Neurocritical Care

, Volume 12, Issue 3, pp 395–399

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
Practical Pearl

DOI: 10.1007/s12028-009-9321-x

Cite this article as:
Kapnadak, S.G., Mikolaenko, I., Enfield, K. et al. Neurocrit Care (2010) 12: 395. doi:10.1007/s12028-009-9321-x

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.

Keywords

Ondine’s curseCentral apneaDiaphragmatic pacingMedullary telangiectasiaTrigeminal neuralgiaGlossopharyngeal neuralgiaNeurovascular medullary compressionHypoventilation

Copyright information

© Springer Science+Business Media, LLC 2010

Authors and Affiliations

  • Siddhartha G. Kapnadak
    • 1
  • Ivan Mikolaenko
    • 1
  • Kyle Enfield
    • 1
  • Daryl R. Gress
    • 1
  • Barnett R. Nathan
    • 1
  1. 1.University of VirginiaCharlottesvilleUSA