Respiratory control and respiratory sensation in a patient with a ganglioglioma within the dorsocaudal brain stem

Am J Respir Crit Care Med. 2000 Jun;161(6):2100-6. doi: 10.1164/ajrccm.161.6.9904087.


We encountered a young woman with severe central sleep apnea caused by a medullary glioma located slightly dorsal to and to the right of the midline, a region not generally associated with CO(2) chemosensitivity. The patient had normal spirometric readings, lung volumes, diffusing capacity, maximal inspiratory pressure, and alveolar-arterial oxygen difference. While awake, she displayed marked irregularity in her breathing pattern; her end-tidal CO(2) (FET(CO(2))) ranged from 5.3 to 10.9%. During voluntary hyperpnea, she could quickly reduce her FET(CO(2)) to 4.2%, but her PCO(2) did not change after administration of acetazolamide or progesterone. Like patients with congenital central hypoventilation syndrome (CCHS), our patient had a relatively intact ventilatory response to exercise; her PCO(2) was high at the start of exercise and increased slightly thereafter. In contrast to CCHS patients, however, our patient had an intact hypoxic ventilatory response (DeltaVE/ DeltaSa(O(2)) = -0.37 L/min/Sa(O(2))). In further contrast to CCHS patients, our patient had a very short breathholding time and described a sensation of air hunger as the factor limiting her breathholding ability. Her heart rate and blood pressure responses to the Valsalva maneuver were normal.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Brain Mapping
  • Brain Stem Neoplasms / diagnosis
  • Brain Stem Neoplasms / physiopathology*
  • Carbon Dioxide / blood
  • Chemoreceptor Cells / physiopathology
  • Female
  • Follow-Up Studies
  • Ganglioglioma / diagnosis
  • Ganglioglioma / physiopathology*
  • Humans
  • Magnetic Resonance Imaging
  • Medulla Oblongata / pathology
  • Medulla Oblongata / physiopathology*
  • Oxygen / blood
  • Pulmonary Ventilation / physiology
  • Sleep Apnea, Central / diagnosis
  • Sleep Apnea, Central / physiopathology*


  • Carbon Dioxide
  • Oxygen