Central sleep apnea in patients with pontine tegmental cap dysplasia treated with supplemental oxygen: a case report.

Abstract

This is the first case report of sleep-disordered breathing in patients with pontine tegmental cap dysplasia, a very rare neurological disorder characterized by an anatomic malformation in the pons. Patients present with hypotonia, cognitive dysfunction, and cranial nerve palsies (eg, hearing loss, trigeminal anesthesia, and swallow dysfunction). Extensive studies have demonstrated the relevance of different pontine neuronal nuclei in breathing regulation, which are structurally abnormal in pontine tegmental cap dysplasia. We present detailed polysomnography data for 3 patients aged 41 years, 20 years, and 1.5 years revealing significant central sleep apnea. We discuss our experience with managing their sleep-disordered breathing in the setting of multiple cranial nerve palsies and corneal anesthesia, and its relative contraindication of noninvasive positive pressure ventilation treatment.

CITATION:

Ju Wang J-D, Doherty D, Ramirez J-M, Chen M. Central sleep apnea in patients with pontine tegmental cap dysplasia treated with supplemental oxygen: a case report. J Clin Sleep Med. 2023;19(4):843-849.

• Authors: Jia-Der Ju Wang, Dan Doherty, Jan-Marino Ramirez, Maida Chen
• Journal: Journal of clinical sleep medicine : JCSM : official publication of the American Academy of Sleep Medicine (J Clin Sleep Med) Vol. 19 Issue 4 Pg. 843-849 (04 01 2023) ISSN: 1550-9397 [Electronic] United States
• PMID: 36708265 (Publication Type: Case Reports, Research Support, N.I.H., Extramural)
• Copyright: © 2023 American Academy of Sleep Medicine.

Chemical References

• Oxygen

Topics

• Humans
• Sleep Apnea, Central (complications, therapy)
• Cranial Nerve Diseases
• Abnormalities, Multiple
• Positive-Pressure Respiration
• Oxygen