Atormac
Neurology India
menu-bar5 Open access journal indexed with Index Medicus
  Users online: 1833  
 Home | Login 
About Editorial board Articlesmenu-bullet NSI Publicationsmenu-bullet Search Instructions Online Submission Subscribe Videos Etcetera Contact
  Navigate Here 
 Search
 
  » Next article
  » Previous article 
  » Table of Contents
  
 Resource Links
  »  Similar in PUBMED
 »  Search Pubmed for
 »  Search in Google Scholar for
 »Related articles
  »  Article in PDF (132 KB)
  »  Citation Manager
  »  Access Statistics
  »  Reader Comments
  »  Email Alert *
  »  Add to My List *
* Registration required (free)  

 
  In this Article
 »  Abstract
 »  Introduction
 »  Case Report
 »  Discussion
 »  References
 »  Article Figures

 Article Access Statistics
    Viewed5725    
    Printed156    
    Emailed1    
    PDF Downloaded415    
    Comments [Add]    
    Cited by others 13    

Recommend this journal

 


 
CASE REPORT
Year : 2009  |  Volume : 57  |  Issue : 2  |  Page : 206-207

Ondine's curse after brainstem infarction


Department of Neurology and Neurosurgery, Federal University of São Paulo, São Paulo, Brazil

Date of Acceptance31-Mar-2009

Correspondence Address:
Jose Luiz Pedroso
Pedro de Toledo Street, 544/206, São Paulo, São Paulo
Brazil
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.51298

Rights and Permissions

 » Abstract 

This report describes a rare case of acquired Ondine's curse. The patient developed central sleep apnea syndrome named Ondine's curse after a brainstem infarction. Lesions involving the descending medullocervical pathways that subserve automatic breathing can result in this syndrome.


Keywords: Brainstem infarction, central sleep apnea, ondine′s curse


How to cite this article:
Pedroso JL, Baiense RF, Scalzaretto AP, Neto PB, Teixeira de Gois AF, Ferraz ME. Ondine's curse after brainstem infarction. Neurol India 2009;57:206-7

How to cite this URL:
Pedroso JL, Baiense RF, Scalzaretto AP, Neto PB, Teixeira de Gois AF, Ferraz ME. Ondine's curse after brainstem infarction. Neurol India [serial online] 2009 [cited 2019 Aug 23];57:206-7. Available from: http://www.neurologyindia.com/text.asp?2009/57/2/206/51298



 » Introduction Top


Ondine's curse is a rare and severe condition and is characterized by loss of automatic respiration during sleep and preserved voluntary breathing. [1],[2] The term stems from a mythological European tradition in which Ondine, a water nymph, condemns her unfaithful lover to loss of all movements and functions that do not require conscious will. A similar situation occurs in Ondine's curse. Patients with this condition are compelled to remain awake in the nights lest they stop breathing and they need night-time mechanical ventilation to survive. [1] In 1962, Severinghaus and Mitchell [2] were the first to use the term 'Ondine's curse' for patients who developed long periods of apnea but would breath on command. The diagnostic criteria for Ondine's curse are not well established. The proposed criteria include: Hypercapnia during non-REM sleep; normal PO 2 (arterial blood gas, (ABG)) during voluntary breathing when awake; alveolar hypoventilation during sleep; and exclusion of pulmonary diseases. [3] We report a patient with Ondine's curse following left lateral medullary infarction, Wallenberg's syndrome.


 » Case Report Top


A 79-year-old white woman presented to the hospital with sudden onset of dizziness, loss of balance, and dysarthria of one hour duration. She was a known case of hypertension. On examination, she was awake and was slurred and dysphonic. She had left side palatal weakness, Horner's syndrome and limb incoordination and also diminished sensations in the right half of the body. Brain magnetic resonance imaging (MRI) revealed hyperintense signal in the left lateral medulla [Figure 1]. Electrocardiogram, echocardiogram, and chest radiograph were normal. Other laboratory tests during the first week of admission were within normal limits. Cervical Doppler studies showed diffuse atheromatous changes and flow velocity in the left vertebral artery was at the lower limit of normal. MR-agniography was normal.

She was prescribed aspirin. During her stay in the intensive care unit (ICU), she had many episodes of sudden apnea, especially during the nights. She developed aspiration pneumonia. The patient had emergency intubation and mechanical ventilation. ABG, when the patient was on pressure support ventilation, showed persistent hypercapnia during sleep (PCO 2 between 80 and 90 and PO 2 under 65) and the gases were normal when she was awake. She could not be weaned off from ventilation because of frequent apnic episodes. She received acetazolamide, levothyroxin, and clomipramine with no benefit. She had prolonged episodes of apnea during the ICU stay, more so when support pressure ventilation was tried. Diaphragmatic pacemaker was considered but could not be done as there is no trained professional to do the procedure at our institute. She died two months postadmission following nosocomial pneumonia, sepsis, and chock.


 » Discussion Top


The patient fulfilled most of the diagnostic criteria for Ondine's curse. Ondine syndrome is commonly described as a congenital central hypoventilation. However, cases of acquired Ondine's curse have been reported.[2],[3],[4] Lesions selectively involving the ventrolateral descending medullocervical pathways that subserve automatic breathing can result in Ondine's curse. [1] There are number of causes that can cause the syndrome: Brainstem infarction, hemorrhage, encephalitis, Leigh syndrome, traumatic Duret hemorrhage, medullary tumors, mitochondrial disease, degenerative diseases (e.g. multisystem atrophy), demyelinating diseases (e.g. multiple sclerosis), and nonspecific anoxic-ischemic insult. [4]

Pharmacological treatment seems not to be of benefit in this condition. Medications tried included acetazolamide, levothyroxin, and clomipramine. None of these medications was of benefit in our patient. Spontaneous recovery has been documented. But most patients need mechanical ventilation for variable periods. Diaphragmatic pacing may be considered in patients with prolonged periods of mechanical ventilation and frequent episodes of apnea. [5] The prognosis of the disease is variable, depending on the location of the lesion and the nature of the pathology. Recovery, although frequent, is usually unpredictable. [6] Death usually occurs during sleep, probably due to total apnea.

 
 » References Top

1.Victor M, Ropper. Chapter in a book: Adam's and Victor's Principles of Neurology. 8 th ed. New York: MGraw-Hill; 2005. p. 474.  Back to cited text no. 1    
2.Schestatsky P, Fernandes LN. Acquired Ondine's curse: Case report. Arq Neuropsiquiatr 2004;62:523-7.  Back to cited text no. 2    
3.Severinghaus JW, Mitchell RA. Ondine's curse-failure of respiratory center automaticity while awake. Clin Res 1962;10:122.  Back to cited text no. 3    
4.Nakajima M, Katsura K, Hashimoto Y, Terasaki T, Uchino M. A case of Online's curse associated a medullary tumor. Rinsho Shinkeigaku 2000;40:811-5.  Back to cited text no. 4    
5.Chervin RD, Guilleminault C. Diaphragm pacing: Review and reassessment. Sleep 1994;17:176-87.  Back to cited text no. 5    
6.Kuhn M, Lutolf M, Reinhart WH. Ondine's curse. Respiration 1999;66:265.  Back to cited text no. 6    


    Figures

  [Figure 1]

This article has been cited by
1 Microstructural cerebral lesions are associated with the severity of central sleep apnea with Cheyne-Stokes-respiration in heart failure and are modified by PAP-therapy
Anna Heidbreder,Jens Spießhöfer,Jörg Stypmann,Matthias Boentert,Peter Young,Thomas Duning
Respiratory Physiology & Neurobiology. 2018; 247: 181
[Pubmed] | [DOI]
2 Ondine’s curse: With Jean Giraudoux’s finishing touches
Régis Olry,Duane E. Haines
Journal of the History of the Neurosciences. 2017; : 1
[Pubmed] | [DOI]
3 Management of a patient with Opalskiæs syndrome in intensive care unit
Ozer Aynaci,Funda Gok,Alper Yosunkaya
Clinical Case Reports. 2017; 5(9): 1518
[Pubmed] | [DOI]
4 Successful delivery of adjuvant external beam radiotherapy for ependymoma in a patient with Ondine?s curse
Mehee Choi,Miranda Thoma,George Tolekidis,Richard W. Byrne,Aidnag Z. Diaz
Medical Dosimetry. 2015; 40(4): 366
[Pubmed] | [DOI]
5 A Case of acquired Ondineæs curse secondary to television injury
Kamakshya P Patra
Journal of Paediatrics and Child Health. 2014; 50(9): 745
[Pubmed] | [DOI]
6 Central respiratory failure occurred in the subacute phase of unilateral Wallenberg’s syndrome: a case report
Eriko Sugawara,Asami Saito,Mitsuo Okamoto,Fumiaki Tanaka,Tatsuya Takahashi
Rinsho Shinkeigaku. 2014; 54(4): 303
[Pubmed] | [DOI]
7 Brainstem Involvement as a Cause of Central Sleep Apnea: Pattern of Microstructural Cerebral Damage in Patients with Cerebral Microangiopathy
Thomas Duning,Michael Deppe,Eva Brand,Jörg Stypmann,Charlotte Becht,Anna Heidbreder,Peter Young,Christoph Kleinschnitz
PLoS ONE. 2013; 8(4): e60304
[Pubmed] | [DOI]
8 Brainstem Involvement as a Cause of Central Sleep Apnea: Pattern of Microstructural Cerebral Damage in Patients with Cerebral Microangiopathy
Duning, T. and Deppe, M. and Brand, E. and Stypmann, J. and Becht, C. and Heidbreder, A. and Young, P.
PLoS ONE. 2013; 8(4)
[Pubmed]
9 Paradoxical herniation in wartime penetrating brain injury with concomitant skull-base trauma
Choi, J.J. and Cirivello, M.J. and Neal, C.J. and Armonda, R.A.
Journal of Craniofacial Surgery. 2011; 22(6): 2163-2167
[Pubmed]
10 Vertebrobasilar insufficiency and obstructive sleep apnea | [Vertebrobazilarnainsuficijencija i opstrukcijska apneja tijekom spavanja]
Grgić, V.
Lijecnicki Vjesnik. 2011; 133(1-2): 50-55
[Pubmed]
11 Fiberoptic endotracheal intubation for the craniovertebral junction operations: A retrospective surveillance | [Krani̇overtebral bölge ameli̇yatlarinda fi̇beropti̇k bronkoskop i̇le endotrakael entübasyon: Retrospekti̇f i̇nceleme]
Özlü, O., Şimşek, S., Ütebey, G., Aksoy, M., Akyol, Ç., Bavbek, M.
Anestezi Dergisi. 2011; 19(3): 177-181
[Pubmed]
12 Central sleep apnea (Ondineæs curse syndrome) in medullary infarction [Centralna Apneja u Spavanju (Sindrom Ondinine Kletve) Kod Infarkta Produžene Moždine]
Planjar-Prvan, M. and Krmpotić, P. and Jergović, I. and Bielen, I.
Acta Medica Croatica. 2010; 64(4): 297-301
[Pubmed]
13 Central alveolar hypoventilation and failure to wean from the ventilator
Ramar, K.
Journal of Clinical Sleep Medicine. 2009; 5(6): 583-585
[Pubmed]



 

Top
Print this article  Email this article
Previous article Next article
Online since 20th March '04
Published by Wolters Kluwer - Medknow