Ondine's Curse with accompanying trigeminal and glossopharyngeal neuralgia secondary to medullary telangiectasia

Siddhartha G. Kapnadak, Ivan Mikolaenko, Kyle Enfield, Daryl R. Gress, Barnett R. Nathan

Research output: Contribution to journalArticle

12 Scopus citations

Abstract

Background Central hypoventilation syndrome ("Ondine's Curse") is an infrequent disorder that can lead to serious acute or chronic health consequences. This syndrome, especially in adults, is rare, and even less frequent in the absence of clear pathogenic lesions on MRI. In addition, we are not aware of any previously reported cases with associated cranial nerve neuralgias. Methods We describe a patient with baseline trigeminal and glossopharyngeal neuralgia, admitted with episodes of severe hypoventilatory failure of central origin, consistent with "Ondine's Curse". After evaluation, she was found to have a medullary capillary telangiectasia, thought to be the causative lesion, and which could explain her complete neurologic and hypoventilatory syndrome. The patient was treated with placement of a diaphragmatic pacing system, which has been effective thus far. Results This case illustrates the need for investigation of centrally mediated apnea, especially when co-occurring cranial nerve neuralgia is present and cardiopulmonary evaluation is negative. It provides an example of capillary telangiectasia as the causative lesion, one that to our knowledge has not been reported before. Conclusions Placement of a diaphragmatic pacing system was warranted and became lifesaving as the patient was deemed to be severely incapacitated by chronic ventilatory insufficiency.

Original languageEnglish (US)
Pages (from-to)395-399
Number of pages5
JournalNeurocritical Care
Volume12
Issue number3
DOIs
StatePublished - Jun 2010

Keywords

  • Central apnea
  • Diaphragmatic pacing
  • Medullary telangiectasia
  • Ondine's curse

ASJC Scopus subject areas

  • Clinical Neurology
  • Critical Care and Intensive Care Medicine

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