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Ondine's curse with accompanying trigeminal and glossopharyngeal neuralgia secondary to medullary telangiectasia.
Neurocrit Care. 2010 Jun; 12(3):395-9.NC

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.

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Authors+Show Affiliations

Kapnadak SG
University of Virginia, Charlottesville, VA, USA. sgk5h@virginia.edu
Mikolaenko I
No affiliation info available
Enfield K
No affiliation info available
Gress DR
No affiliation info available
Nathan BR
No affiliation info available

MeSH

AgedCentral Nervous System Vascular MalformationsCritical CareDiagnosis, DifferentialDiaphragmElectric Stimulation TherapyElectroencephalographyFemaleGlossopharyngeal Nerve DiseasesHumansHypoventilationMagnetic Resonance ImagingMedulla OblongataRecurrenceRespiration, ArtificialSleep Apnea, CentralTrigeminal Neuralgia

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

20066515

Citation

Kapnadak, Siddhartha G., et al. "Ondine's Curse With Accompanying Trigeminal and Glossopharyngeal Neuralgia Secondary to Medullary Telangiectasia." Neurocritical Care, vol. 12, no. 3, 2010, pp. 395-9.
Kapnadak SG, Mikolaenko I, Enfield K, et al. Ondine's curse with accompanying trigeminal and glossopharyngeal neuralgia secondary to medullary telangiectasia. Neurocrit Care. 2010;12(3):395-9.
Kapnadak, S. G., Mikolaenko, I., Enfield, K., Gress, D. R., & Nathan, B. R. (2010). Ondine's curse with accompanying trigeminal and glossopharyngeal neuralgia secondary to medullary telangiectasia. Neurocritical Care, 12(3), 395-9. https://doi.org/10.1007/s12028-009-9321-x
Kapnadak SG, et al. Ondine's Curse With Accompanying Trigeminal and Glossopharyngeal Neuralgia Secondary to Medullary Telangiectasia. Neurocrit Care. 2010;12(3):395-9. PubMed PMID: 20066515.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Ondine's curse with accompanying trigeminal and glossopharyngeal neuralgia secondary to medullary telangiectasia. AU - Kapnadak,Siddhartha G, AU - Mikolaenko,Ivan, AU - Enfield,Kyle, AU - Gress,Daryl R, AU - Nathan,Barnett R, PY - 2010/1/13/entrez PY - 2010/1/13/pubmed PY - 2010/8/4/medline SP - 395 EP - 9 JF - Neurocritical care JO - Neurocrit Care VL - 12 IS - 3 N2 - 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. SN - 1556-0961 UR - https://www.unboundmedicine.com/medline/citation/20066515/Ondine's_curse_with_accompanying_trigeminal_and_glossopharyngeal_neuralgia_secondary_to_medullary_telangiectasia_ L2 - https://dx.doi.org/10.1007/s12028-009-9321-x DB - PRIME DP - Unbound Medicine ER -