Movement Disorders (revue)

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Abnormal Response to Cortical Activation in Early Stages of Huntington Disease

Identifieur interne : 000132 ( France/Analysis ); précédent : 000131; suivant : 000133

Abnormal Response to Cortical Activation in Early Stages of Huntington Disease

Auteurs : Fanny Mochel [France] ; Tra-My N'Guyen [France] ; Dinesh Deelchand [États-Unis] ; Daisy Rinaldi [France] ; Romain Valabregue [France] ; Claire Wary [France] ; Pierre G. Carlier [France] ; Alexandra Durr [France] ; Pierre-Gilles Henry [États-Unis]

Source :

RBID : Pascal:12-0280059

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English descriptors

Abstract

Background: We wished to identify noninvasive in vivo biomarkers of brain energy deficit in Huntington disease. Methods: We studied 15 early affected patients (mean motor United Huntington Disease Rating Scale, 18 ± 9) and 15 age- and sex-matched controls. We coupled 31phosphorus nuclear magnetic resonance spectroscopy with activation of the occipital cortex in order to measure the relative concentrations of adenosine triphosphate, phosphocreatine, and inorganic phosphate before, during, and after visual stimulation. Results: In controls, we observed an 11% increase in the inorganic phosphate/phosphocreatine ratio (P = .024) and a 13% increase in the inorganic phosphate/ adenosine triphosphate ratio (P = .016) during brain activation, reflecting increased adenosine diphosphate concentrations. Subsequently, controls had a return to baseline levels during recovery (P = .012 and .022, respectively). In contrast, both ratios were unchanged in patients during and after visual stimulation. Conclusions: 31Phosphorus nuclear magnetic resonance spectroscopy could provide functional biomarkers of brain energy deficit to monitor therapeutic efficacy in Huntington disease.

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Pascal:12-0280059

Le document en format XML

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<name sortKey="Wary, Claire" sort="Wary, Claire" uniqKey="Wary C" first="Claire" last="Wary">Claire Wary</name>
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<s1>Université Pierre et Marie Curie</s1>
<s2>Paris</s2>
<s3>FRA</s3>
<sZ>1 aut.</sZ>
<sZ>5 aut.</sZ>
<sZ>6 aut.</sZ>
<sZ>7 aut.</sZ>
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<country>France</country>
<placeName>
<settlement type="city">Paris</settlement>
</placeName>
<orgName type="university">Université Pierre-et-Marie-Curie</orgName>
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<settlement type="city">Paris</settlement>
<region type="region" nuts="2">Île-de-France</region>
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<s1>Institut de Myologie, Laboratoire de RMN, Hôpital La Salpêtrière</s1>
<s2>Paris</s2>
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<sZ>6 aut.</sZ>
<sZ>7 aut.</sZ>
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<country>France</country>
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<settlement type="city">Paris</settlement>
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<s1>CLA, I
<sup>2</sup>
BM, MIRCen, IdM NMR Laboratory</s1>
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<s3>FRA</s3>
<sZ>6 aut.</sZ>
<sZ>7 aut.</sZ>
</inist:fA14>
<country>France</country>
<placeName>
<settlement type="city">Paris</settlement>
</placeName>
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<name sortKey="Carlier, Pierre G" sort="Carlier, Pierre G" uniqKey="Carlier P" first="Pierre G." last="Carlier">Pierre G. Carlier</name>
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<inist:fA14 i1="04">
<s1>Université Pierre et Marie Curie</s1>
<s2>Paris</s2>
<s3>FRA</s3>
<sZ>1 aut.</sZ>
<sZ>5 aut.</sZ>
<sZ>6 aut.</sZ>
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<country>France</country>
<placeName>
<settlement type="city">Paris</settlement>
</placeName>
<orgName type="university">Université Pierre-et-Marie-Curie</orgName>
<placeName>
<settlement type="city">Paris</settlement>
<region type="region" nuts="2">Île-de-France</region>
</placeName>
</affiliation>
<affiliation wicri:level="1">
<inist:fA14 i1="07">
<s1>Institut de Myologie, Laboratoire de RMN, Hôpital La Salpêtrière</s1>
<s2>Paris</s2>
<s3>FRA</s3>
<sZ>6 aut.</sZ>
<sZ>7 aut.</sZ>
</inist:fA14>
<country>France</country>
<placeName>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
<affiliation wicri:level="1">
<inist:fA14 i1="08">
<s1>CLA, I
<sup>2</sup>
BM, MIRCen, IdM NMR Laboratory</s1>
<s2>Paris</s2>
<s3>FRA</s3>
<sZ>6 aut.</sZ>
<sZ>7 aut.</sZ>
</inist:fA14>
<country>France</country>
<placeName>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
</author>
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<name sortKey="Durr, Alexandra" sort="Durr, Alexandra" uniqKey="Durr A" first="Alexandra" last="Durr">Alexandra Durr</name>
<affiliation wicri:level="1">
<inist:fA14 i1="01">
<s1>INSERM UMR S975, Institut du Cerveau et de la Moelle, Hôpital La Salpêtrière</s1>
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<s3>FRA</s3>
<sZ>1 aut.</sZ>
<sZ>2 aut.</sZ>
<sZ>4 aut.</sZ>
<sZ>5 aut.</sZ>
<sZ>8 aut.</sZ>
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<country>France</country>
<placeName>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
<affiliation wicri:level="1">
<inist:fA14 i1="02">
<s1>AP-HP, Département de Génétique, Hôpital La Salpêtrière</s1>
<s2>Paris</s2>
<s3>FRA</s3>
<sZ>1 aut.</sZ>
<sZ>8 aut.</sZ>
</inist:fA14>
<country>France</country>
<placeName>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
</author>
<author>
<name sortKey="Henry, Pierre Gilles" sort="Henry, Pierre Gilles" uniqKey="Henry P" first="Pierre-Gilles" last="Henry">Pierre-Gilles Henry</name>
<affiliation wicri:level="2">
<inist:fA14 i1="05">
<s1>University of Minnesota, Center for Magnetic Resonance Research, 2021 6th Street SE</s1>
<s2>Minneapolis, Minnesota</s2>
<s3>USA</s3>
<sZ>3 aut.</sZ>
<sZ>9 aut.</sZ>
</inist:fA14>
<country>États-Unis</country>
<placeName>
<region type="state">Minnesota</region>
</placeName>
</affiliation>
</author>
</analytic>
<series>
<title level="j" type="main">Movement disorders</title>
<title level="j" type="abbreviated">Mov. disord.</title>
<idno type="ISSN">0885-3185</idno>
<imprint>
<date when="2012">2012</date>
</imprint>
</series>
</biblStruct>
</sourceDesc>
<seriesStmt>
<title level="j" type="main">Movement disorders</title>
<title level="j" type="abbreviated">Mov. disord.</title>
<idno type="ISSN">0885-3185</idno>
</seriesStmt>
</fileDesc>
<profileDesc>
<textClass>
<keywords scheme="KwdEn" xml:lang="en">
<term>Adenosine Triphosphate (metabolism)</term>
<term>Case-Control Studies</term>
<term>Cerebral Cortex (metabolism)</term>
<term>Cerebral Cortex (physiopathology)</term>
<term>Cerebral Cortex (radionuclide imaging)</term>
<term>Early stage</term>
<term>Energy metabolism</term>
<term>Female</term>
<term>Humans</term>
<term>Huntington Disease (pathology)</term>
<term>Huntington Disease (radionuclide imaging)</term>
<term>Huntington disease</term>
<term>Linear Models</term>
<term>Magnetic Resonance Spectroscopy</term>
<term>Male</term>
<term>Nervous system diseases</term>
<term>Phosphates (metabolism)</term>
<term>Phosphocreatine (metabolism)</term>
<term>Phosphorus Isotopes (metabolism)</term>
<term>Spectrometry</term>
</keywords>
<keywords scheme="MESH" type="chemical" qualifier="metabolism" xml:lang="en">
<term>Adenosine Triphosphate</term>
<term>Phosphates</term>
<term>Phosphocreatine</term>
<term>Phosphorus Isotopes</term>
</keywords>
<keywords scheme="MESH" qualifier="metabolism" xml:lang="en">
<term>Cerebral Cortex</term>
</keywords>
<keywords scheme="MESH" qualifier="pathology" xml:lang="en">
<term>Huntington Disease</term>
</keywords>
<keywords scheme="MESH" qualifier="physiopathology" xml:lang="en">
<term>Cerebral Cortex</term>
</keywords>
<keywords scheme="MESH" qualifier="radionuclide imaging" xml:lang="en">
<term>Cerebral Cortex</term>
<term>Huntington Disease</term>
</keywords>
<keywords scheme="MESH" xml:lang="en">
<term>Case-Control Studies</term>
<term>Female</term>
<term>Humans</term>
<term>Linear Models</term>
<term>Magnetic Resonance Spectroscopy</term>
<term>Male</term>
</keywords>
<keywords scheme="Pascal" xml:lang="fr">
<term>Chorée de Huntington</term>
<term>Pathologie du système nerveux</term>
<term>Stade précoce</term>
<term>Spectrométrie</term>
<term>Métabolisme énergétique</term>
</keywords>
<keywords scheme="Wicri" type="topic" xml:lang="fr">
<term>Spectrométrie</term>
</keywords>
<keywords scheme="mix" xml:lang="en">
<term>Huntington disease</term>
<term>NMR spectroscopy</term>
<term>energy metabolism</term>
</keywords>
</textClass>
</profileDesc>
</teiHeader>
<front>
<div type="abstract" xml:lang="en">Background: We wished to identify noninvasive in vivo biomarkers of brain energy deficit in Huntington disease. Methods: We studied 15 early affected patients (mean motor United Huntington Disease Rating Scale, 18 ± 9) and 15 age- and sex-matched controls. We coupled
<sup>31</sup>
phosphorus nuclear magnetic resonance spectroscopy with activation of the occipital cortex in order to measure the relative concentrations of adenosine triphosphate, phosphocreatine, and inorganic phosphate before, during, and after visual stimulation. Results: In controls, we observed an 11% increase in the inorganic phosphate/phosphocreatine ratio (P = .024) and a 13% increase in the inorganic phosphate/ adenosine triphosphate ratio (P = .016) during brain activation, reflecting increased adenosine diphosphate concentrations. Subsequently, controls had a return to baseline levels during recovery (P = .012 and .022, respectively). In contrast, both ratios were unchanged in patients during and after visual stimulation. Conclusions:
<sup>31</sup>
Phosphorus nuclear magnetic resonance spectroscopy could provide functional biomarkers of brain energy deficit to monitor therapeutic efficacy in Huntington disease.</div>
</front>
</TEI>
<affiliations>
<list>
<country>
<li>France</li>
<li>États-Unis</li>
</country>
<region>
<li>Minnesota</li>
<li>Île-de-France</li>
</region>
<settlement>
<li>Paris</li>
</settlement>
<orgName>
<li>Université Pierre-et-Marie-Curie</li>
</orgName>
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<name sortKey="Carlier, Pierre G" sort="Carlier, Pierre G" uniqKey="Carlier P" first="Pierre G." last="Carlier">Pierre G. Carlier</name>
<name sortKey="Carlier, Pierre G" sort="Carlier, Pierre G" uniqKey="Carlier P" first="Pierre G." last="Carlier">Pierre G. Carlier</name>
<name sortKey="Durr, Alexandra" sort="Durr, Alexandra" uniqKey="Durr A" first="Alexandra" last="Durr">Alexandra Durr</name>
<name sortKey="Durr, Alexandra" sort="Durr, Alexandra" uniqKey="Durr A" first="Alexandra" last="Durr">Alexandra Durr</name>
<name sortKey="Mochel, Fanny" sort="Mochel, Fanny" uniqKey="Mochel F" first="Fanny" last="Mochel">Fanny Mochel</name>
<name sortKey="Mochel, Fanny" sort="Mochel, Fanny" uniqKey="Mochel F" first="Fanny" last="Mochel">Fanny Mochel</name>
<name sortKey="Mochel, Fanny" sort="Mochel, Fanny" uniqKey="Mochel F" first="Fanny" last="Mochel">Fanny Mochel</name>
<name sortKey="N Guyen, Tra My" sort="N Guyen, Tra My" uniqKey="N Guyen T" first="Tra-My" last="N'Guyen">Tra-My N'Guyen</name>
<name sortKey="Rinaldi, Daisy" sort="Rinaldi, Daisy" uniqKey="Rinaldi D" first="Daisy" last="Rinaldi">Daisy Rinaldi</name>
<name sortKey="Rinaldi, Daisy" sort="Rinaldi, Daisy" uniqKey="Rinaldi D" first="Daisy" last="Rinaldi">Daisy Rinaldi</name>
<name sortKey="Valabregue, Romain" sort="Valabregue, Romain" uniqKey="Valabregue R" first="Romain" last="Valabregue">Romain Valabregue</name>
<name sortKey="Valabregue, Romain" sort="Valabregue, Romain" uniqKey="Valabregue R" first="Romain" last="Valabregue">Romain Valabregue</name>
<name sortKey="Valabregue, Romain" sort="Valabregue, Romain" uniqKey="Valabregue R" first="Romain" last="Valabregue">Romain Valabregue</name>
<name sortKey="Wary, Claire" sort="Wary, Claire" uniqKey="Wary C" first="Claire" last="Wary">Claire Wary</name>
<name sortKey="Wary, Claire" sort="Wary, Claire" uniqKey="Wary C" first="Claire" last="Wary">Claire Wary</name>
<name sortKey="Wary, Claire" sort="Wary, Claire" uniqKey="Wary C" first="Claire" last="Wary">Claire Wary</name>
</country>
<country name="États-Unis">
<region name="Minnesota">
<name sortKey="Deelchand, Dinesh" sort="Deelchand, Dinesh" uniqKey="Deelchand D" first="Dinesh" last="Deelchand">Dinesh Deelchand</name>
</region>
<name sortKey="Henry, Pierre Gilles" sort="Henry, Pierre Gilles" uniqKey="Henry P" first="Pierre-Gilles" last="Henry">Pierre-Gilles Henry</name>
</country>
</tree>
</affiliations>
</record>

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