Does the Supplementary Motor Area Keep Patients with Ondine's Curse Syndrome Breathing While Awake?
Identifieur interne : 001752 ( Ncbi/Merge ); précédent : 001751; suivant : 001753Does the Supplementary Motor Area Keep Patients with Ondine's Curse Syndrome Breathing While Awake?
Auteurs : Lysandre Tremoureux [France] ; Mathieu Raux [France] ; Anna L. Hudson [France, Australie] ; Anja Ranohavimparany [France] ; Christian Straus [France] ; Thomas Similowski [France]Source :
- PLoS ONE [ 1932-6203 ] ; 2014.
Descripteurs français
- KwdFr :
- MESH :
- physiologie : Cortex moteur.
- physiopathologie : Apnée centrale du sommeil, Hypoventilation.
- Adulte, Dioxyde de carbone, Femelle, Humains, Hypoventilation, Jeune adulte, Mâle, Respiration, Vigilance, Électroencéphalographie, Études cas-témoins.
English descriptors
- KwdEn :
- MESH :
- chemical : Carbon Dioxide.
- congenital : Hypoventilation.
- physiology : Motor Cortex.
- physiopathology : Hypoventilation, Sleep Apnea, Central.
- Adult, Case-Control Studies, Electroencephalography, Female, Humans, Male, Respiration, Wakefulness, Young Adult.
Abstract
Congenital central hypoventilation syndrome (CCHS) is a rare neuro-respiratory disorder associated with mutations of the PHOX2B gene. Patients with this disease experience severe hypoventilation during sleep and are consequently ventilator-dependent. However, they breathe almost normally while awake, indicating the existence of cortical mechanisms compensating for the deficient brainstem generation of automatic breathing. Current evidence indicates that the supplementary motor area plays an important role in modulating ventilation in awake normal humans. We hypothesized that the wake-related maintenance of spontaneous breathing in patients with CCHS could involve supplementary motor area.
We studied 7 CCHS patients (5 women; age: 20–30; BMI: 22.1±4 kg.m−2) during resting breathing and during exposure to carbon dioxide and inspiratory mechanical constraints. They were compared with 8 healthy individuals. Segments of electroencephalographic tracings were selected according to ventilatory flow signal, from 2.5 seconds to 1.5 seconds after the onset of inspiration. After artefact rejection, 80 or more such segments were ensemble averaged. A slow upward shift of the EEG signal starting between 2 and 0.5 s before inspiration (pre-inspiratory potential) was considered suggestive of supplementary motor area activation.
In the control group, pre-inspiratory potentials were generally absent during resting breathing and carbon dioxide stimulation, and consistently identified in the presence of inspiratory constraints (expected). In CCHS patients, pre-inspiratory potentials were systematically identified in all study conditions, including resting breathing. They were therefore significantly more frequent than in controls.
This study provides a neurophysiological substrate to the wakefulness drive to breathe that is characteristic of CCHS and suggests that the supplementary motor area contributes to this phenomenon. Whether or not this “cortical breathing” can be taken advantage of therapeutically, or has clinical consequences (like competition with attentional resources) remains to be determined.
Url:
DOI: 10.1371/journal.pone.0084534
PubMed: 24475031
PubMed Central: 3901646
Links toward previous steps (curation, corpus...)
- to stream Pmc, to step Corpus: 002B43
- to stream Pmc, to step Curation: 002993
- to stream Pmc, to step Checkpoint: 001633
- to stream PubMed, to step Corpus: 003989
- to stream PubMed, to step Curation: 003873
- to stream PubMed, to step Checkpoint: 003873
Links to Exploration step
PMC:3901646Le document en format XML
<record><TEI><teiHeader><fileDesc><titleStmt><title xml:lang="en">Does the Supplementary Motor Area Keep Patients with Ondine's Curse Syndrome Breathing While Awake?</title>
<author><name sortKey="Tremoureux, Lysandre" sort="Tremoureux, Lysandre" uniqKey="Tremoureux L" first="Lysandre" last="Tremoureux">Lysandre Tremoureux</name>
<affiliation wicri:level="3"><nlm:aff id="aff1"><addr-line>UMR_S1158, Inserm-Université Paris 6, Paris, France</addr-line>
</nlm:aff>
<country xml:lang="fr">France</country>
<wicri:regionArea>UMR_S1158, Inserm-Université Paris 6, Paris</wicri:regionArea>
<placeName><region type="region">Île-de-France</region>
<region type="old region">Île-de-France</region>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
</author>
<author><name sortKey="Raux, Mathieu" sort="Raux, Mathieu" uniqKey="Raux M" first="Mathieu" last="Raux">Mathieu Raux</name>
<affiliation wicri:level="3"><nlm:aff id="aff1"><addr-line>UMR_S1158, Inserm-Université Paris 6, Paris, France</addr-line>
</nlm:aff>
<country xml:lang="fr">France</country>
<wicri:regionArea>UMR_S1158, Inserm-Université Paris 6, Paris</wicri:regionArea>
<placeName><region type="region">Île-de-France</region>
<region type="old region">Île-de-France</region>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
<affiliation wicri:level="3"><nlm:aff id="aff2"><addr-line>Département d'Anesthésie Réanimation—Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Assistance Publique—Hôpitaux de Paris, Paris, France</addr-line>
</nlm:aff>
<country xml:lang="fr">France</country>
<wicri:regionArea>Département d'Anesthésie Réanimation—Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Assistance Publique—Hôpitaux de Paris, Paris</wicri:regionArea>
<placeName><region type="region">Île-de-France</region>
<region type="old region">Île-de-France</region>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
</author>
<author><name sortKey="Hudson, Anna L" sort="Hudson, Anna L" uniqKey="Hudson A" first="Anna L." last="Hudson">Anna L. Hudson</name>
<affiliation wicri:level="3"><nlm:aff id="aff1"><addr-line>UMR_S1158, Inserm-Université Paris 6, Paris, France</addr-line>
</nlm:aff>
<country xml:lang="fr">France</country>
<wicri:regionArea>UMR_S1158, Inserm-Université Paris 6, Paris</wicri:regionArea>
<placeName><region type="region">Île-de-France</region>
<region type="old region">Île-de-France</region>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
<affiliation wicri:level="3"><nlm:aff id="aff3"><addr-line>Neuroscience Research Australia and University of New South Wales, Sydney, Australia</addr-line>
</nlm:aff>
<country xml:lang="fr">Australie</country>
<wicri:regionArea>Neuroscience Research Australia and University of New South Wales, Sydney</wicri:regionArea>
<placeName><settlement type="city">Sydney</settlement>
<region type="état">Nouvelle-Galles du Sud</region>
</placeName>
</affiliation>
</author>
<author><name sortKey="Ranohavimparany, Anja" sort="Ranohavimparany, Anja" uniqKey="Ranohavimparany A" first="Anja" last="Ranohavimparany">Anja Ranohavimparany</name>
<affiliation wicri:level="3"><nlm:aff id="aff1"><addr-line>UMR_S1158, Inserm-Université Paris 6, Paris, France</addr-line>
</nlm:aff>
<country xml:lang="fr">France</country>
<wicri:regionArea>UMR_S1158, Inserm-Université Paris 6, Paris</wicri:regionArea>
<placeName><region type="region">Île-de-France</region>
<region type="old region">Île-de-France</region>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
</author>
<author><name sortKey="Straus, Christian" sort="Straus, Christian" uniqKey="Straus C" first="Christian" last="Straus">Christian Straus</name>
<affiliation wicri:level="3"><nlm:aff id="aff1"><addr-line>UMR_S1158, Inserm-Université Paris 6, Paris, France</addr-line>
</nlm:aff>
<country xml:lang="fr">France</country>
<wicri:regionArea>UMR_S1158, Inserm-Université Paris 6, Paris</wicri:regionArea>
<placeName><region type="region">Île-de-France</region>
<region type="old region">Île-de-France</region>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
<affiliation wicri:level="3"><nlm:aff id="aff4"><addr-line>Service Central des Explorations Fonctionelles Respiratoires de l'Exercice et de la Dyspnée—Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Assistance Publique—Hôpitaux de Paris, Paris, France</addr-line>
</nlm:aff>
<country xml:lang="fr">France</country>
<wicri:regionArea>Service Central des Explorations Fonctionelles Respiratoires de l'Exercice et de la Dyspnée—Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Assistance Publique—Hôpitaux de Paris, Paris</wicri:regionArea>
<placeName><region type="region">Île-de-France</region>
<region type="old region">Île-de-France</region>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
<affiliation wicri:level="3"><nlm:aff id="aff5"><addr-line>Centre National de Référence Maladies Rares pour le syndrome d'Ondine (adultes)—Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Assistance Publique—Hôpitaux de Paris, Paris, France</addr-line>
</nlm:aff>
<country xml:lang="fr">France</country>
<wicri:regionArea>Centre National de Référence Maladies Rares pour le syndrome d'Ondine (adultes)—Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Assistance Publique—Hôpitaux de Paris, Paris</wicri:regionArea>
<placeName><region type="region">Île-de-France</region>
<region type="old region">Île-de-France</region>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
</author>
<author><name sortKey="Similowski, Thomas" sort="Similowski, Thomas" uniqKey="Similowski T" first="Thomas" last="Similowski">Thomas Similowski</name>
<affiliation wicri:level="3"><nlm:aff id="aff1"><addr-line>UMR_S1158, Inserm-Université Paris 6, Paris, France</addr-line>
</nlm:aff>
<country xml:lang="fr">France</country>
<wicri:regionArea>UMR_S1158, Inserm-Université Paris 6, Paris</wicri:regionArea>
<placeName><region type="region">Île-de-France</region>
<region type="old region">Île-de-France</region>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
<affiliation wicri:level="3"><nlm:aff id="aff5"><addr-line>Centre National de Référence Maladies Rares pour le syndrome d'Ondine (adultes)—Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Assistance Publique—Hôpitaux de Paris, Paris, France</addr-line>
</nlm:aff>
<country xml:lang="fr">France</country>
<wicri:regionArea>Centre National de Référence Maladies Rares pour le syndrome d'Ondine (adultes)—Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Assistance Publique—Hôpitaux de Paris, Paris</wicri:regionArea>
<placeName><region type="region">Île-de-France</region>
<region type="old region">Île-de-France</region>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
<affiliation wicri:level="3"><nlm:aff id="aff6"><addr-line>Service de Pneumologie et Réanimation Médicale—Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Assistance Publique—Hôpitaux de Paris, Paris, France</addr-line>
</nlm:aff>
<country xml:lang="fr">France</country>
<wicri:regionArea>Service de Pneumologie et Réanimation Médicale—Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Assistance Publique—Hôpitaux de Paris, Paris</wicri:regionArea>
<placeName><region type="region">Île-de-France</region>
<region type="old region">Île-de-France</region>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
</author>
</titleStmt>
<publicationStmt><idno type="wicri:source">PMC</idno>
<idno type="pmid">24475031</idno>
<idno type="pmc">3901646</idno>
<idno type="url">http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3901646</idno>
<idno type="RBID">PMC:3901646</idno>
<idno type="doi">10.1371/journal.pone.0084534</idno>
<date when="2014">2014</date>
<idno type="wicri:Area/Pmc/Corpus">002B43</idno>
<idno type="wicri:explorRef" wicri:stream="Pmc" wicri:step="Corpus" wicri:corpus="PMC">002B43</idno>
<idno type="wicri:Area/Pmc/Curation">002993</idno>
<idno type="wicri:explorRef" wicri:stream="Pmc" wicri:step="Curation">002993</idno>
<idno type="wicri:Area/Pmc/Checkpoint">001633</idno>
<idno type="wicri:explorRef" wicri:stream="Pmc" wicri:step="Checkpoint">001633</idno>
<idno type="wicri:source">PubMed</idno>
<idno type="wicri:Area/PubMed/Corpus">003989</idno>
<idno type="wicri:explorRef" wicri:stream="PubMed" wicri:step="Corpus" wicri:corpus="PubMed">003989</idno>
<idno type="wicri:Area/PubMed/Curation">003873</idno>
<idno type="wicri:explorRef" wicri:stream="PubMed" wicri:step="Curation">003873</idno>
<idno type="wicri:Area/PubMed/Checkpoint">003873</idno>
<idno type="wicri:explorRef" wicri:stream="Checkpoint" wicri:step="PubMed">003873</idno>
<idno type="wicri:Area/Ncbi/Merge">001752</idno>
</publicationStmt>
<sourceDesc><biblStruct><analytic><title xml:lang="en" level="a" type="main">Does the Supplementary Motor Area Keep Patients with Ondine's Curse Syndrome Breathing While Awake?</title>
<author><name sortKey="Tremoureux, Lysandre" sort="Tremoureux, Lysandre" uniqKey="Tremoureux L" first="Lysandre" last="Tremoureux">Lysandre Tremoureux</name>
<affiliation wicri:level="3"><nlm:aff id="aff1"><addr-line>UMR_S1158, Inserm-Université Paris 6, Paris, France</addr-line>
</nlm:aff>
<country xml:lang="fr">France</country>
<wicri:regionArea>UMR_S1158, Inserm-Université Paris 6, Paris</wicri:regionArea>
<placeName><region type="region">Île-de-France</region>
<region type="old region">Île-de-France</region>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
</author>
<author><name sortKey="Raux, Mathieu" sort="Raux, Mathieu" uniqKey="Raux M" first="Mathieu" last="Raux">Mathieu Raux</name>
<affiliation wicri:level="3"><nlm:aff id="aff1"><addr-line>UMR_S1158, Inserm-Université Paris 6, Paris, France</addr-line>
</nlm:aff>
<country xml:lang="fr">France</country>
<wicri:regionArea>UMR_S1158, Inserm-Université Paris 6, Paris</wicri:regionArea>
<placeName><region type="region">Île-de-France</region>
<region type="old region">Île-de-France</region>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
<affiliation wicri:level="3"><nlm:aff id="aff2"><addr-line>Département d'Anesthésie Réanimation—Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Assistance Publique—Hôpitaux de Paris, Paris, France</addr-line>
</nlm:aff>
<country xml:lang="fr">France</country>
<wicri:regionArea>Département d'Anesthésie Réanimation—Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Assistance Publique—Hôpitaux de Paris, Paris</wicri:regionArea>
<placeName><region type="region">Île-de-France</region>
<region type="old region">Île-de-France</region>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
</author>
<author><name sortKey="Hudson, Anna L" sort="Hudson, Anna L" uniqKey="Hudson A" first="Anna L." last="Hudson">Anna L. Hudson</name>
<affiliation wicri:level="3"><nlm:aff id="aff1"><addr-line>UMR_S1158, Inserm-Université Paris 6, Paris, France</addr-line>
</nlm:aff>
<country xml:lang="fr">France</country>
<wicri:regionArea>UMR_S1158, Inserm-Université Paris 6, Paris</wicri:regionArea>
<placeName><region type="region">Île-de-France</region>
<region type="old region">Île-de-France</region>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
<affiliation wicri:level="3"><nlm:aff id="aff3"><addr-line>Neuroscience Research Australia and University of New South Wales, Sydney, Australia</addr-line>
</nlm:aff>
<country xml:lang="fr">Australie</country>
<wicri:regionArea>Neuroscience Research Australia and University of New South Wales, Sydney</wicri:regionArea>
<placeName><settlement type="city">Sydney</settlement>
<region type="état">Nouvelle-Galles du Sud</region>
</placeName>
</affiliation>
</author>
<author><name sortKey="Ranohavimparany, Anja" sort="Ranohavimparany, Anja" uniqKey="Ranohavimparany A" first="Anja" last="Ranohavimparany">Anja Ranohavimparany</name>
<affiliation wicri:level="3"><nlm:aff id="aff1"><addr-line>UMR_S1158, Inserm-Université Paris 6, Paris, France</addr-line>
</nlm:aff>
<country xml:lang="fr">France</country>
<wicri:regionArea>UMR_S1158, Inserm-Université Paris 6, Paris</wicri:regionArea>
<placeName><region type="region">Île-de-France</region>
<region type="old region">Île-de-France</region>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
</author>
<author><name sortKey="Straus, Christian" sort="Straus, Christian" uniqKey="Straus C" first="Christian" last="Straus">Christian Straus</name>
<affiliation wicri:level="3"><nlm:aff id="aff1"><addr-line>UMR_S1158, Inserm-Université Paris 6, Paris, France</addr-line>
</nlm:aff>
<country xml:lang="fr">France</country>
<wicri:regionArea>UMR_S1158, Inserm-Université Paris 6, Paris</wicri:regionArea>
<placeName><region type="region">Île-de-France</region>
<region type="old region">Île-de-France</region>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
<affiliation wicri:level="3"><nlm:aff id="aff4"><addr-line>Service Central des Explorations Fonctionelles Respiratoires de l'Exercice et de la Dyspnée—Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Assistance Publique—Hôpitaux de Paris, Paris, France</addr-line>
</nlm:aff>
<country xml:lang="fr">France</country>
<wicri:regionArea>Service Central des Explorations Fonctionelles Respiratoires de l'Exercice et de la Dyspnée—Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Assistance Publique—Hôpitaux de Paris, Paris</wicri:regionArea>
<placeName><region type="region">Île-de-France</region>
<region type="old region">Île-de-France</region>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
<affiliation wicri:level="3"><nlm:aff id="aff5"><addr-line>Centre National de Référence Maladies Rares pour le syndrome d'Ondine (adultes)—Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Assistance Publique—Hôpitaux de Paris, Paris, France</addr-line>
</nlm:aff>
<country xml:lang="fr">France</country>
<wicri:regionArea>Centre National de Référence Maladies Rares pour le syndrome d'Ondine (adultes)—Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Assistance Publique—Hôpitaux de Paris, Paris</wicri:regionArea>
<placeName><region type="region">Île-de-France</region>
<region type="old region">Île-de-France</region>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
</author>
<author><name sortKey="Similowski, Thomas" sort="Similowski, Thomas" uniqKey="Similowski T" first="Thomas" last="Similowski">Thomas Similowski</name>
<affiliation wicri:level="3"><nlm:aff id="aff1"><addr-line>UMR_S1158, Inserm-Université Paris 6, Paris, France</addr-line>
</nlm:aff>
<country xml:lang="fr">France</country>
<wicri:regionArea>UMR_S1158, Inserm-Université Paris 6, Paris</wicri:regionArea>
<placeName><region type="region">Île-de-France</region>
<region type="old region">Île-de-France</region>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
<affiliation wicri:level="3"><nlm:aff id="aff5"><addr-line>Centre National de Référence Maladies Rares pour le syndrome d'Ondine (adultes)—Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Assistance Publique—Hôpitaux de Paris, Paris, France</addr-line>
</nlm:aff>
<country xml:lang="fr">France</country>
<wicri:regionArea>Centre National de Référence Maladies Rares pour le syndrome d'Ondine (adultes)—Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Assistance Publique—Hôpitaux de Paris, Paris</wicri:regionArea>
<placeName><region type="region">Île-de-France</region>
<region type="old region">Île-de-France</region>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
<affiliation wicri:level="3"><nlm:aff id="aff6"><addr-line>Service de Pneumologie et Réanimation Médicale—Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Assistance Publique—Hôpitaux de Paris, Paris, France</addr-line>
</nlm:aff>
<country xml:lang="fr">France</country>
<wicri:regionArea>Service de Pneumologie et Réanimation Médicale—Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Assistance Publique—Hôpitaux de Paris, Paris</wicri:regionArea>
<placeName><region type="region">Île-de-France</region>
<region type="old region">Île-de-France</region>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
</author>
</analytic>
<series><title level="j">PLoS ONE</title>
<idno type="eISSN">1932-6203</idno>
<imprint><date when="2014">2014</date>
</imprint>
</series>
</biblStruct>
</sourceDesc>
</fileDesc>
<profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Adult</term>
<term>Carbon Dioxide</term>
<term>Case-Control Studies</term>
<term>Electroencephalography</term>
<term>Female</term>
<term>Humans</term>
<term>Hypoventilation (congenital)</term>
<term>Hypoventilation (physiopathology)</term>
<term>Male</term>
<term>Motor Cortex (physiology)</term>
<term>Respiration</term>
<term>Sleep Apnea, Central (physiopathology)</term>
<term>Wakefulness</term>
<term>Young Adult</term>
</keywords>
<keywords scheme="KwdFr" xml:lang="fr"><term>Adulte</term>
<term>Apnée centrale du sommeil (physiopathologie)</term>
<term>Cortex moteur (physiologie)</term>
<term>Dioxyde de carbone</term>
<term>Femelle</term>
<term>Humains</term>
<term>Hypoventilation ()</term>
<term>Hypoventilation (physiopathologie)</term>
<term>Jeune adulte</term>
<term>Mâle</term>
<term>Respiration</term>
<term>Vigilance</term>
<term>Électroencéphalographie</term>
<term>Études cas-témoins</term>
</keywords>
<keywords scheme="MESH" type="chemical" xml:lang="en"><term>Carbon Dioxide</term>
</keywords>
<keywords scheme="MESH" qualifier="congenital" xml:lang="en"><term>Hypoventilation</term>
</keywords>
<keywords scheme="MESH" qualifier="physiologie" xml:lang="fr"><term>Cortex moteur</term>
</keywords>
<keywords scheme="MESH" qualifier="physiology" xml:lang="en"><term>Motor Cortex</term>
</keywords>
<keywords scheme="MESH" qualifier="physiopathologie" xml:lang="fr"><term>Apnée centrale du sommeil</term>
<term>Hypoventilation</term>
</keywords>
<keywords scheme="MESH" qualifier="physiopathology" xml:lang="en"><term>Hypoventilation</term>
<term>Sleep Apnea, Central</term>
</keywords>
<keywords scheme="MESH" xml:lang="en"><term>Adult</term>
<term>Case-Control Studies</term>
<term>Electroencephalography</term>
<term>Female</term>
<term>Humans</term>
<term>Male</term>
<term>Respiration</term>
<term>Wakefulness</term>
<term>Young Adult</term>
</keywords>
<keywords scheme="MESH" xml:lang="fr"><term>Adulte</term>
<term>Dioxyde de carbone</term>
<term>Femelle</term>
<term>Humains</term>
<term>Hypoventilation</term>
<term>Jeune adulte</term>
<term>Mâle</term>
<term>Respiration</term>
<term>Vigilance</term>
<term>Électroencéphalographie</term>
<term>Études cas-témoins</term>
</keywords>
</textClass>
</profileDesc>
</teiHeader>
<front><div type="abstract" xml:lang="en"><sec><title>Background</title>
<p>Congenital central hypoventilation syndrome (CCHS) is a rare neuro-respiratory disorder associated with mutations of the PHOX2B gene. Patients with this disease experience severe hypoventilation during sleep and are consequently ventilator-dependent. However, they breathe almost normally while awake, indicating the existence of cortical mechanisms compensating for the deficient brainstem generation of automatic breathing. Current evidence indicates that the supplementary motor area plays an important role in modulating ventilation in awake normal humans. We hypothesized that the wake-related maintenance of spontaneous breathing in patients with CCHS could involve supplementary motor area.</p>
</sec>
<sec><title>Methods</title>
<p>We studied 7 CCHS patients (5 women; age: 20–30; BMI: 22.1±4 kg.m<sup>−2</sup>
) during resting breathing and during exposure to carbon dioxide and inspiratory mechanical constraints. They were compared with 8 healthy individuals. Segments of electroencephalographic tracings were selected according to ventilatory flow signal, from 2.5 seconds to 1.5 seconds after the onset of inspiration. After artefact rejection, 80 or more such segments were ensemble averaged. A slow upward shift of the EEG signal starting between 2 and 0.5 s before inspiration (pre-inspiratory potential) was considered suggestive of supplementary motor area activation.</p>
</sec>
<sec><title>Results</title>
<p>In the control group, pre-inspiratory potentials were generally absent during resting breathing and carbon dioxide stimulation, and consistently identified in the presence of inspiratory constraints (expected). In CCHS patients, pre-inspiratory potentials were systematically identified in all study conditions, including resting breathing. They were therefore significantly more frequent than in controls.</p>
</sec>
<sec><title>Conclusions</title>
<p>This study provides a neurophysiological substrate to the wakefulness drive to breathe that is characteristic of CCHS and suggests that the supplementary motor area contributes to this phenomenon. Whether or not this “cortical breathing” can be taken advantage of therapeutically, or has clinical consequences (like competition with attentional resources) remains to be determined.</p>
</sec>
</div>
</front>
<back><div1 type="bibliography"><listBibl><biblStruct><analytic><author><name sortKey="Weese Mayer, De" uniqKey="Weese Mayer D">DE Weese-Mayer</name>
</author>
<author><name sortKey="Berry Kravis, Em" uniqKey="Berry Kravis E">EM Berry-Kravis</name>
</author>
<author><name sortKey="Ceccherini, I" uniqKey="Ceccherini I">I Ceccherini</name>
</author>
<author><name sortKey="Keens, Tg" uniqKey="Keens T">TG Keens</name>
</author>
<author><name sortKey="Loghmanee, Da" uniqKey="Loghmanee D">DA Loghmanee</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Dubreuil, V" uniqKey="Dubreuil V">V Dubreuil</name>
</author>
<author><name sortKey="Ramanantsoa, N" uniqKey="Ramanantsoa N">N Ramanantsoa</name>
</author>
<author><name sortKey="Trochet, D" uniqKey="Trochet D">D Trochet</name>
</author>
<author><name sortKey="Vaubourg, V" uniqKey="Vaubourg V">V Vaubourg</name>
</author>
<author><name sortKey="Amiel, J" uniqKey="Amiel J">J Amiel</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Shea, Sa" uniqKey="Shea S">SA Shea</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Shea, Sa" uniqKey="Shea S">SA Shea</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Hudson, Al" uniqKey="Hudson A">AL Hudson</name>
</author>
<author><name sortKey="Gandevia, Sc" uniqKey="Gandevia S">SC Gandevia</name>
</author>
<author><name sortKey="Butler, Je" uniqKey="Butler J">JE Butler</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Haouzi, P" uniqKey="Haouzi P">P Haouzi</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Corfield, Dr" uniqKey="Corfield D">DR Corfield</name>
</author>
<author><name sortKey="Morrell, Mj" uniqKey="Morrell M">MJ Morrell</name>
</author>
<author><name sortKey="Guz, A" uniqKey="Guz A">A Guz</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Datta, Ak" uniqKey="Datta A">AK Datta</name>
</author>
<author><name sortKey="Shea, Sa" uniqKey="Shea S">SA Shea</name>
</author>
<author><name sortKey="Horner, Rl" uniqKey="Horner R">RL Horner</name>
</author>
<author><name sortKey="Guz, A" uniqKey="Guz A">A Guz</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Wiegand, L" uniqKey="Wiegand L">L Wiegand</name>
</author>
<author><name sortKey="Zwillich, Cw" uniqKey="Zwillich C">CW Zwillich</name>
</author>
<author><name sortKey="White, Dp" uniqKey="White D">DP White</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Axen, K" uniqKey="Axen K">K Axen</name>
</author>
<author><name sortKey="Haas, Ss" uniqKey="Haas S">SS Haas</name>
</author>
<author><name sortKey="Haas, F" uniqKey="Haas F">F Haas</name>
</author>
<author><name sortKey="Gaudino, D" uniqKey="Gaudino D">D Gaudino</name>
</author>
<author><name sortKey="Haas, A" uniqKey="Haas A">A Haas</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Yanos, J" uniqKey="Yanos J">J Yanos</name>
</author>
<author><name sortKey="Banner, A" uniqKey="Banner A">A Banner</name>
</author>
<author><name sortKey="Stanko, R" uniqKey="Stanko R">R Stanko</name>
</author>
<author><name sortKey="Gentry, S" uniqKey="Gentry S">S Gentry</name>
</author>
<author><name sortKey="Greenawalt, K" uniqKey="Greenawalt K">K Greenawalt</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Raux, M" uniqKey="Raux M">M Raux</name>
</author>
<author><name sortKey="Straus, C" uniqKey="Straus C">C Straus</name>
</author>
<author><name sortKey="Redolfi, S" uniqKey="Redolfi S">S Redolfi</name>
</author>
<author><name sortKey="Morelot Panzini, C" uniqKey="Morelot Panzini C">C Morelot-Panzini</name>
</author>
<author><name sortKey="Couturier, A" uniqKey="Couturier A">A Couturier</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Tremoureux, L" uniqKey="Tremoureux L">L Tremoureux</name>
</author>
<author><name sortKey="Raux, M" uniqKey="Raux M">M Raux</name>
</author>
<author><name sortKey="Jutand, L" uniqKey="Jutand L">L Jutand</name>
</author>
<author><name sortKey="Similowski, T" uniqKey="Similowski T">T Similowski</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Raux, M" uniqKey="Raux M">M Raux</name>
</author>
<author><name sortKey="Xie, H" uniqKey="Xie H">H Xie</name>
</author>
<author><name sortKey="Similowski, T" uniqKey="Similowski T">T Similowski</name>
</author>
<author><name sortKey="Koski, L" uniqKey="Koski L">L Koski</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Sharshar, T" uniqKey="Sharshar T">T Sharshar</name>
</author>
<author><name sortKey="Hopkinson, Ns" uniqKey="Hopkinson N">NS Hopkinson</name>
</author>
<author><name sortKey="Jonville, S" uniqKey="Jonville S">S Jonville</name>
</author>
<author><name sortKey="Prigent, H" uniqKey="Prigent H">H Prigent</name>
</author>
<author><name sortKey="Carlier, R" uniqKey="Carlier R">R Carlier</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Laviolette, L" uniqKey="Laviolette L">L Laviolette</name>
</author>
<author><name sortKey="Nierat, Mc" uniqKey="Nierat M">MC Nierat</name>
</author>
<author><name sortKey="Hudson, Al" uniqKey="Hudson A">AL Hudson</name>
</author>
<author><name sortKey="Raux, M" uniqKey="Raux M">M Raux</name>
</author>
<author><name sortKey="Allard, E" uniqKey="Allard E">E Allard</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Straus, C" uniqKey="Straus C">C Straus</name>
</author>
<author><name sortKey="Similowski, T" uniqKey="Similowski T">T Similowski</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Eberhard, A" uniqKey="Eberhard A">A Eberhard</name>
</author>
<author><name sortKey="Calabrese, P" uniqKey="Calabrese P">P Calabrese</name>
</author>
<author><name sortKey="Baconnier, P" uniqKey="Baconnier P">P Baconnier</name>
</author>
<author><name sortKey="Benchetrit, G" uniqKey="Benchetrit G">G Benchetrit</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Raux, M" uniqKey="Raux M">M Raux</name>
</author>
<author><name sortKey="Ray, P" uniqKey="Ray P">P Ray</name>
</author>
<author><name sortKey="Prella, M" uniqKey="Prella M">M Prella</name>
</author>
<author><name sortKey="Duguet, A" uniqKey="Duguet A">A Duguet</name>
</author>
<author><name sortKey="Demoule, A" uniqKey="Demoule A">A Demoule</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Jutand, L" uniqKey="Jutand L">L Jutand</name>
</author>
<author><name sortKey="Tremoureux, L" uniqKey="Tremoureux L">L Tremoureux</name>
</author>
<author><name sortKey="Pichon, A" uniqKey="Pichon A">A Pichon</name>
</author>
<author><name sortKey="Delpech, N" uniqKey="Delpech N">N Delpech</name>
</author>
<author><name sortKey="Denjean, A" uniqKey="Denjean A">A Denjean</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Raux, M" uniqKey="Raux M">M Raux</name>
</author>
<author><name sortKey="Tremoureux, L" uniqKey="Tremoureux L">L Tremoureux</name>
</author>
<author><name sortKey="Couturier, A" uniqKey="Couturier A">A Couturier</name>
</author>
<author><name sortKey="Hug, F" uniqKey="Hug F">F Hug</name>
</author>
<author><name sortKey="Similowski, T" uniqKey="Similowski T">T Similowski</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Colebatch, Jg" uniqKey="Colebatch J">JG Colebatch</name>
</author>
<author><name sortKey="Adams, L" uniqKey="Adams L">L Adams</name>
</author>
<author><name sortKey="Murphy, K" uniqKey="Murphy K">K Murphy</name>
</author>
<author><name sortKey="Martin, Aj" uniqKey="Martin A">AJ Martin</name>
</author>
<author><name sortKey="Lammertsma, Aa" uniqKey="Lammertsma A">AA Lammertsma</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Mckay, Lc" uniqKey="Mckay L">LC McKay</name>
</author>
<author><name sortKey="Evans, Kc" uniqKey="Evans K">KC Evans</name>
</author>
<author><name sortKey="Frackowiak, Rs" uniqKey="Frackowiak R">RS Frackowiak</name>
</author>
<author><name sortKey="Corfield, Dr" uniqKey="Corfield D">DR Corfield</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Raux, M" uniqKey="Raux M">M Raux</name>
</author>
<author><name sortKey="Tyvaert, L" uniqKey="Tyvaert L">L Tyvaert</name>
</author>
<author><name sortKey="Ferreira, M" uniqKey="Ferreira M">M Ferreira</name>
</author>
<author><name sortKey="Kindler, F" uniqKey="Kindler F">F Kindler</name>
</author>
<author><name sortKey="Morelot Panzini, C" uniqKey="Morelot Panzini C">C Morélot-Panzini</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Haouzi, P" uniqKey="Haouzi P">P Haouzi</name>
</author>
<author><name sortKey="Bell, Hj" uniqKey="Bell H">HJ Bell</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Aminoff, Mj" uniqKey="Aminoff M">MJ Aminoff</name>
</author>
<author><name sortKey="Sears, Ta" uniqKey="Sears T">TA Sears</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Mehiri, S" uniqKey="Mehiri S">S Mehiri</name>
</author>
<author><name sortKey="Straus, C" uniqKey="Straus C">C Straus</name>
</author>
<author><name sortKey="Arnulf, I" uniqKey="Arnulf I">I Arnulf</name>
</author>
<author><name sortKey="Attali, V" uniqKey="Attali V">V Attali</name>
</author>
<author><name sortKey="Zelter, M" uniqKey="Zelter M">M Zelter</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Murphy, K" uniqKey="Murphy K">K Murphy</name>
</author>
<author><name sortKey="Mier, A" uniqKey="Mier A">A Mier</name>
</author>
<author><name sortKey="Adams, L" uniqKey="Adams L">L Adams</name>
</author>
<author><name sortKey="Guz, A" uniqKey="Guz A">A Guz</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Straus, C" uniqKey="Straus C">C Straus</name>
</author>
<author><name sortKey="Locher, C" uniqKey="Locher C">C Locher</name>
</author>
<author><name sortKey="Zelter, M" uniqKey="Zelter M">M Zelter</name>
</author>
<author><name sortKey="Derenne, Jp" uniqKey="Derenne J">JP Derenne</name>
</author>
<author><name sortKey="Similowski, T" uniqKey="Similowski T">T Similowski</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Davey, Nj" uniqKey="Davey N">NJ Davey</name>
</author>
<author><name sortKey="Murphy, K" uniqKey="Murphy K">K Murphy</name>
</author>
<author><name sortKey="Maskill, Dw" uniqKey="Maskill D">DW Maskill</name>
</author>
<author><name sortKey="Guz, A" uniqKey="Guz A">A Guz</name>
</author>
<author><name sortKey="Ellaway, Ph" uniqKey="Ellaway P">PH Ellaway</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Hudson, Al" uniqKey="Hudson A">AL Hudson</name>
</author>
<author><name sortKey="Butler, Je" uniqKey="Butler J">JE Butler</name>
</author>
<author><name sortKey="Gandevia, Sc" uniqKey="Gandevia S">SC Gandevia</name>
</author>
<author><name sortKey="De Troyer, A" uniqKey="De Troyer A">A De Troyer</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Hudson, Al" uniqKey="Hudson A">AL Hudson</name>
</author>
<author><name sortKey="Butler, Je" uniqKey="Butler J">JE Butler</name>
</author>
<author><name sortKey="Gandevia, Sc" uniqKey="Gandevia S">SC Gandevia</name>
</author>
<author><name sortKey="De Troyer, A" uniqKey="De Troyer A">A De Troyer</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Massimini, M" uniqKey="Massimini M">M Massimini</name>
</author>
<author><name sortKey="Ferrarelli, F" uniqKey="Ferrarelli F">F Ferrarelli</name>
</author>
<author><name sortKey="Huber, R" uniqKey="Huber R">R Huber</name>
</author>
<author><name sortKey="Esser, Sk" uniqKey="Esser S">SK Esser</name>
</author>
<author><name sortKey="Singh, H" uniqKey="Singh H">H Singh</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Wu, T" uniqKey="Wu T">T Wu</name>
</author>
<author><name sortKey="Kansaku, K" uniqKey="Kansaku K">K Kansaku</name>
</author>
<author><name sortKey="Hallett, M" uniqKey="Hallett M">M Hallett</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Passingham, Re" uniqKey="Passingham R">RE Passingham</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Shea, Sa" uniqKey="Shea S">SA Shea</name>
</author>
<author><name sortKey="Andres, Lp" uniqKey="Andres L">LP Andres</name>
</author>
<author><name sortKey="Paydarfar, D" uniqKey="Paydarfar D">D Paydarfar</name>
</author>
<author><name sortKey="Banzett, Rb" uniqKey="Banzett R">RB Banzett</name>
</author>
<author><name sortKey="Shannon, Dc" uniqKey="Shannon D">DC Shannon</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Guilleminault, C" uniqKey="Guilleminault C">C Guilleminault</name>
</author>
<author><name sortKey="Mcquitty, J" uniqKey="Mcquitty J">J McQuitty</name>
</author>
<author><name sortKey="Ariagno, Rl" uniqKey="Ariagno R">RL Ariagno</name>
</author>
<author><name sortKey="Challamel, Mj" uniqKey="Challamel M">MJ Challamel</name>
</author>
<author><name sortKey="Korobkin, R" uniqKey="Korobkin R">R Korobkin</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Denot Ledunois, S" uniqKey="Denot Ledunois S">S Denot-Ledunois</name>
</author>
<author><name sortKey="Vardon, G" uniqKey="Vardon G">G Vardon</name>
</author>
<author><name sortKey="Perruchet, P" uniqKey="Perruchet P">P Perruchet</name>
</author>
<author><name sortKey="Gallego, J" uniqKey="Gallego J">J Gallego</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Blanc Gras, N" uniqKey="Blanc Gras N">N Blanc-Gras</name>
</author>
<author><name sortKey="Esteve, F" uniqKey="Esteve F">F Esteve</name>
</author>
<author><name sortKey="Benchetrit, G" uniqKey="Benchetrit G">G Benchetrit</name>
</author>
<author><name sortKey="Gallego, J" uniqKey="Gallego J">J Gallego</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Gallego, J" uniqKey="Gallego J">J Gallego</name>
</author>
<author><name sortKey="Perruchet, P" uniqKey="Perruchet P">P Perruchet</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Cohen, E" uniqKey="Cohen E">E Cohen</name>
</author>
<author><name sortKey="Murphy, K" uniqKey="Murphy K">K Murphy</name>
</author>
<author><name sortKey="Adams, L" uniqKey="Adams L">L Adams</name>
</author>
<author><name sortKey="Guz, A" uniqKey="Guz A">A Guz</name>
</author>
<author><name sortKey="Benchetrit, G" uniqKey="Benchetrit G">G Benchetrit</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Li, S" uniqKey="Li S">S Li</name>
</author>
<author><name sortKey="Rymer, Wz" uniqKey="Rymer W">WZ Rymer</name>
</author>
</analytic>
</biblStruct>
</listBibl>
</div1>
</back>
</TEI>
<double pmid="24475031"><pmc><TEI><teiHeader><fileDesc><titleStmt><title xml:lang="en">Does the Supplementary Motor Area Keep Patients with Ondine's Curse Syndrome Breathing While Awake?</title>
<author><name sortKey="Tremoureux, Lysandre" sort="Tremoureux, Lysandre" uniqKey="Tremoureux L" first="Lysandre" last="Tremoureux">Lysandre Tremoureux</name>
<affiliation wicri:level="3"><nlm:aff id="aff1"><addr-line>UMR_S1158, Inserm-Université Paris 6, Paris, France</addr-line>
</nlm:aff>
<country xml:lang="fr">France</country>
<wicri:regionArea>UMR_S1158, Inserm-Université Paris 6, Paris</wicri:regionArea>
<placeName><region type="region">Île-de-France</region>
<region type="old region">Île-de-France</region>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
</author>
<author><name sortKey="Raux, Mathieu" sort="Raux, Mathieu" uniqKey="Raux M" first="Mathieu" last="Raux">Mathieu Raux</name>
<affiliation wicri:level="3"><nlm:aff id="aff1"><addr-line>UMR_S1158, Inserm-Université Paris 6, Paris, France</addr-line>
</nlm:aff>
<country xml:lang="fr">France</country>
<wicri:regionArea>UMR_S1158, Inserm-Université Paris 6, Paris</wicri:regionArea>
<placeName><region type="region">Île-de-France</region>
<region type="old region">Île-de-France</region>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
<affiliation wicri:level="3"><nlm:aff id="aff2"><addr-line>Département d'Anesthésie Réanimation—Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Assistance Publique—Hôpitaux de Paris, Paris, France</addr-line>
</nlm:aff>
<country xml:lang="fr">France</country>
<wicri:regionArea>Département d'Anesthésie Réanimation—Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Assistance Publique—Hôpitaux de Paris, Paris</wicri:regionArea>
<placeName><region type="region">Île-de-France</region>
<region type="old region">Île-de-France</region>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
</author>
<author><name sortKey="Hudson, Anna L" sort="Hudson, Anna L" uniqKey="Hudson A" first="Anna L." last="Hudson">Anna L. Hudson</name>
<affiliation wicri:level="3"><nlm:aff id="aff1"><addr-line>UMR_S1158, Inserm-Université Paris 6, Paris, France</addr-line>
</nlm:aff>
<country xml:lang="fr">France</country>
<wicri:regionArea>UMR_S1158, Inserm-Université Paris 6, Paris</wicri:regionArea>
<placeName><region type="region">Île-de-France</region>
<region type="old region">Île-de-France</region>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
<affiliation wicri:level="3"><nlm:aff id="aff3"><addr-line>Neuroscience Research Australia and University of New South Wales, Sydney, Australia</addr-line>
</nlm:aff>
<country xml:lang="fr">Australie</country>
<wicri:regionArea>Neuroscience Research Australia and University of New South Wales, Sydney</wicri:regionArea>
<placeName><settlement type="city">Sydney</settlement>
<region type="état">Nouvelle-Galles du Sud</region>
</placeName>
</affiliation>
</author>
<author><name sortKey="Ranohavimparany, Anja" sort="Ranohavimparany, Anja" uniqKey="Ranohavimparany A" first="Anja" last="Ranohavimparany">Anja Ranohavimparany</name>
<affiliation wicri:level="3"><nlm:aff id="aff1"><addr-line>UMR_S1158, Inserm-Université Paris 6, Paris, France</addr-line>
</nlm:aff>
<country xml:lang="fr">France</country>
<wicri:regionArea>UMR_S1158, Inserm-Université Paris 6, Paris</wicri:regionArea>
<placeName><region type="region">Île-de-France</region>
<region type="old region">Île-de-France</region>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
</author>
<author><name sortKey="Straus, Christian" sort="Straus, Christian" uniqKey="Straus C" first="Christian" last="Straus">Christian Straus</name>
<affiliation wicri:level="3"><nlm:aff id="aff1"><addr-line>UMR_S1158, Inserm-Université Paris 6, Paris, France</addr-line>
</nlm:aff>
<country xml:lang="fr">France</country>
<wicri:regionArea>UMR_S1158, Inserm-Université Paris 6, Paris</wicri:regionArea>
<placeName><region type="region">Île-de-France</region>
<region type="old region">Île-de-France</region>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
<affiliation wicri:level="3"><nlm:aff id="aff4"><addr-line>Service Central des Explorations Fonctionelles Respiratoires de l'Exercice et de la Dyspnée—Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Assistance Publique—Hôpitaux de Paris, Paris, France</addr-line>
</nlm:aff>
<country xml:lang="fr">France</country>
<wicri:regionArea>Service Central des Explorations Fonctionelles Respiratoires de l'Exercice et de la Dyspnée—Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Assistance Publique—Hôpitaux de Paris, Paris</wicri:regionArea>
<placeName><region type="region">Île-de-France</region>
<region type="old region">Île-de-France</region>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
<affiliation wicri:level="3"><nlm:aff id="aff5"><addr-line>Centre National de Référence Maladies Rares pour le syndrome d'Ondine (adultes)—Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Assistance Publique—Hôpitaux de Paris, Paris, France</addr-line>
</nlm:aff>
<country xml:lang="fr">France</country>
<wicri:regionArea>Centre National de Référence Maladies Rares pour le syndrome d'Ondine (adultes)—Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Assistance Publique—Hôpitaux de Paris, Paris</wicri:regionArea>
<placeName><region type="region">Île-de-France</region>
<region type="old region">Île-de-France</region>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
</author>
<author><name sortKey="Similowski, Thomas" sort="Similowski, Thomas" uniqKey="Similowski T" first="Thomas" last="Similowski">Thomas Similowski</name>
<affiliation wicri:level="3"><nlm:aff id="aff1"><addr-line>UMR_S1158, Inserm-Université Paris 6, Paris, France</addr-line>
</nlm:aff>
<country xml:lang="fr">France</country>
<wicri:regionArea>UMR_S1158, Inserm-Université Paris 6, Paris</wicri:regionArea>
<placeName><region type="region">Île-de-France</region>
<region type="old region">Île-de-France</region>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
<affiliation wicri:level="3"><nlm:aff id="aff5"><addr-line>Centre National de Référence Maladies Rares pour le syndrome d'Ondine (adultes)—Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Assistance Publique—Hôpitaux de Paris, Paris, France</addr-line>
</nlm:aff>
<country xml:lang="fr">France</country>
<wicri:regionArea>Centre National de Référence Maladies Rares pour le syndrome d'Ondine (adultes)—Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Assistance Publique—Hôpitaux de Paris, Paris</wicri:regionArea>
<placeName><region type="region">Île-de-France</region>
<region type="old region">Île-de-France</region>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
<affiliation wicri:level="3"><nlm:aff id="aff6"><addr-line>Service de Pneumologie et Réanimation Médicale—Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Assistance Publique—Hôpitaux de Paris, Paris, France</addr-line>
</nlm:aff>
<country xml:lang="fr">France</country>
<wicri:regionArea>Service de Pneumologie et Réanimation Médicale—Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Assistance Publique—Hôpitaux de Paris, Paris</wicri:regionArea>
<placeName><region type="region">Île-de-France</region>
<region type="old region">Île-de-France</region>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
</author>
</titleStmt>
<publicationStmt><idno type="wicri:source">PMC</idno>
<idno type="pmid">24475031</idno>
<idno type="pmc">3901646</idno>
<idno type="url">http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3901646</idno>
<idno type="RBID">PMC:3901646</idno>
<idno type="doi">10.1371/journal.pone.0084534</idno>
<date when="2014">2014</date>
<idno type="wicri:Area/Pmc/Corpus">002B43</idno>
<idno type="wicri:explorRef" wicri:stream="Pmc" wicri:step="Corpus" wicri:corpus="PMC">002B43</idno>
<idno type="wicri:Area/Pmc/Curation">002993</idno>
<idno type="wicri:explorRef" wicri:stream="Pmc" wicri:step="Curation">002993</idno>
<idno type="wicri:Area/Pmc/Checkpoint">001633</idno>
<idno type="wicri:explorRef" wicri:stream="Pmc" wicri:step="Checkpoint">001633</idno>
</publicationStmt>
<sourceDesc><biblStruct><analytic><title xml:lang="en" level="a" type="main">Does the Supplementary Motor Area Keep Patients with Ondine's Curse Syndrome Breathing While Awake?</title>
<author><name sortKey="Tremoureux, Lysandre" sort="Tremoureux, Lysandre" uniqKey="Tremoureux L" first="Lysandre" last="Tremoureux">Lysandre Tremoureux</name>
<affiliation wicri:level="3"><nlm:aff id="aff1"><addr-line>UMR_S1158, Inserm-Université Paris 6, Paris, France</addr-line>
</nlm:aff>
<country xml:lang="fr">France</country>
<wicri:regionArea>UMR_S1158, Inserm-Université Paris 6, Paris</wicri:regionArea>
<placeName><region type="region">Île-de-France</region>
<region type="old region">Île-de-France</region>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
</author>
<author><name sortKey="Raux, Mathieu" sort="Raux, Mathieu" uniqKey="Raux M" first="Mathieu" last="Raux">Mathieu Raux</name>
<affiliation wicri:level="3"><nlm:aff id="aff1"><addr-line>UMR_S1158, Inserm-Université Paris 6, Paris, France</addr-line>
</nlm:aff>
<country xml:lang="fr">France</country>
<wicri:regionArea>UMR_S1158, Inserm-Université Paris 6, Paris</wicri:regionArea>
<placeName><region type="region">Île-de-France</region>
<region type="old region">Île-de-France</region>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
<affiliation wicri:level="3"><nlm:aff id="aff2"><addr-line>Département d'Anesthésie Réanimation—Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Assistance Publique—Hôpitaux de Paris, Paris, France</addr-line>
</nlm:aff>
<country xml:lang="fr">France</country>
<wicri:regionArea>Département d'Anesthésie Réanimation—Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Assistance Publique—Hôpitaux de Paris, Paris</wicri:regionArea>
<placeName><region type="region">Île-de-France</region>
<region type="old region">Île-de-France</region>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
</author>
<author><name sortKey="Hudson, Anna L" sort="Hudson, Anna L" uniqKey="Hudson A" first="Anna L." last="Hudson">Anna L. Hudson</name>
<affiliation wicri:level="3"><nlm:aff id="aff1"><addr-line>UMR_S1158, Inserm-Université Paris 6, Paris, France</addr-line>
</nlm:aff>
<country xml:lang="fr">France</country>
<wicri:regionArea>UMR_S1158, Inserm-Université Paris 6, Paris</wicri:regionArea>
<placeName><region type="region">Île-de-France</region>
<region type="old region">Île-de-France</region>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
<affiliation wicri:level="3"><nlm:aff id="aff3"><addr-line>Neuroscience Research Australia and University of New South Wales, Sydney, Australia</addr-line>
</nlm:aff>
<country xml:lang="fr">Australie</country>
<wicri:regionArea>Neuroscience Research Australia and University of New South Wales, Sydney</wicri:regionArea>
<placeName><settlement type="city">Sydney</settlement>
<region type="état">Nouvelle-Galles du Sud</region>
</placeName>
</affiliation>
</author>
<author><name sortKey="Ranohavimparany, Anja" sort="Ranohavimparany, Anja" uniqKey="Ranohavimparany A" first="Anja" last="Ranohavimparany">Anja Ranohavimparany</name>
<affiliation wicri:level="3"><nlm:aff id="aff1"><addr-line>UMR_S1158, Inserm-Université Paris 6, Paris, France</addr-line>
</nlm:aff>
<country xml:lang="fr">France</country>
<wicri:regionArea>UMR_S1158, Inserm-Université Paris 6, Paris</wicri:regionArea>
<placeName><region type="region">Île-de-France</region>
<region type="old region">Île-de-France</region>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
</author>
<author><name sortKey="Straus, Christian" sort="Straus, Christian" uniqKey="Straus C" first="Christian" last="Straus">Christian Straus</name>
<affiliation wicri:level="3"><nlm:aff id="aff1"><addr-line>UMR_S1158, Inserm-Université Paris 6, Paris, France</addr-line>
</nlm:aff>
<country xml:lang="fr">France</country>
<wicri:regionArea>UMR_S1158, Inserm-Université Paris 6, Paris</wicri:regionArea>
<placeName><region type="region">Île-de-France</region>
<region type="old region">Île-de-France</region>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
<affiliation wicri:level="3"><nlm:aff id="aff4"><addr-line>Service Central des Explorations Fonctionelles Respiratoires de l'Exercice et de la Dyspnée—Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Assistance Publique—Hôpitaux de Paris, Paris, France</addr-line>
</nlm:aff>
<country xml:lang="fr">France</country>
<wicri:regionArea>Service Central des Explorations Fonctionelles Respiratoires de l'Exercice et de la Dyspnée—Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Assistance Publique—Hôpitaux de Paris, Paris</wicri:regionArea>
<placeName><region type="region">Île-de-France</region>
<region type="old region">Île-de-France</region>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
<affiliation wicri:level="3"><nlm:aff id="aff5"><addr-line>Centre National de Référence Maladies Rares pour le syndrome d'Ondine (adultes)—Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Assistance Publique—Hôpitaux de Paris, Paris, France</addr-line>
</nlm:aff>
<country xml:lang="fr">France</country>
<wicri:regionArea>Centre National de Référence Maladies Rares pour le syndrome d'Ondine (adultes)—Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Assistance Publique—Hôpitaux de Paris, Paris</wicri:regionArea>
<placeName><region type="region">Île-de-France</region>
<region type="old region">Île-de-France</region>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
</author>
<author><name sortKey="Similowski, Thomas" sort="Similowski, Thomas" uniqKey="Similowski T" first="Thomas" last="Similowski">Thomas Similowski</name>
<affiliation wicri:level="3"><nlm:aff id="aff1"><addr-line>UMR_S1158, Inserm-Université Paris 6, Paris, France</addr-line>
</nlm:aff>
<country xml:lang="fr">France</country>
<wicri:regionArea>UMR_S1158, Inserm-Université Paris 6, Paris</wicri:regionArea>
<placeName><region type="region">Île-de-France</region>
<region type="old region">Île-de-France</region>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
<affiliation wicri:level="3"><nlm:aff id="aff5"><addr-line>Centre National de Référence Maladies Rares pour le syndrome d'Ondine (adultes)—Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Assistance Publique—Hôpitaux de Paris, Paris, France</addr-line>
</nlm:aff>
<country xml:lang="fr">France</country>
<wicri:regionArea>Centre National de Référence Maladies Rares pour le syndrome d'Ondine (adultes)—Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Assistance Publique—Hôpitaux de Paris, Paris</wicri:regionArea>
<placeName><region type="region">Île-de-France</region>
<region type="old region">Île-de-France</region>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
<affiliation wicri:level="3"><nlm:aff id="aff6"><addr-line>Service de Pneumologie et Réanimation Médicale—Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Assistance Publique—Hôpitaux de Paris, Paris, France</addr-line>
</nlm:aff>
<country xml:lang="fr">France</country>
<wicri:regionArea>Service de Pneumologie et Réanimation Médicale—Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Assistance Publique—Hôpitaux de Paris, Paris</wicri:regionArea>
<placeName><region type="region">Île-de-France</region>
<region type="old region">Île-de-France</region>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
</author>
</analytic>
<series><title level="j">PLoS ONE</title>
<idno type="eISSN">1932-6203</idno>
<imprint><date when="2014">2014</date>
</imprint>
</series>
</biblStruct>
</sourceDesc>
</fileDesc>
<profileDesc><textClass></textClass>
</profileDesc>
</teiHeader>
<front><div type="abstract" xml:lang="en"><sec><title>Background</title>
<p>Congenital central hypoventilation syndrome (CCHS) is a rare neuro-respiratory disorder associated with mutations of the PHOX2B gene. Patients with this disease experience severe hypoventilation during sleep and are consequently ventilator-dependent. However, they breathe almost normally while awake, indicating the existence of cortical mechanisms compensating for the deficient brainstem generation of automatic breathing. Current evidence indicates that the supplementary motor area plays an important role in modulating ventilation in awake normal humans. We hypothesized that the wake-related maintenance of spontaneous breathing in patients with CCHS could involve supplementary motor area.</p>
</sec>
<sec><title>Methods</title>
<p>We studied 7 CCHS patients (5 women; age: 20–30; BMI: 22.1±4 kg.m<sup>−2</sup>
) during resting breathing and during exposure to carbon dioxide and inspiratory mechanical constraints. They were compared with 8 healthy individuals. Segments of electroencephalographic tracings were selected according to ventilatory flow signal, from 2.5 seconds to 1.5 seconds after the onset of inspiration. After artefact rejection, 80 or more such segments were ensemble averaged. A slow upward shift of the EEG signal starting between 2 and 0.5 s before inspiration (pre-inspiratory potential) was considered suggestive of supplementary motor area activation.</p>
</sec>
<sec><title>Results</title>
<p>In the control group, pre-inspiratory potentials were generally absent during resting breathing and carbon dioxide stimulation, and consistently identified in the presence of inspiratory constraints (expected). In CCHS patients, pre-inspiratory potentials were systematically identified in all study conditions, including resting breathing. They were therefore significantly more frequent than in controls.</p>
</sec>
<sec><title>Conclusions</title>
<p>This study provides a neurophysiological substrate to the wakefulness drive to breathe that is characteristic of CCHS and suggests that the supplementary motor area contributes to this phenomenon. Whether or not this “cortical breathing” can be taken advantage of therapeutically, or has clinical consequences (like competition with attentional resources) remains to be determined.</p>
</sec>
</div>
</front>
<back><div1 type="bibliography"><listBibl><biblStruct><analytic><author><name sortKey="Weese Mayer, De" uniqKey="Weese Mayer D">DE Weese-Mayer</name>
</author>
<author><name sortKey="Berry Kravis, Em" uniqKey="Berry Kravis E">EM Berry-Kravis</name>
</author>
<author><name sortKey="Ceccherini, I" uniqKey="Ceccherini I">I Ceccherini</name>
</author>
<author><name sortKey="Keens, Tg" uniqKey="Keens T">TG Keens</name>
</author>
<author><name sortKey="Loghmanee, Da" uniqKey="Loghmanee D">DA Loghmanee</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Dubreuil, V" uniqKey="Dubreuil V">V Dubreuil</name>
</author>
<author><name sortKey="Ramanantsoa, N" uniqKey="Ramanantsoa N">N Ramanantsoa</name>
</author>
<author><name sortKey="Trochet, D" uniqKey="Trochet D">D Trochet</name>
</author>
<author><name sortKey="Vaubourg, V" uniqKey="Vaubourg V">V Vaubourg</name>
</author>
<author><name sortKey="Amiel, J" uniqKey="Amiel J">J Amiel</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Shea, Sa" uniqKey="Shea S">SA Shea</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Shea, Sa" uniqKey="Shea S">SA Shea</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Hudson, Al" uniqKey="Hudson A">AL Hudson</name>
</author>
<author><name sortKey="Gandevia, Sc" uniqKey="Gandevia S">SC Gandevia</name>
</author>
<author><name sortKey="Butler, Je" uniqKey="Butler J">JE Butler</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Haouzi, P" uniqKey="Haouzi P">P Haouzi</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Corfield, Dr" uniqKey="Corfield D">DR Corfield</name>
</author>
<author><name sortKey="Morrell, Mj" uniqKey="Morrell M">MJ Morrell</name>
</author>
<author><name sortKey="Guz, A" uniqKey="Guz A">A Guz</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Datta, Ak" uniqKey="Datta A">AK Datta</name>
</author>
<author><name sortKey="Shea, Sa" uniqKey="Shea S">SA Shea</name>
</author>
<author><name sortKey="Horner, Rl" uniqKey="Horner R">RL Horner</name>
</author>
<author><name sortKey="Guz, A" uniqKey="Guz A">A Guz</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Wiegand, L" uniqKey="Wiegand L">L Wiegand</name>
</author>
<author><name sortKey="Zwillich, Cw" uniqKey="Zwillich C">CW Zwillich</name>
</author>
<author><name sortKey="White, Dp" uniqKey="White D">DP White</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Axen, K" uniqKey="Axen K">K Axen</name>
</author>
<author><name sortKey="Haas, Ss" uniqKey="Haas S">SS Haas</name>
</author>
<author><name sortKey="Haas, F" uniqKey="Haas F">F Haas</name>
</author>
<author><name sortKey="Gaudino, D" uniqKey="Gaudino D">D Gaudino</name>
</author>
<author><name sortKey="Haas, A" uniqKey="Haas A">A Haas</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Yanos, J" uniqKey="Yanos J">J Yanos</name>
</author>
<author><name sortKey="Banner, A" uniqKey="Banner A">A Banner</name>
</author>
<author><name sortKey="Stanko, R" uniqKey="Stanko R">R Stanko</name>
</author>
<author><name sortKey="Gentry, S" uniqKey="Gentry S">S Gentry</name>
</author>
<author><name sortKey="Greenawalt, K" uniqKey="Greenawalt K">K Greenawalt</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Raux, M" uniqKey="Raux M">M Raux</name>
</author>
<author><name sortKey="Straus, C" uniqKey="Straus C">C Straus</name>
</author>
<author><name sortKey="Redolfi, S" uniqKey="Redolfi S">S Redolfi</name>
</author>
<author><name sortKey="Morelot Panzini, C" uniqKey="Morelot Panzini C">C Morelot-Panzini</name>
</author>
<author><name sortKey="Couturier, A" uniqKey="Couturier A">A Couturier</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Tremoureux, L" uniqKey="Tremoureux L">L Tremoureux</name>
</author>
<author><name sortKey="Raux, M" uniqKey="Raux M">M Raux</name>
</author>
<author><name sortKey="Jutand, L" uniqKey="Jutand L">L Jutand</name>
</author>
<author><name sortKey="Similowski, T" uniqKey="Similowski T">T Similowski</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Raux, M" uniqKey="Raux M">M Raux</name>
</author>
<author><name sortKey="Xie, H" uniqKey="Xie H">H Xie</name>
</author>
<author><name sortKey="Similowski, T" uniqKey="Similowski T">T Similowski</name>
</author>
<author><name sortKey="Koski, L" uniqKey="Koski L">L Koski</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Sharshar, T" uniqKey="Sharshar T">T Sharshar</name>
</author>
<author><name sortKey="Hopkinson, Ns" uniqKey="Hopkinson N">NS Hopkinson</name>
</author>
<author><name sortKey="Jonville, S" uniqKey="Jonville S">S Jonville</name>
</author>
<author><name sortKey="Prigent, H" uniqKey="Prigent H">H Prigent</name>
</author>
<author><name sortKey="Carlier, R" uniqKey="Carlier R">R Carlier</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Laviolette, L" uniqKey="Laviolette L">L Laviolette</name>
</author>
<author><name sortKey="Nierat, Mc" uniqKey="Nierat M">MC Nierat</name>
</author>
<author><name sortKey="Hudson, Al" uniqKey="Hudson A">AL Hudson</name>
</author>
<author><name sortKey="Raux, M" uniqKey="Raux M">M Raux</name>
</author>
<author><name sortKey="Allard, E" uniqKey="Allard E">E Allard</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Straus, C" uniqKey="Straus C">C Straus</name>
</author>
<author><name sortKey="Similowski, T" uniqKey="Similowski T">T Similowski</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Eberhard, A" uniqKey="Eberhard A">A Eberhard</name>
</author>
<author><name sortKey="Calabrese, P" uniqKey="Calabrese P">P Calabrese</name>
</author>
<author><name sortKey="Baconnier, P" uniqKey="Baconnier P">P Baconnier</name>
</author>
<author><name sortKey="Benchetrit, G" uniqKey="Benchetrit G">G Benchetrit</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Raux, M" uniqKey="Raux M">M Raux</name>
</author>
<author><name sortKey="Ray, P" uniqKey="Ray P">P Ray</name>
</author>
<author><name sortKey="Prella, M" uniqKey="Prella M">M Prella</name>
</author>
<author><name sortKey="Duguet, A" uniqKey="Duguet A">A Duguet</name>
</author>
<author><name sortKey="Demoule, A" uniqKey="Demoule A">A Demoule</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Jutand, L" uniqKey="Jutand L">L Jutand</name>
</author>
<author><name sortKey="Tremoureux, L" uniqKey="Tremoureux L">L Tremoureux</name>
</author>
<author><name sortKey="Pichon, A" uniqKey="Pichon A">A Pichon</name>
</author>
<author><name sortKey="Delpech, N" uniqKey="Delpech N">N Delpech</name>
</author>
<author><name sortKey="Denjean, A" uniqKey="Denjean A">A Denjean</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Raux, M" uniqKey="Raux M">M Raux</name>
</author>
<author><name sortKey="Tremoureux, L" uniqKey="Tremoureux L">L Tremoureux</name>
</author>
<author><name sortKey="Couturier, A" uniqKey="Couturier A">A Couturier</name>
</author>
<author><name sortKey="Hug, F" uniqKey="Hug F">F Hug</name>
</author>
<author><name sortKey="Similowski, T" uniqKey="Similowski T">T Similowski</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Colebatch, Jg" uniqKey="Colebatch J">JG Colebatch</name>
</author>
<author><name sortKey="Adams, L" uniqKey="Adams L">L Adams</name>
</author>
<author><name sortKey="Murphy, K" uniqKey="Murphy K">K Murphy</name>
</author>
<author><name sortKey="Martin, Aj" uniqKey="Martin A">AJ Martin</name>
</author>
<author><name sortKey="Lammertsma, Aa" uniqKey="Lammertsma A">AA Lammertsma</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Mckay, Lc" uniqKey="Mckay L">LC McKay</name>
</author>
<author><name sortKey="Evans, Kc" uniqKey="Evans K">KC Evans</name>
</author>
<author><name sortKey="Frackowiak, Rs" uniqKey="Frackowiak R">RS Frackowiak</name>
</author>
<author><name sortKey="Corfield, Dr" uniqKey="Corfield D">DR Corfield</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Raux, M" uniqKey="Raux M">M Raux</name>
</author>
<author><name sortKey="Tyvaert, L" uniqKey="Tyvaert L">L Tyvaert</name>
</author>
<author><name sortKey="Ferreira, M" uniqKey="Ferreira M">M Ferreira</name>
</author>
<author><name sortKey="Kindler, F" uniqKey="Kindler F">F Kindler</name>
</author>
<author><name sortKey="Morelot Panzini, C" uniqKey="Morelot Panzini C">C Morélot-Panzini</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Haouzi, P" uniqKey="Haouzi P">P Haouzi</name>
</author>
<author><name sortKey="Bell, Hj" uniqKey="Bell H">HJ Bell</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Aminoff, Mj" uniqKey="Aminoff M">MJ Aminoff</name>
</author>
<author><name sortKey="Sears, Ta" uniqKey="Sears T">TA Sears</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Mehiri, S" uniqKey="Mehiri S">S Mehiri</name>
</author>
<author><name sortKey="Straus, C" uniqKey="Straus C">C Straus</name>
</author>
<author><name sortKey="Arnulf, I" uniqKey="Arnulf I">I Arnulf</name>
</author>
<author><name sortKey="Attali, V" uniqKey="Attali V">V Attali</name>
</author>
<author><name sortKey="Zelter, M" uniqKey="Zelter M">M Zelter</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Murphy, K" uniqKey="Murphy K">K Murphy</name>
</author>
<author><name sortKey="Mier, A" uniqKey="Mier A">A Mier</name>
</author>
<author><name sortKey="Adams, L" uniqKey="Adams L">L Adams</name>
</author>
<author><name sortKey="Guz, A" uniqKey="Guz A">A Guz</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Straus, C" uniqKey="Straus C">C Straus</name>
</author>
<author><name sortKey="Locher, C" uniqKey="Locher C">C Locher</name>
</author>
<author><name sortKey="Zelter, M" uniqKey="Zelter M">M Zelter</name>
</author>
<author><name sortKey="Derenne, Jp" uniqKey="Derenne J">JP Derenne</name>
</author>
<author><name sortKey="Similowski, T" uniqKey="Similowski T">T Similowski</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Davey, Nj" uniqKey="Davey N">NJ Davey</name>
</author>
<author><name sortKey="Murphy, K" uniqKey="Murphy K">K Murphy</name>
</author>
<author><name sortKey="Maskill, Dw" uniqKey="Maskill D">DW Maskill</name>
</author>
<author><name sortKey="Guz, A" uniqKey="Guz A">A Guz</name>
</author>
<author><name sortKey="Ellaway, Ph" uniqKey="Ellaway P">PH Ellaway</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Hudson, Al" uniqKey="Hudson A">AL Hudson</name>
</author>
<author><name sortKey="Butler, Je" uniqKey="Butler J">JE Butler</name>
</author>
<author><name sortKey="Gandevia, Sc" uniqKey="Gandevia S">SC Gandevia</name>
</author>
<author><name sortKey="De Troyer, A" uniqKey="De Troyer A">A De Troyer</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Hudson, Al" uniqKey="Hudson A">AL Hudson</name>
</author>
<author><name sortKey="Butler, Je" uniqKey="Butler J">JE Butler</name>
</author>
<author><name sortKey="Gandevia, Sc" uniqKey="Gandevia S">SC Gandevia</name>
</author>
<author><name sortKey="De Troyer, A" uniqKey="De Troyer A">A De Troyer</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Massimini, M" uniqKey="Massimini M">M Massimini</name>
</author>
<author><name sortKey="Ferrarelli, F" uniqKey="Ferrarelli F">F Ferrarelli</name>
</author>
<author><name sortKey="Huber, R" uniqKey="Huber R">R Huber</name>
</author>
<author><name sortKey="Esser, Sk" uniqKey="Esser S">SK Esser</name>
</author>
<author><name sortKey="Singh, H" uniqKey="Singh H">H Singh</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Wu, T" uniqKey="Wu T">T Wu</name>
</author>
<author><name sortKey="Kansaku, K" uniqKey="Kansaku K">K Kansaku</name>
</author>
<author><name sortKey="Hallett, M" uniqKey="Hallett M">M Hallett</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Passingham, Re" uniqKey="Passingham R">RE Passingham</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Shea, Sa" uniqKey="Shea S">SA Shea</name>
</author>
<author><name sortKey="Andres, Lp" uniqKey="Andres L">LP Andres</name>
</author>
<author><name sortKey="Paydarfar, D" uniqKey="Paydarfar D">D Paydarfar</name>
</author>
<author><name sortKey="Banzett, Rb" uniqKey="Banzett R">RB Banzett</name>
</author>
<author><name sortKey="Shannon, Dc" uniqKey="Shannon D">DC Shannon</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Guilleminault, C" uniqKey="Guilleminault C">C Guilleminault</name>
</author>
<author><name sortKey="Mcquitty, J" uniqKey="Mcquitty J">J McQuitty</name>
</author>
<author><name sortKey="Ariagno, Rl" uniqKey="Ariagno R">RL Ariagno</name>
</author>
<author><name sortKey="Challamel, Mj" uniqKey="Challamel M">MJ Challamel</name>
</author>
<author><name sortKey="Korobkin, R" uniqKey="Korobkin R">R Korobkin</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Denot Ledunois, S" uniqKey="Denot Ledunois S">S Denot-Ledunois</name>
</author>
<author><name sortKey="Vardon, G" uniqKey="Vardon G">G Vardon</name>
</author>
<author><name sortKey="Perruchet, P" uniqKey="Perruchet P">P Perruchet</name>
</author>
<author><name sortKey="Gallego, J" uniqKey="Gallego J">J Gallego</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Blanc Gras, N" uniqKey="Blanc Gras N">N Blanc-Gras</name>
</author>
<author><name sortKey="Esteve, F" uniqKey="Esteve F">F Esteve</name>
</author>
<author><name sortKey="Benchetrit, G" uniqKey="Benchetrit G">G Benchetrit</name>
</author>
<author><name sortKey="Gallego, J" uniqKey="Gallego J">J Gallego</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Gallego, J" uniqKey="Gallego J">J Gallego</name>
</author>
<author><name sortKey="Perruchet, P" uniqKey="Perruchet P">P Perruchet</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Cohen, E" uniqKey="Cohen E">E Cohen</name>
</author>
<author><name sortKey="Murphy, K" uniqKey="Murphy K">K Murphy</name>
</author>
<author><name sortKey="Adams, L" uniqKey="Adams L">L Adams</name>
</author>
<author><name sortKey="Guz, A" uniqKey="Guz A">A Guz</name>
</author>
<author><name sortKey="Benchetrit, G" uniqKey="Benchetrit G">G Benchetrit</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Li, S" uniqKey="Li S">S Li</name>
</author>
<author><name sortKey="Rymer, Wz" uniqKey="Rymer W">WZ Rymer</name>
</author>
</analytic>
</biblStruct>
</listBibl>
</div1>
</back>
</TEI>
</pmc>
<pubmed><TEI><teiHeader><fileDesc><titleStmt><title xml:lang="en">Does the supplementary motor area keep patients with Ondine's curse syndrome breathing while awake?</title>
<author><name sortKey="Tremoureux, Lysandre" sort="Tremoureux, Lysandre" uniqKey="Tremoureux L" first="Lysandre" last="Tremoureux">Lysandre Tremoureux</name>
<affiliation wicri:level="3"><nlm:affiliation>UMR_S1158, Inserm-Université Paris 6, Paris, France.</nlm:affiliation>
<country xml:lang="fr">France</country>
<wicri:regionArea>UMR_S1158, Inserm-Université Paris 6, Paris</wicri:regionArea>
<placeName><region type="region">Île-de-France</region>
<region type="old region">Île-de-France</region>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
</author>
<author><name sortKey="Raux, Mathieu" sort="Raux, Mathieu" uniqKey="Raux M" first="Mathieu" last="Raux">Mathieu Raux</name>
<affiliation wicri:level="3"><nlm:affiliation>UMR_S1158, Inserm-Université Paris 6, Paris, France ; Département d'Anesthésie Réanimation-Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Assistance Publique-Hôpitaux de Paris, Paris, France.</nlm:affiliation>
<country xml:lang="fr">France</country>
<wicri:regionArea>UMR_S1158, Inserm-Université Paris 6, Paris, France ; Département d'Anesthésie Réanimation-Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Assistance Publique-Hôpitaux de Paris, Paris</wicri:regionArea>
<placeName><region type="region">Île-de-France</region>
<region type="old region">Île-de-France</region>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
</author>
<author><name sortKey="Hudson, Anna L" sort="Hudson, Anna L" uniqKey="Hudson A" first="Anna L" last="Hudson">Anna L. Hudson</name>
<affiliation wicri:level="3"><nlm:affiliation>UMR_S1158, Inserm-Université Paris 6, Paris, France ; Neuroscience Research Australia and University of New South Wales, Sydney, Australia.</nlm:affiliation>
<country xml:lang="fr">Australie</country>
<wicri:regionArea>UMR_S1158, Inserm-Université Paris 6, Paris, France ; Neuroscience Research Australia and University of New South Wales, Sydney</wicri:regionArea>
<placeName><settlement type="city">Sydney</settlement>
<region type="état">Nouvelle-Galles du Sud</region>
</placeName>
</affiliation>
</author>
<author><name sortKey="Ranohavimparany, Anja" sort="Ranohavimparany, Anja" uniqKey="Ranohavimparany A" first="Anja" last="Ranohavimparany">Anja Ranohavimparany</name>
<affiliation wicri:level="3"><nlm:affiliation>UMR_S1158, Inserm-Université Paris 6, Paris, France.</nlm:affiliation>
<country xml:lang="fr">France</country>
<wicri:regionArea>UMR_S1158, Inserm-Université Paris 6, Paris</wicri:regionArea>
<placeName><region type="region">Île-de-France</region>
<region type="old region">Île-de-France</region>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
</author>
<author><name sortKey="Straus, Christian" sort="Straus, Christian" uniqKey="Straus C" first="Christian" last="Straus">Christian Straus</name>
<affiliation wicri:level="3"><nlm:affiliation>UMR_S1158, Inserm-Université Paris 6, Paris, France ; Service Central des Explorations Fonctionelles Respiratoires de l'Exercice et de la Dyspnée-Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Assistance Publique-Hôpitaux de Paris, Paris, France ; Centre National de Référence Maladies Rares pour le syndrome d'Ondine (adultes)-Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Assistance Publique-Hôpitaux de Paris, Paris, France.</nlm:affiliation>
<country xml:lang="fr">France</country>
<wicri:regionArea>UMR_S1158, Inserm-Université Paris 6, Paris, France ; Service Central des Explorations Fonctionelles Respiratoires de l'Exercice et de la Dyspnée-Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Assistance Publique-Hôpitaux de Paris, Paris, France ; Centre National de Référence Maladies Rares pour le syndrome d'Ondine (adultes)-Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Assistance Publique-Hôpitaux de Paris, Paris</wicri:regionArea>
<placeName><region type="region">Île-de-France</region>
<region type="old region">Île-de-France</region>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
</author>
<author><name sortKey="Similowski, Thomas" sort="Similowski, Thomas" uniqKey="Similowski T" first="Thomas" last="Similowski">Thomas Similowski</name>
<affiliation wicri:level="3"><nlm:affiliation>UMR_S1158, Inserm-Université Paris 6, Paris, France ; Centre National de Référence Maladies Rares pour le syndrome d'Ondine (adultes)-Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Assistance Publique-Hôpitaux de Paris, Paris, France ; Service de Pneumologie et Réanimation Médicale-Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Assistance Publique-Hôpitaux de Paris, Paris, France.</nlm:affiliation>
<country xml:lang="fr">France</country>
<wicri:regionArea>UMR_S1158, Inserm-Université Paris 6, Paris, France ; Centre National de Référence Maladies Rares pour le syndrome d'Ondine (adultes)-Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Assistance Publique-Hôpitaux de Paris, Paris, France ; Service de Pneumologie et Réanimation Médicale-Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Assistance Publique-Hôpitaux de Paris, Paris</wicri:regionArea>
<placeName><region type="region">Île-de-France</region>
<region type="old region">Île-de-France</region>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
</author>
</titleStmt>
<publicationStmt><idno type="wicri:source">PubMed</idno>
<date when="2014">2014</date>
<idno type="RBID">pubmed:24475031</idno>
<idno type="pmid">24475031</idno>
<idno type="doi">10.1371/journal.pone.0084534</idno>
<idno type="wicri:Area/PubMed/Corpus">003989</idno>
<idno type="wicri:explorRef" wicri:stream="PubMed" wicri:step="Corpus" wicri:corpus="PubMed">003989</idno>
<idno type="wicri:Area/PubMed/Curation">003873</idno>
<idno type="wicri:explorRef" wicri:stream="PubMed" wicri:step="Curation">003873</idno>
<idno type="wicri:Area/PubMed/Checkpoint">003873</idno>
<idno type="wicri:explorRef" wicri:stream="Checkpoint" wicri:step="PubMed">003873</idno>
</publicationStmt>
<sourceDesc><biblStruct><analytic><title xml:lang="en">Does the supplementary motor area keep patients with Ondine's curse syndrome breathing while awake?</title>
<author><name sortKey="Tremoureux, Lysandre" sort="Tremoureux, Lysandre" uniqKey="Tremoureux L" first="Lysandre" last="Tremoureux">Lysandre Tremoureux</name>
<affiliation wicri:level="3"><nlm:affiliation>UMR_S1158, Inserm-Université Paris 6, Paris, France.</nlm:affiliation>
<country xml:lang="fr">France</country>
<wicri:regionArea>UMR_S1158, Inserm-Université Paris 6, Paris</wicri:regionArea>
<placeName><region type="region">Île-de-France</region>
<region type="old region">Île-de-France</region>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
</author>
<author><name sortKey="Raux, Mathieu" sort="Raux, Mathieu" uniqKey="Raux M" first="Mathieu" last="Raux">Mathieu Raux</name>
<affiliation wicri:level="3"><nlm:affiliation>UMR_S1158, Inserm-Université Paris 6, Paris, France ; Département d'Anesthésie Réanimation-Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Assistance Publique-Hôpitaux de Paris, Paris, France.</nlm:affiliation>
<country xml:lang="fr">France</country>
<wicri:regionArea>UMR_S1158, Inserm-Université Paris 6, Paris, France ; Département d'Anesthésie Réanimation-Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Assistance Publique-Hôpitaux de Paris, Paris</wicri:regionArea>
<placeName><region type="region">Île-de-France</region>
<region type="old region">Île-de-France</region>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
</author>
<author><name sortKey="Hudson, Anna L" sort="Hudson, Anna L" uniqKey="Hudson A" first="Anna L" last="Hudson">Anna L. Hudson</name>
<affiliation wicri:level="3"><nlm:affiliation>UMR_S1158, Inserm-Université Paris 6, Paris, France ; Neuroscience Research Australia and University of New South Wales, Sydney, Australia.</nlm:affiliation>
<country xml:lang="fr">Australie</country>
<wicri:regionArea>UMR_S1158, Inserm-Université Paris 6, Paris, France ; Neuroscience Research Australia and University of New South Wales, Sydney</wicri:regionArea>
<placeName><settlement type="city">Sydney</settlement>
<region type="état">Nouvelle-Galles du Sud</region>
</placeName>
</affiliation>
</author>
<author><name sortKey="Ranohavimparany, Anja" sort="Ranohavimparany, Anja" uniqKey="Ranohavimparany A" first="Anja" last="Ranohavimparany">Anja Ranohavimparany</name>
<affiliation wicri:level="3"><nlm:affiliation>UMR_S1158, Inserm-Université Paris 6, Paris, France.</nlm:affiliation>
<country xml:lang="fr">France</country>
<wicri:regionArea>UMR_S1158, Inserm-Université Paris 6, Paris</wicri:regionArea>
<placeName><region type="region">Île-de-France</region>
<region type="old region">Île-de-France</region>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
</author>
<author><name sortKey="Straus, Christian" sort="Straus, Christian" uniqKey="Straus C" first="Christian" last="Straus">Christian Straus</name>
<affiliation wicri:level="3"><nlm:affiliation>UMR_S1158, Inserm-Université Paris 6, Paris, France ; Service Central des Explorations Fonctionelles Respiratoires de l'Exercice et de la Dyspnée-Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Assistance Publique-Hôpitaux de Paris, Paris, France ; Centre National de Référence Maladies Rares pour le syndrome d'Ondine (adultes)-Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Assistance Publique-Hôpitaux de Paris, Paris, France.</nlm:affiliation>
<country xml:lang="fr">France</country>
<wicri:regionArea>UMR_S1158, Inserm-Université Paris 6, Paris, France ; Service Central des Explorations Fonctionelles Respiratoires de l'Exercice et de la Dyspnée-Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Assistance Publique-Hôpitaux de Paris, Paris, France ; Centre National de Référence Maladies Rares pour le syndrome d'Ondine (adultes)-Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Assistance Publique-Hôpitaux de Paris, Paris</wicri:regionArea>
<placeName><region type="region">Île-de-France</region>
<region type="old region">Île-de-France</region>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
</author>
<author><name sortKey="Similowski, Thomas" sort="Similowski, Thomas" uniqKey="Similowski T" first="Thomas" last="Similowski">Thomas Similowski</name>
<affiliation wicri:level="3"><nlm:affiliation>UMR_S1158, Inserm-Université Paris 6, Paris, France ; Centre National de Référence Maladies Rares pour le syndrome d'Ondine (adultes)-Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Assistance Publique-Hôpitaux de Paris, Paris, France ; Service de Pneumologie et Réanimation Médicale-Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Assistance Publique-Hôpitaux de Paris, Paris, France.</nlm:affiliation>
<country xml:lang="fr">France</country>
<wicri:regionArea>UMR_S1158, Inserm-Université Paris 6, Paris, France ; Centre National de Référence Maladies Rares pour le syndrome d'Ondine (adultes)-Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Assistance Publique-Hôpitaux de Paris, Paris, France ; Service de Pneumologie et Réanimation Médicale-Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Assistance Publique-Hôpitaux de Paris, Paris</wicri:regionArea>
<placeName><region type="region">Île-de-France</region>
<region type="old region">Île-de-France</region>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
</author>
</analytic>
<series><title level="j">PloS one</title>
<idno type="eISSN">1932-6203</idno>
<imprint><date when="2014" type="published">2014</date>
</imprint>
</series>
</biblStruct>
</sourceDesc>
</fileDesc>
<profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Adult</term>
<term>Carbon Dioxide</term>
<term>Case-Control Studies</term>
<term>Electroencephalography</term>
<term>Female</term>
<term>Humans</term>
<term>Hypoventilation (congenital)</term>
<term>Hypoventilation (physiopathology)</term>
<term>Male</term>
<term>Motor Cortex (physiology)</term>
<term>Respiration</term>
<term>Sleep Apnea, Central (physiopathology)</term>
<term>Wakefulness</term>
<term>Young Adult</term>
</keywords>
<keywords scheme="KwdFr" xml:lang="fr"><term>Adulte</term>
<term>Apnée centrale du sommeil (physiopathologie)</term>
<term>Cortex moteur (physiologie)</term>
<term>Dioxyde de carbone</term>
<term>Femelle</term>
<term>Humains</term>
<term>Hypoventilation ()</term>
<term>Hypoventilation (physiopathologie)</term>
<term>Jeune adulte</term>
<term>Mâle</term>
<term>Respiration</term>
<term>Vigilance</term>
<term>Électroencéphalographie</term>
<term>Études cas-témoins</term>
</keywords>
<keywords scheme="MESH" type="chemical" xml:lang="en"><term>Carbon Dioxide</term>
</keywords>
<keywords scheme="MESH" qualifier="congenital" xml:lang="en"><term>Hypoventilation</term>
</keywords>
<keywords scheme="MESH" qualifier="physiologie" xml:lang="fr"><term>Cortex moteur</term>
</keywords>
<keywords scheme="MESH" qualifier="physiology" xml:lang="en"><term>Motor Cortex</term>
</keywords>
<keywords scheme="MESH" qualifier="physiopathologie" xml:lang="fr"><term>Apnée centrale du sommeil</term>
<term>Hypoventilation</term>
</keywords>
<keywords scheme="MESH" qualifier="physiopathology" xml:lang="en"><term>Hypoventilation</term>
<term>Sleep Apnea, Central</term>
</keywords>
<keywords scheme="MESH" xml:lang="en"><term>Adult</term>
<term>Case-Control Studies</term>
<term>Electroencephalography</term>
<term>Female</term>
<term>Humans</term>
<term>Male</term>
<term>Respiration</term>
<term>Wakefulness</term>
<term>Young Adult</term>
</keywords>
<keywords scheme="MESH" xml:lang="fr"><term>Adulte</term>
<term>Dioxyde de carbone</term>
<term>Femelle</term>
<term>Humains</term>
<term>Hypoventilation</term>
<term>Jeune adulte</term>
<term>Mâle</term>
<term>Respiration</term>
<term>Vigilance</term>
<term>Électroencéphalographie</term>
<term>Études cas-témoins</term>
</keywords>
</textClass>
</profileDesc>
</teiHeader>
<front><div type="abstract" xml:lang="en">Congenital central hypoventilation syndrome (CCHS) is a rare neuro-respiratory disorder associated with mutations of the PHOX2B gene. Patients with this disease experience severe hypoventilation during sleep and are consequently ventilator-dependent. However, they breathe almost normally while awake, indicating the existence of cortical mechanisms compensating for the deficient brainstem generation of automatic breathing. Current evidence indicates that the supplementary motor area plays an important role in modulating ventilation in awake normal humans. We hypothesized that the wake-related maintenance of spontaneous breathing in patients with CCHS could involve supplementary motor area.</div>
</front>
</TEI>
</pubmed>
</double>
</record>
Pour manipuler ce document sous Unix (Dilib)
EXPLOR_STEP=$WICRI_ROOT/Wicri/Asie/explor/AustralieFrV1/Data/Ncbi/Merge
HfdSelect -h $EXPLOR_STEP/biblio.hfd -nk 001752 | SxmlIndent | more
Ou
HfdSelect -h $EXPLOR_AREA/Data/Ncbi/Merge/biblio.hfd -nk 001752 | SxmlIndent | more
Pour mettre un lien sur cette page dans le réseau Wicri
{{Explor lien |wiki= Wicri/Asie |area= AustralieFrV1 |flux= Ncbi |étape= Merge |type= RBID |clé= PMC:3901646 |texte= Does the Supplementary Motor Area Keep Patients with Ondine's Curse Syndrome Breathing While Awake? }}
Pour générer des pages wiki
HfdIndexSelect -h $EXPLOR_AREA/Data/Ncbi/Merge/RBID.i -Sk "pubmed:24475031" \ | HfdSelect -Kh $EXPLOR_AREA/Data/Ncbi/Merge/biblio.hfd \ | NlmPubMed2Wicri -a AustralieFrV1
This area was generated with Dilib version V0.6.33. |