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



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.


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.


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.


Placement of a diaphragmatic pacing system was warranted and became lifesaving as the patient was deemed to be severely incapacitated by chronic ventilatory insufficiency.


Ondine’s curse Central apnea Diaphragmatic pacing Medullary telangiectasia Trigeminal neuralgia Glossopharyngeal neuralgia Neurovascular medullary compression Hypoventilation 


  1. 1.
    Marin-Sanabria EA, Kobayashi N, Miyake S, Kohmura E. Snoring associated with Ondine’s curse in a patient with brainstem glioma. J Clin Neurosci. 2006;13:370–3.CrossRefPubMedGoogle Scholar
  2. 2.
    Eckert DJ, Jordan AS, Merchia P, Malhotra A. Central sleep apnea: pathophysiology and treatment. Chest. 2007;131:595–607.CrossRefPubMedGoogle Scholar
  3. 3.
    Nannapaneni R, Behari S, Todd NV, Mendelow AD. Retracing “Ondine’s curse”. Neurosurgery. 2005;57:354–63.CrossRefPubMedGoogle Scholar
  4. 4.
    Idiopathic congenital central hypoventilation syndrome: diagnosis and management. American Thoracic Society. Am J Respir Crit Care Med. 1999;160:368–73.Google Scholar
  5. 5.
    Antic NA, Malow BA, Lange N, et al. PHOX2B mutation-confirmed congenital central hypoventilation syndrome: presentation in adulthood. Am J Respir Crit Care Med. 2006;174:923–7.CrossRefPubMedGoogle Scholar
  6. 6.
    Nakajima M, Katsura K, Hashimoto Y, Terasaki T, Uchino M. A case of Ondine curse associated with a medullary tumor. Rinsho Shinkeigaku. 2000;40:811–5.PubMedGoogle Scholar
  7. 7.
    Juan G, Ramon M, Ciscar MA, et al. Acute respiratory insufficiency as initial manifestation of brain stem lesions. Arch Bronconeumol. 1999;35:560–3.PubMedGoogle Scholar
  8. 8.
    Schestatsky P, Fernandes LN. Acquired Ondine’s curse: case report. Arq Neuropsiquiatr. 2004;62:523–7.PubMedGoogle Scholar
  9. 9.
    Warren HG, Kotsenas AL, Czervionke LF. Trigeminal and concurrent glossopharyngeal neuralgia secondary to lateral medullary infarction. AJNR Am J Neuroradiol. 2006;27:705–7.PubMedGoogle Scholar
  10. 10.
    Scaglione C, Salvi F, Riguzzi P, Vergelli M, Tassinari CA, Mascalchi M. Symptomatic unruptured capillary telangiectasia of the brain stem: report of three cases and review of the literature. J Neurol Neurosurg Psychiatry. 2001;71:390–3.CrossRefPubMedGoogle Scholar
  11. 11.
    Chervin RD, Guilleminault C. Diaphragm pacing: review and reassessment. Sleep. 1994;17:176–87.PubMedGoogle Scholar
  12. 12.
    Chervin RD, Guilleminault C. Diaphragm pacing for respiratory insufficiency. J Clin Neurophysiol. 1997;14:369–77.CrossRefPubMedGoogle Scholar

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

Personalised recommendations