Director Neuroscience Critical Care Service Community Hospital Munster
Introduction: Acute ischemic strokes effecting dorsomedial pons and medulla can result in interruption of apneustic and pneumotaxic canters, which coordinate the involuntary control of respiration and also effects the vasomotor control. Patients can clinically develop a potentially fatal syndrome of central hypoventilation or "Ondine's curse" which may accompany profound bradycardia and cardiac arrest. "Odine's curse" is named after a mythical story of a man doomed to a life where he could only breathe while awake and conscious, and when he sleeps was unable to breathe. We present a puzzling case of Ondine's curse due to dorsomedial pontomedullary ischemic stroke.
Description: 57-year-old man with history of hypertension, dyslipidemia and diabetes presented with new onset gait unsteadiness and vertiginous symptoms. He was noted to have a peripheral 7th cranial nerve paralysis, which was initially thought to be due to Bell's palsy. While sleeping in hospital patient became apneic and bradycardic, and unable to respond to environmental stimuli. He was intubated and shortly after was able to wake up. Following a short weaning trial he was extubated and did well throughout the day. MRI of brain showed a small ischemic stroke in the dorsomedial pontomedullary junction. The very next night while sleeping patient became apneic again with profound bradycardia leading to a cardiac arrest. Return of spontaneous circulation was achieved within six minutes with high quality CPR. He was extubated again and monitored with capnography. Noninvasive positive pressure ventilation was mandatorily started any time he sleeps and a cardiac pacemaker was placed.
Discussion: Respiratory complications of acute ischemic stroke including hypoventilation syndrome or Ondine's curse occur rarely with dorsomedial pontomedullary ischemic strokes. The left peripheral 7th cranial nerve in this patient was likely due to infarction involving intra-axial fascicular portion of facial nerve which is in close proximity to the pontine apneustic centers. This is an extremely unusual case and it is only with the recognition of this rare occurrence that this patient's life was saved with noninvasive positive pressure ventilation while sleeping and a cardiac pacemaker.
