central apneas without significant sleep-disordered breathing during the rest of the night. Total apnea-hypopnea index (AHI) was minimal, but the severity of apneic-episodes during sleep onset warranted immediate attention. Continuous EEG was unremarkable. BiPAP ST and ASV were considered but given her developmental delay and risk of worsening apneic episodes this was deferred. The patient was started on low-dose clonazepam and empiric oxygen therapy at 2 liters/min, resulting in significant improvement of central sleep apnea events. No further apneas have occurred. Conclusion The onset of sleep causes ventilatory instability with transition from alpha to theta activity. During this transitory state, wakeful CO2 eupnea converges with the emerging CO2 apnea threshold of sleep which predisposes to apneic episodes. Although normal in healthy individuals, various predisposing factors such as a low CO₂ reserve, heightened chemoreceptor sensitivity, or increased loop gain can significantly increase the risk and magnitude of these central apneic events. The challenge in this case was in limiting excessive ventilation with the use of potentially deleterious non-invasive ventilation while maintaining her drive to breath. Our intervention targeted normalizing her baseline hypocapnia with pharmacotherapy and supplemental oxygen which improved her symptoms thereafter on follow-up visits, avoiding the need for intermittent non-invasive ventilation.

1441 Severe Sleep Onset Central Apneas in a Young Female with Seizure Disorder and Arnold Chiari Malformation Post-Decompression

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