Brain metabolite alterations and cognitive dysfunction in early Huntington’s Disease
Identifieur interne : 000206 ( Pmc/Checkpoint ); précédent : 000205; suivant : 000207Brain metabolite alterations and cognitive dysfunction in early Huntington’s Disease
Auteurs : Paul G. Unschuld [États-Unis] ; Richard A. E. Edden [États-Unis] ; Aaron Carass [États-Unis] ; Xinyang Liu [États-Unis] ; Megan Shanahan [États-Unis] ; Xin Wang [États-Unis] ; Kenichi Oishi [États-Unis] ; Jason Brandt [États-Unis] ; Susan S. Bassett [États-Unis] ; Graham W. Redgrave [États-Unis] ; Russell L. Margolis [États-Unis] ; Peter C. M. Van Zijl [États-Unis] ; Peter B. Barker [États-Unis] ; Christopher A. Ross [États-Unis]Source :
- Movement Disorders [ 0885-3185 ] ; 2012.
Abstract
Huntington’s Disease (HD) is a neurodegenerative disorder characterized by early cognitive decline, which progresses at later stages to dementia and severe movement disorder. HD is caused by a cytosine-adenine-guanine triplet-repeat expansion mutation in the
Twelve individuals with the HD-mutation in premanifest or early stage of disease versus twelve healthy controls underwent 1H magnetic-resonance-spectroscopy (7.2ml voxel in the posterior cingulate cortex) at 7-Tesla, and also T1-weighted structural magnetic-resonance-imaging. All participants received standardized tests of cognitive functioning including the Montreal Cognitive Assessment and standardized quantified neurological examination within an hour before scanning.
Individuals with the HD mutation had significantly lower posterior cingulate cortex N-acetylaspartate (−9.6%, p=0.02) and glutamate levels (−10.1%, p=0.02) than controls. By contrast, in this small group, measures of brain morphology including striatal and ventricle volumes did not differ significantly. Linear regression with Montreal Cognitive Assessment scores revealed significant correlations with N-acetylaspartate (r2=0.50, p=0.01) and glutamate (r2=0.64, p=0.002) in HD subjects.
Our data suggest a relationship between reduced N-acetylaspartate and glutamate levels in the posterior cingulate cortex with cognitive decline in early stages of HD. N-acetylaspartate and glutamate magnetic-resonance-spectroscopy signals of the posterior cingulate cortex region may serve as potential biomarkers of disease progression or treatment outcome in HD and other neurodegenerative disorders with early cognitive dysfunction, when structural brain changes are still minor.
Url:
DOI: 10.1002/mds.25010
PubMed: 22649062
PubMed Central: 3383395
Affiliations:
Links toward previous steps (curation, corpus...)
Links to Exploration step
PMC:3383395Le document en format XML
<record><TEI><teiHeader><fileDesc><titleStmt><title xml:lang="en">Brain metabolite alterations and cognitive dysfunction in early Huntington’s Disease</title>
<author><name sortKey="Unschuld, Paul G" sort="Unschuld, Paul G" uniqKey="Unschuld P" first="Paul G." last="Unschuld">Paul G. Unschuld</name>
<affiliation wicri:level="2"><nlm:aff id="A1">Department of Psychiatry and Behavioral Sciences, Johns Hopkins University School of Medicine, Baltimore, MD, USA</nlm:aff>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>Department of Psychiatry and Behavioral Sciences, Johns Hopkins University School of Medicine, Baltimore, MD</wicri:regionArea>
<placeName><region type="state">Maryland</region>
</placeName>
</affiliation>
</author>
<author><name sortKey="Edden, Richard A E" sort="Edden, Richard A E" uniqKey="Edden R" first="Richard A. E." last="Edden">Richard A. E. Edden</name>
<affiliation wicri:level="2"><nlm:aff id="A4">The Russell H. Morgan Department of Radiology and Radiological Science, Division of MR Research, Johns Hopkins University School of Medicine, Baltimore, MD, USA</nlm:aff>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>The Russell H. Morgan Department of Radiology and Radiological Science, Division of MR Research, Johns Hopkins University School of Medicine, Baltimore, MD</wicri:regionArea>
<placeName><region type="state">Maryland</region>
</placeName>
</affiliation>
<affiliation wicri:level="2"><nlm:aff id="A5">F.M. Kirby Research Center for Functional Brain Imaging, Kennedy Krieger Institute, Baltimore, MD, USA</nlm:aff>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>F.M. Kirby Research Center for Functional Brain Imaging, Kennedy Krieger Institute, Baltimore, MD</wicri:regionArea>
<placeName><region type="state">Maryland</region>
</placeName>
</affiliation>
</author>
<author><name sortKey="Carass, Aaron" sort="Carass, Aaron" uniqKey="Carass A" first="Aaron" last="Carass">Aaron Carass</name>
<affiliation wicri:level="2"><nlm:aff id="A4">The Russell H. Morgan Department of Radiology and Radiological Science, Division of MR Research, Johns Hopkins University School of Medicine, Baltimore, MD, USA</nlm:aff>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>The Russell H. Morgan Department of Radiology and Radiological Science, Division of MR Research, Johns Hopkins University School of Medicine, Baltimore, MD</wicri:regionArea>
<placeName><region type="state">Maryland</region>
</placeName>
</affiliation>
</author>
<author><name sortKey="Liu, Xinyang" sort="Liu, Xinyang" uniqKey="Liu X" first="Xinyang" last="Liu">Xinyang Liu</name>
<affiliation wicri:level="2"><nlm:aff id="A6">Brigham and Women’s Hospital, Harvard Medical School, Boston, MA, USA</nlm:aff>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>Brigham and Women’s Hospital, Harvard Medical School, Boston, MA</wicri:regionArea>
<placeName><region type="state">Massachusetts</region>
</placeName>
</affiliation>
</author>
<author><name sortKey="Shanahan, Megan" sort="Shanahan, Megan" uniqKey="Shanahan M" first="Megan" last="Shanahan">Megan Shanahan</name>
<affiliation wicri:level="2"><nlm:aff id="A1">Department of Psychiatry and Behavioral Sciences, Johns Hopkins University School of Medicine, Baltimore, MD, USA</nlm:aff>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>Department of Psychiatry and Behavioral Sciences, Johns Hopkins University School of Medicine, Baltimore, MD</wicri:regionArea>
<placeName><region type="state">Maryland</region>
</placeName>
</affiliation>
</author>
<author><name sortKey="Wang, Xin" sort="Wang, Xin" uniqKey="Wang X" first="Xin" last="Wang">Xin Wang</name>
<affiliation wicri:level="2"><nlm:aff id="A4">The Russell H. Morgan Department of Radiology and Radiological Science, Division of MR Research, Johns Hopkins University School of Medicine, Baltimore, MD, USA</nlm:aff>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>The Russell H. Morgan Department of Radiology and Radiological Science, Division of MR Research, Johns Hopkins University School of Medicine, Baltimore, MD</wicri:regionArea>
<placeName><region type="state">Maryland</region>
</placeName>
</affiliation>
</author>
<author><name sortKey="Oishi, Kenichi" sort="Oishi, Kenichi" uniqKey="Oishi K" first="Kenichi" last="Oishi">Kenichi Oishi</name>
<affiliation wicri:level="2"><nlm:aff id="A4">The Russell H. Morgan Department of Radiology and Radiological Science, Division of MR Research, Johns Hopkins University School of Medicine, Baltimore, MD, USA</nlm:aff>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>The Russell H. Morgan Department of Radiology and Radiological Science, Division of MR Research, Johns Hopkins University School of Medicine, Baltimore, MD</wicri:regionArea>
<placeName><region type="state">Maryland</region>
</placeName>
</affiliation>
</author>
<author><name sortKey="Brandt, Jason" sort="Brandt, Jason" uniqKey="Brandt J" first="Jason" last="Brandt">Jason Brandt</name>
<affiliation wicri:level="2"><nlm:aff id="A1">Department of Psychiatry and Behavioral Sciences, Johns Hopkins University School of Medicine, Baltimore, MD, USA</nlm:aff>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>Department of Psychiatry and Behavioral Sciences, Johns Hopkins University School of Medicine, Baltimore, MD</wicri:regionArea>
<placeName><region type="state">Maryland</region>
</placeName>
</affiliation>
<affiliation wicri:level="2"><nlm:aff id="A2">Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, MD, USA</nlm:aff>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, MD</wicri:regionArea>
<placeName><region type="state">Maryland</region>
</placeName>
</affiliation>
</author>
<author><name sortKey="Bassett, Susan S" sort="Bassett, Susan S" uniqKey="Bassett S" first="Susan S." last="Bassett">Susan S. Bassett</name>
<affiliation wicri:level="2"><nlm:aff id="A1">Department of Psychiatry and Behavioral Sciences, Johns Hopkins University School of Medicine, Baltimore, MD, USA</nlm:aff>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>Department of Psychiatry and Behavioral Sciences, Johns Hopkins University School of Medicine, Baltimore, MD</wicri:regionArea>
<placeName><region type="state">Maryland</region>
</placeName>
</affiliation>
</author>
<author><name sortKey="Redgrave, Graham W" sort="Redgrave, Graham W" uniqKey="Redgrave G" first="Graham W." last="Redgrave">Graham W. Redgrave</name>
<affiliation wicri:level="2"><nlm:aff id="A1">Department of Psychiatry and Behavioral Sciences, Johns Hopkins University School of Medicine, Baltimore, MD, USA</nlm:aff>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>Department of Psychiatry and Behavioral Sciences, Johns Hopkins University School of Medicine, Baltimore, MD</wicri:regionArea>
<placeName><region type="state">Maryland</region>
</placeName>
</affiliation>
</author>
<author><name sortKey="Margolis, Russell L" sort="Margolis, Russell L" uniqKey="Margolis R" first="Russell L." last="Margolis">Russell L. Margolis</name>
<affiliation wicri:level="2"><nlm:aff id="A1">Department of Psychiatry and Behavioral Sciences, Johns Hopkins University School of Medicine, Baltimore, MD, USA</nlm:aff>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>Department of Psychiatry and Behavioral Sciences, Johns Hopkins University School of Medicine, Baltimore, MD</wicri:regionArea>
<placeName><region type="state">Maryland</region>
</placeName>
</affiliation>
<affiliation wicri:level="2"><nlm:aff id="A2">Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, MD, USA</nlm:aff>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, MD</wicri:regionArea>
<placeName><region type="state">Maryland</region>
</placeName>
</affiliation>
</author>
<author><name sortKey="Van Zijl, Peter C M" sort="Van Zijl, Peter C M" uniqKey="Van Zijl P" first="Peter C. M." last="Van Zijl">Peter C. M. Van Zijl</name>
<affiliation wicri:level="2"><nlm:aff id="A4">The Russell H. Morgan Department of Radiology and Radiological Science, Division of MR Research, Johns Hopkins University School of Medicine, Baltimore, MD, USA</nlm:aff>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>The Russell H. Morgan Department of Radiology and Radiological Science, Division of MR Research, Johns Hopkins University School of Medicine, Baltimore, MD</wicri:regionArea>
<placeName><region type="state">Maryland</region>
</placeName>
</affiliation>
<affiliation wicri:level="2"><nlm:aff id="A5">F.M. Kirby Research Center for Functional Brain Imaging, Kennedy Krieger Institute, Baltimore, MD, USA</nlm:aff>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>F.M. Kirby Research Center for Functional Brain Imaging, Kennedy Krieger Institute, Baltimore, MD</wicri:regionArea>
<placeName><region type="state">Maryland</region>
</placeName>
</affiliation>
</author>
<author><name sortKey="Barker, Peter B" sort="Barker, Peter B" uniqKey="Barker P" first="Peter B." last="Barker">Peter B. Barker</name>
<affiliation wicri:level="2"><nlm:aff id="A4">The Russell H. Morgan Department of Radiology and Radiological Science, Division of MR Research, Johns Hopkins University School of Medicine, Baltimore, MD, USA</nlm:aff>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>The Russell H. Morgan Department of Radiology and Radiological Science, Division of MR Research, Johns Hopkins University School of Medicine, Baltimore, MD</wicri:regionArea>
<placeName><region type="state">Maryland</region>
</placeName>
</affiliation>
<affiliation wicri:level="2"><nlm:aff id="A5">F.M. Kirby Research Center for Functional Brain Imaging, Kennedy Krieger Institute, Baltimore, MD, USA</nlm:aff>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>F.M. Kirby Research Center for Functional Brain Imaging, Kennedy Krieger Institute, Baltimore, MD</wicri:regionArea>
<placeName><region type="state">Maryland</region>
</placeName>
</affiliation>
</author>
<author><name sortKey="Ross, Christopher A" sort="Ross, Christopher A" uniqKey="Ross C" first="Christopher A." last="Ross">Christopher A. Ross</name>
<affiliation wicri:level="2"><nlm:aff id="A1">Department of Psychiatry and Behavioral Sciences, Johns Hopkins University School of Medicine, Baltimore, MD, USA</nlm:aff>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>Department of Psychiatry and Behavioral Sciences, Johns Hopkins University School of Medicine, Baltimore, MD</wicri:regionArea>
<placeName><region type="state">Maryland</region>
</placeName>
</affiliation>
<affiliation wicri:level="2"><nlm:aff id="A2">Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, MD, USA</nlm:aff>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, MD</wicri:regionArea>
<placeName><region type="state">Maryland</region>
</placeName>
</affiliation>
<affiliation wicri:level="2"><nlm:aff id="A3">Department of Neuroscience and Pharmacology, Johns Hopkins University School of Medicine, Baltimore, MD, USA</nlm:aff>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>Department of Neuroscience and Pharmacology, Johns Hopkins University School of Medicine, Baltimore, MD</wicri:regionArea>
<placeName><region type="state">Maryland</region>
</placeName>
</affiliation>
</author>
</titleStmt>
<publicationStmt><idno type="wicri:source">PMC</idno>
<idno type="pmid">22649062</idno>
<idno type="pmc">3383395</idno>
<idno type="url">http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3383395</idno>
<idno type="RBID">PMC:3383395</idno>
<idno type="doi">10.1002/mds.25010</idno>
<date when="2012">2012</date>
<idno type="wicri:Area/Pmc/Corpus">000194</idno>
<idno type="wicri:Area/Pmc/Curation">000194</idno>
<idno type="wicri:Area/Pmc/Checkpoint">000206</idno>
</publicationStmt>
<sourceDesc><biblStruct><analytic><title xml:lang="en" level="a" type="main">Brain metabolite alterations and cognitive dysfunction in early Huntington’s Disease</title>
<author><name sortKey="Unschuld, Paul G" sort="Unschuld, Paul G" uniqKey="Unschuld P" first="Paul G." last="Unschuld">Paul G. Unschuld</name>
<affiliation wicri:level="2"><nlm:aff id="A1">Department of Psychiatry and Behavioral Sciences, Johns Hopkins University School of Medicine, Baltimore, MD, USA</nlm:aff>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>Department of Psychiatry and Behavioral Sciences, Johns Hopkins University School of Medicine, Baltimore, MD</wicri:regionArea>
<placeName><region type="state">Maryland</region>
</placeName>
</affiliation>
</author>
<author><name sortKey="Edden, Richard A E" sort="Edden, Richard A E" uniqKey="Edden R" first="Richard A. E." last="Edden">Richard A. E. Edden</name>
<affiliation wicri:level="2"><nlm:aff id="A4">The Russell H. Morgan Department of Radiology and Radiological Science, Division of MR Research, Johns Hopkins University School of Medicine, Baltimore, MD, USA</nlm:aff>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>The Russell H. Morgan Department of Radiology and Radiological Science, Division of MR Research, Johns Hopkins University School of Medicine, Baltimore, MD</wicri:regionArea>
<placeName><region type="state">Maryland</region>
</placeName>
</affiliation>
<affiliation wicri:level="2"><nlm:aff id="A5">F.M. Kirby Research Center for Functional Brain Imaging, Kennedy Krieger Institute, Baltimore, MD, USA</nlm:aff>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>F.M. Kirby Research Center for Functional Brain Imaging, Kennedy Krieger Institute, Baltimore, MD</wicri:regionArea>
<placeName><region type="state">Maryland</region>
</placeName>
</affiliation>
</author>
<author><name sortKey="Carass, Aaron" sort="Carass, Aaron" uniqKey="Carass A" first="Aaron" last="Carass">Aaron Carass</name>
<affiliation wicri:level="2"><nlm:aff id="A4">The Russell H. Morgan Department of Radiology and Radiological Science, Division of MR Research, Johns Hopkins University School of Medicine, Baltimore, MD, USA</nlm:aff>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>The Russell H. Morgan Department of Radiology and Radiological Science, Division of MR Research, Johns Hopkins University School of Medicine, Baltimore, MD</wicri:regionArea>
<placeName><region type="state">Maryland</region>
</placeName>
</affiliation>
</author>
<author><name sortKey="Liu, Xinyang" sort="Liu, Xinyang" uniqKey="Liu X" first="Xinyang" last="Liu">Xinyang Liu</name>
<affiliation wicri:level="2"><nlm:aff id="A6">Brigham and Women’s Hospital, Harvard Medical School, Boston, MA, USA</nlm:aff>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>Brigham and Women’s Hospital, Harvard Medical School, Boston, MA</wicri:regionArea>
<placeName><region type="state">Massachusetts</region>
</placeName>
</affiliation>
</author>
<author><name sortKey="Shanahan, Megan" sort="Shanahan, Megan" uniqKey="Shanahan M" first="Megan" last="Shanahan">Megan Shanahan</name>
<affiliation wicri:level="2"><nlm:aff id="A1">Department of Psychiatry and Behavioral Sciences, Johns Hopkins University School of Medicine, Baltimore, MD, USA</nlm:aff>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>Department of Psychiatry and Behavioral Sciences, Johns Hopkins University School of Medicine, Baltimore, MD</wicri:regionArea>
<placeName><region type="state">Maryland</region>
</placeName>
</affiliation>
</author>
<author><name sortKey="Wang, Xin" sort="Wang, Xin" uniqKey="Wang X" first="Xin" last="Wang">Xin Wang</name>
<affiliation wicri:level="2"><nlm:aff id="A4">The Russell H. Morgan Department of Radiology and Radiological Science, Division of MR Research, Johns Hopkins University School of Medicine, Baltimore, MD, USA</nlm:aff>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>The Russell H. Morgan Department of Radiology and Radiological Science, Division of MR Research, Johns Hopkins University School of Medicine, Baltimore, MD</wicri:regionArea>
<placeName><region type="state">Maryland</region>
</placeName>
</affiliation>
</author>
<author><name sortKey="Oishi, Kenichi" sort="Oishi, Kenichi" uniqKey="Oishi K" first="Kenichi" last="Oishi">Kenichi Oishi</name>
<affiliation wicri:level="2"><nlm:aff id="A4">The Russell H. Morgan Department of Radiology and Radiological Science, Division of MR Research, Johns Hopkins University School of Medicine, Baltimore, MD, USA</nlm:aff>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>The Russell H. Morgan Department of Radiology and Radiological Science, Division of MR Research, Johns Hopkins University School of Medicine, Baltimore, MD</wicri:regionArea>
<placeName><region type="state">Maryland</region>
</placeName>
</affiliation>
</author>
<author><name sortKey="Brandt, Jason" sort="Brandt, Jason" uniqKey="Brandt J" first="Jason" last="Brandt">Jason Brandt</name>
<affiliation wicri:level="2"><nlm:aff id="A1">Department of Psychiatry and Behavioral Sciences, Johns Hopkins University School of Medicine, Baltimore, MD, USA</nlm:aff>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>Department of Psychiatry and Behavioral Sciences, Johns Hopkins University School of Medicine, Baltimore, MD</wicri:regionArea>
<placeName><region type="state">Maryland</region>
</placeName>
</affiliation>
<affiliation wicri:level="2"><nlm:aff id="A2">Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, MD, USA</nlm:aff>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, MD</wicri:regionArea>
<placeName><region type="state">Maryland</region>
</placeName>
</affiliation>
</author>
<author><name sortKey="Bassett, Susan S" sort="Bassett, Susan S" uniqKey="Bassett S" first="Susan S." last="Bassett">Susan S. Bassett</name>
<affiliation wicri:level="2"><nlm:aff id="A1">Department of Psychiatry and Behavioral Sciences, Johns Hopkins University School of Medicine, Baltimore, MD, USA</nlm:aff>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>Department of Psychiatry and Behavioral Sciences, Johns Hopkins University School of Medicine, Baltimore, MD</wicri:regionArea>
<placeName><region type="state">Maryland</region>
</placeName>
</affiliation>
</author>
<author><name sortKey="Redgrave, Graham W" sort="Redgrave, Graham W" uniqKey="Redgrave G" first="Graham W." last="Redgrave">Graham W. Redgrave</name>
<affiliation wicri:level="2"><nlm:aff id="A1">Department of Psychiatry and Behavioral Sciences, Johns Hopkins University School of Medicine, Baltimore, MD, USA</nlm:aff>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>Department of Psychiatry and Behavioral Sciences, Johns Hopkins University School of Medicine, Baltimore, MD</wicri:regionArea>
<placeName><region type="state">Maryland</region>
</placeName>
</affiliation>
</author>
<author><name sortKey="Margolis, Russell L" sort="Margolis, Russell L" uniqKey="Margolis R" first="Russell L." last="Margolis">Russell L. Margolis</name>
<affiliation wicri:level="2"><nlm:aff id="A1">Department of Psychiatry and Behavioral Sciences, Johns Hopkins University School of Medicine, Baltimore, MD, USA</nlm:aff>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>Department of Psychiatry and Behavioral Sciences, Johns Hopkins University School of Medicine, Baltimore, MD</wicri:regionArea>
<placeName><region type="state">Maryland</region>
</placeName>
</affiliation>
<affiliation wicri:level="2"><nlm:aff id="A2">Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, MD, USA</nlm:aff>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, MD</wicri:regionArea>
<placeName><region type="state">Maryland</region>
</placeName>
</affiliation>
</author>
<author><name sortKey="Van Zijl, Peter C M" sort="Van Zijl, Peter C M" uniqKey="Van Zijl P" first="Peter C. M." last="Van Zijl">Peter C. M. Van Zijl</name>
<affiliation wicri:level="2"><nlm:aff id="A4">The Russell H. Morgan Department of Radiology and Radiological Science, Division of MR Research, Johns Hopkins University School of Medicine, Baltimore, MD, USA</nlm:aff>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>The Russell H. Morgan Department of Radiology and Radiological Science, Division of MR Research, Johns Hopkins University School of Medicine, Baltimore, MD</wicri:regionArea>
<placeName><region type="state">Maryland</region>
</placeName>
</affiliation>
<affiliation wicri:level="2"><nlm:aff id="A5">F.M. Kirby Research Center for Functional Brain Imaging, Kennedy Krieger Institute, Baltimore, MD, USA</nlm:aff>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>F.M. Kirby Research Center for Functional Brain Imaging, Kennedy Krieger Institute, Baltimore, MD</wicri:regionArea>
<placeName><region type="state">Maryland</region>
</placeName>
</affiliation>
</author>
<author><name sortKey="Barker, Peter B" sort="Barker, Peter B" uniqKey="Barker P" first="Peter B." last="Barker">Peter B. Barker</name>
<affiliation wicri:level="2"><nlm:aff id="A4">The Russell H. Morgan Department of Radiology and Radiological Science, Division of MR Research, Johns Hopkins University School of Medicine, Baltimore, MD, USA</nlm:aff>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>The Russell H. Morgan Department of Radiology and Radiological Science, Division of MR Research, Johns Hopkins University School of Medicine, Baltimore, MD</wicri:regionArea>
<placeName><region type="state">Maryland</region>
</placeName>
</affiliation>
<affiliation wicri:level="2"><nlm:aff id="A5">F.M. Kirby Research Center for Functional Brain Imaging, Kennedy Krieger Institute, Baltimore, MD, USA</nlm:aff>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>F.M. Kirby Research Center for Functional Brain Imaging, Kennedy Krieger Institute, Baltimore, MD</wicri:regionArea>
<placeName><region type="state">Maryland</region>
</placeName>
</affiliation>
</author>
<author><name sortKey="Ross, Christopher A" sort="Ross, Christopher A" uniqKey="Ross C" first="Christopher A." last="Ross">Christopher A. Ross</name>
<affiliation wicri:level="2"><nlm:aff id="A1">Department of Psychiatry and Behavioral Sciences, Johns Hopkins University School of Medicine, Baltimore, MD, USA</nlm:aff>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>Department of Psychiatry and Behavioral Sciences, Johns Hopkins University School of Medicine, Baltimore, MD</wicri:regionArea>
<placeName><region type="state">Maryland</region>
</placeName>
</affiliation>
<affiliation wicri:level="2"><nlm:aff id="A2">Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, MD, USA</nlm:aff>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, MD</wicri:regionArea>
<placeName><region type="state">Maryland</region>
</placeName>
</affiliation>
<affiliation wicri:level="2"><nlm:aff id="A3">Department of Neuroscience and Pharmacology, Johns Hopkins University School of Medicine, Baltimore, MD, USA</nlm:aff>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>Department of Neuroscience and Pharmacology, Johns Hopkins University School of Medicine, Baltimore, MD</wicri:regionArea>
<placeName><region type="state">Maryland</region>
</placeName>
</affiliation>
</author>
</analytic>
<series><title level="j">Movement Disorders</title>
<idno type="ISSN">0885-3185</idno>
<idno type="eISSN">1531-8257</idno>
<imprint><date when="2012">2012</date>
</imprint>
</series>
</biblStruct>
</sourceDesc>
</fileDesc>
<profileDesc><textClass></textClass>
</profileDesc>
</teiHeader>
<front><div type="abstract" xml:lang="en"><sec id="S1"><title>Background</title>
<p id="P1">Huntington’s Disease (HD) is a neurodegenerative disorder characterized by early cognitive decline, which progresses at later stages to dementia and severe movement disorder. HD is caused by a cytosine-adenine-guanine triplet-repeat expansion mutation in the <italic>Huntingtin</italic>
gene, allowing early diagnosis by genetic testing. This study aims to identify the relationship of N-acetylaspartate and other brain metabolites to cognitive function in HD-mutation carriers by using high field strength magnetic-resonance-spectroscopy at 7-Tesla.</p>
</sec>
<sec id="S2"><title>Methods</title>
<p id="P2">Twelve individuals with the HD-mutation in premanifest or early stage of disease versus twelve healthy controls underwent 1H magnetic-resonance-spectroscopy (7.2ml voxel in the posterior cingulate cortex) at 7-Tesla, and also T1-weighted structural magnetic-resonance-imaging. All participants received standardized tests of cognitive functioning including the Montreal Cognitive Assessment and standardized quantified neurological examination within an hour before scanning.</p>
</sec>
<sec id="S3"><title>Results</title>
<p id="P3">Individuals with the HD mutation had significantly lower posterior cingulate cortex N-acetylaspartate (−9.6%, p=0.02) and glutamate levels (−10.1%, p=0.02) than controls. By contrast, in this small group, measures of brain morphology including striatal and ventricle volumes did not differ significantly. Linear regression with Montreal Cognitive Assessment scores revealed significant correlations with N-acetylaspartate (r<sup>2</sup>
=0.50, p=0.01) and glutamate (r<sup>2</sup>
=0.64, p=0.002) in HD subjects.</p>
</sec>
<sec id="S4"><title>Conclusions</title>
<p id="P4">Our data suggest a relationship between reduced N-acetylaspartate and glutamate levels in the posterior cingulate cortex with cognitive decline in early stages of HD. N-acetylaspartate and glutamate magnetic-resonance-spectroscopy signals of the posterior cingulate cortex region may serve as potential biomarkers of disease progression or treatment outcome in HD and other neurodegenerative disorders with early cognitive dysfunction, when structural brain changes are still minor.</p>
</sec>
</div>
</front>
</TEI>
<pmc article-type="research-article"><pmc-comment>The publisher of this article does not allow downloading of the full text in XML form.</pmc-comment>
<pmc-dir>properties manuscript</pmc-dir>
<front><journal-meta><journal-id journal-id-type="nlm-journal-id">8610688</journal-id>
<journal-id journal-id-type="pubmed-jr-id">5937</journal-id>
<journal-id journal-id-type="nlm-ta">Mov Disord</journal-id>
<journal-id journal-id-type="iso-abbrev">Mov. Disord.</journal-id>
<journal-title-group><journal-title>Movement Disorders</journal-title>
</journal-title-group>
<issn pub-type="ppub">0885-3185</issn>
<issn pub-type="epub">1531-8257</issn>
</journal-meta>
<article-meta><article-id pub-id-type="pmid">22649062</article-id>
<article-id pub-id-type="pmc">3383395</article-id>
<article-id pub-id-type="doi">10.1002/mds.25010</article-id>
<article-id pub-id-type="manuscript">NIHMS368111</article-id>
<article-categories><subj-group subj-group-type="heading"><subject>Article</subject>
</subj-group>
</article-categories>
<title-group><article-title>Brain metabolite alterations and cognitive dysfunction in early Huntington’s Disease</article-title>
</title-group>
<contrib-group><contrib contrib-type="author"><name><surname>Unschuld</surname>
<given-names>Paul G.</given-names>
</name>
<xref ref-type="aff" rid="A1">1</xref>
</contrib>
<contrib contrib-type="author"><name><surname>Edden</surname>
<given-names>Richard A. E.</given-names>
</name>
<xref ref-type="aff" rid="A4">4</xref>
<xref ref-type="aff" rid="A5">5</xref>
</contrib>
<contrib contrib-type="author"><name><surname>Carass</surname>
<given-names>Aaron</given-names>
</name>
<xref ref-type="aff" rid="A4">4</xref>
</contrib>
<contrib contrib-type="author"><name><surname>Liu</surname>
<given-names>Xinyang</given-names>
</name>
<xref ref-type="aff" rid="A6">6</xref>
</contrib>
<contrib contrib-type="author"><name><surname>Shanahan</surname>
<given-names>Megan</given-names>
</name>
<xref ref-type="aff" rid="A1">1</xref>
</contrib>
<contrib contrib-type="author"><name><surname>Wang</surname>
<given-names>Xin</given-names>
</name>
<xref ref-type="aff" rid="A4">4</xref>
</contrib>
<contrib contrib-type="author"><name><surname>Oishi</surname>
<given-names>Kenichi</given-names>
</name>
<xref ref-type="aff" rid="A4">4</xref>
</contrib>
<contrib contrib-type="author"><name><surname>Brandt</surname>
<given-names>Jason</given-names>
</name>
<xref ref-type="aff" rid="A1">1</xref>
<xref ref-type="aff" rid="A2">2</xref>
</contrib>
<contrib contrib-type="author"><name><surname>Bassett</surname>
<given-names>Susan S.</given-names>
</name>
<xref ref-type="aff" rid="A1">1</xref>
</contrib>
<contrib contrib-type="author"><name><surname>Redgrave</surname>
<given-names>Graham W.</given-names>
</name>
<xref ref-type="aff" rid="A1">1</xref>
</contrib>
<contrib contrib-type="author"><name><surname>Margolis</surname>
<given-names>Russell L.</given-names>
</name>
<xref ref-type="aff" rid="A1">1</xref>
<xref ref-type="aff" rid="A2">2</xref>
</contrib>
<contrib contrib-type="author"><name><surname>van Zijl</surname>
<given-names>Peter C. M.</given-names>
</name>
<xref ref-type="aff" rid="A4">4</xref>
<xref ref-type="aff" rid="A5">5</xref>
</contrib>
<contrib contrib-type="author"><name><surname>Barker</surname>
<given-names>Peter B.</given-names>
</name>
<xref ref-type="aff" rid="A4">4</xref>
<xref ref-type="aff" rid="A5">5</xref>
</contrib>
<contrib contrib-type="author"><name><surname>Ross</surname>
<given-names>Christopher A.</given-names>
</name>
<xref ref-type="aff" rid="A1">1</xref>
<xref ref-type="aff" rid="A2">2</xref>
<xref ref-type="aff" rid="A3">3</xref>
</contrib>
</contrib-group>
<aff id="A1"><label>1</label>
Department of Psychiatry and Behavioral Sciences, Johns Hopkins University School of Medicine, Baltimore, MD, USA</aff>
<aff id="A2"><label>2</label>
Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, MD, USA</aff>
<aff id="A3"><label>3</label>
Department of Neuroscience and Pharmacology, Johns Hopkins University School of Medicine, Baltimore, MD, USA</aff>
<aff id="A4"><label>4</label>
The Russell H. Morgan Department of Radiology and Radiological Science, Division of MR Research, Johns Hopkins University School of Medicine, Baltimore, MD, USA</aff>
<aff id="A5"><label>5</label>
F.M. Kirby Research Center for Functional Brain Imaging, Kennedy Krieger Institute, Baltimore, MD, USA</aff>
<aff id="A6"><label>6</label>
Brigham and Women’s Hospital, Harvard Medical School, Boston, MA, USA</aff>
<author-notes><corresp id="FN1">Corresponding author: Paul G. Unschuld, MD, Department of Psychiatry and Behavioral Sciences, Division of Psychiatric Neuroimaging, The Johns Hopkins University School of Medicine, 600 N. Wolfe Street/Phipps 300, Baltimore, Maryland 21287, USA, <email>unschuld@jhmi.edu</email>
, Tel.: +001-410-502-6945</corresp>
</author-notes>
<pub-date pub-type="nihms-submitted"><day>12</day>
<month>4</month>
<year>2012</year>
</pub-date>
<pub-date pub-type="epub"><day>30</day>
<month>5</month>
<year>2012</year>
</pub-date>
<pub-date pub-type="ppub"><month>6</month>
<year>2012</year>
</pub-date>
<pub-date pub-type="pmc-release"><day>01</day>
<month>6</month>
<year>2013</year>
</pub-date>
<volume>27</volume>
<issue>7</issue>
<fpage>895</fpage>
<lpage>902</lpage>
<abstract><sec id="S1"><title>Background</title>
<p id="P1">Huntington’s Disease (HD) is a neurodegenerative disorder characterized by early cognitive decline, which progresses at later stages to dementia and severe movement disorder. HD is caused by a cytosine-adenine-guanine triplet-repeat expansion mutation in the <italic>Huntingtin</italic>
gene, allowing early diagnosis by genetic testing. This study aims to identify the relationship of N-acetylaspartate and other brain metabolites to cognitive function in HD-mutation carriers by using high field strength magnetic-resonance-spectroscopy at 7-Tesla.</p>
</sec>
<sec id="S2"><title>Methods</title>
<p id="P2">Twelve individuals with the HD-mutation in premanifest or early stage of disease versus twelve healthy controls underwent 1H magnetic-resonance-spectroscopy (7.2ml voxel in the posterior cingulate cortex) at 7-Tesla, and also T1-weighted structural magnetic-resonance-imaging. All participants received standardized tests of cognitive functioning including the Montreal Cognitive Assessment and standardized quantified neurological examination within an hour before scanning.</p>
</sec>
<sec id="S3"><title>Results</title>
<p id="P3">Individuals with the HD mutation had significantly lower posterior cingulate cortex N-acetylaspartate (−9.6%, p=0.02) and glutamate levels (−10.1%, p=0.02) than controls. By contrast, in this small group, measures of brain morphology including striatal and ventricle volumes did not differ significantly. Linear regression with Montreal Cognitive Assessment scores revealed significant correlations with N-acetylaspartate (r<sup>2</sup>
=0.50, p=0.01) and glutamate (r<sup>2</sup>
=0.64, p=0.002) in HD subjects.</p>
</sec>
<sec id="S4"><title>Conclusions</title>
<p id="P4">Our data suggest a relationship between reduced N-acetylaspartate and glutamate levels in the posterior cingulate cortex with cognitive decline in early stages of HD. N-acetylaspartate and glutamate magnetic-resonance-spectroscopy signals of the posterior cingulate cortex region may serve as potential biomarkers of disease progression or treatment outcome in HD and other neurodegenerative disorders with early cognitive dysfunction, when structural brain changes are still minor.</p>
</sec>
</abstract>
<kwd-group><kwd>MRS</kwd>
<kwd>NAA</kwd>
<kwd>glutamate</kwd>
<kwd>cognition</kwd>
<kwd>neurodegeneration</kwd>
<kwd>biomarker</kwd>
</kwd-group>
</article-meta>
</front>
</pmc>
<affiliations><list><country><li>États-Unis</li>
</country>
<region><li>Maryland</li>
<li>Massachusetts</li>
</region>
</list>
<tree><country name="États-Unis"><region name="Maryland"><name sortKey="Unschuld, Paul G" sort="Unschuld, Paul G" uniqKey="Unschuld P" first="Paul G." last="Unschuld">Paul G. Unschuld</name>
</region>
<name sortKey="Barker, Peter B" sort="Barker, Peter B" uniqKey="Barker P" first="Peter B." last="Barker">Peter B. Barker</name>
<name sortKey="Barker, Peter B" sort="Barker, Peter B" uniqKey="Barker P" first="Peter B." last="Barker">Peter B. Barker</name>
<name sortKey="Bassett, Susan S" sort="Bassett, Susan S" uniqKey="Bassett S" first="Susan S." last="Bassett">Susan S. Bassett</name>
<name sortKey="Brandt, Jason" sort="Brandt, Jason" uniqKey="Brandt J" first="Jason" last="Brandt">Jason Brandt</name>
<name sortKey="Brandt, Jason" sort="Brandt, Jason" uniqKey="Brandt J" first="Jason" last="Brandt">Jason Brandt</name>
<name sortKey="Carass, Aaron" sort="Carass, Aaron" uniqKey="Carass A" first="Aaron" last="Carass">Aaron Carass</name>
<name sortKey="Edden, Richard A E" sort="Edden, Richard A E" uniqKey="Edden R" first="Richard A. E." last="Edden">Richard A. E. Edden</name>
<name sortKey="Edden, Richard A E" sort="Edden, Richard A E" uniqKey="Edden R" first="Richard A. E." last="Edden">Richard A. E. Edden</name>
<name sortKey="Liu, Xinyang" sort="Liu, Xinyang" uniqKey="Liu X" first="Xinyang" last="Liu">Xinyang Liu</name>
<name sortKey="Margolis, Russell L" sort="Margolis, Russell L" uniqKey="Margolis R" first="Russell L." last="Margolis">Russell L. Margolis</name>
<name sortKey="Margolis, Russell L" sort="Margolis, Russell L" uniqKey="Margolis R" first="Russell L." last="Margolis">Russell L. Margolis</name>
<name sortKey="Oishi, Kenichi" sort="Oishi, Kenichi" uniqKey="Oishi K" first="Kenichi" last="Oishi">Kenichi Oishi</name>
<name sortKey="Redgrave, Graham W" sort="Redgrave, Graham W" uniqKey="Redgrave G" first="Graham W." last="Redgrave">Graham W. Redgrave</name>
<name sortKey="Ross, Christopher A" sort="Ross, Christopher A" uniqKey="Ross C" first="Christopher A." last="Ross">Christopher A. Ross</name>
<name sortKey="Ross, Christopher A" sort="Ross, Christopher A" uniqKey="Ross C" first="Christopher A." last="Ross">Christopher A. Ross</name>
<name sortKey="Ross, Christopher A" sort="Ross, Christopher A" uniqKey="Ross C" first="Christopher A." last="Ross">Christopher A. Ross</name>
<name sortKey="Shanahan, Megan" sort="Shanahan, Megan" uniqKey="Shanahan M" first="Megan" last="Shanahan">Megan Shanahan</name>
<name sortKey="Van Zijl, Peter C M" sort="Van Zijl, Peter C M" uniqKey="Van Zijl P" first="Peter C. M." last="Van Zijl">Peter C. M. Van Zijl</name>
<name sortKey="Van Zijl, Peter C M" sort="Van Zijl, Peter C M" uniqKey="Van Zijl P" first="Peter C. M." last="Van Zijl">Peter C. M. Van Zijl</name>
<name sortKey="Wang, Xin" sort="Wang, Xin" uniqKey="Wang X" first="Xin" last="Wang">Xin Wang</name>
</country>
</tree>
</affiliations>
</record>
Pour manipuler ce document sous Unix (Dilib)
EXPLOR_STEP=$WICRI_ROOT/Wicri/Santé/explor/MovDisordV3/Data/Pmc/Checkpoint
HfdSelect -h $EXPLOR_STEP/biblio.hfd -nk 000206 | SxmlIndent | more
Ou
HfdSelect -h $EXPLOR_AREA/Data/Pmc/Checkpoint/biblio.hfd -nk 000206 | SxmlIndent | more
Pour mettre un lien sur cette page dans le réseau Wicri
{{Explor lien |wiki= Wicri/Santé |area= MovDisordV3 |flux= Pmc |étape= Checkpoint |type= RBID |clé= PMC:3383395 |texte= Brain metabolite alterations and cognitive dysfunction in early Huntington’s Disease }}
Pour générer des pages wiki
HfdIndexSelect -h $EXPLOR_AREA/Data/Pmc/Checkpoint/RBID.i -Sk "pubmed:22649062" \ | HfdSelect -Kh $EXPLOR_AREA/Data/Pmc/Checkpoint/biblio.hfd \ | NlmPubMed2Wicri -a MovDisordV3
![]() | This area was generated with Dilib version V0.6.23. | ![]() |