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<meta name="DC.Title" content="MR Spectroscopy Findings in Early Stages of Motor Neuron Disease" />
<meta name="DC.Identifier" content="10.3174/ajnr.A2217" />
<meta name="DC.Date" content="2010-11-01" />
<meta name="DC.Publisher" content="American Journal of Neuroradiology" />
<meta name="DC.Rights" content="Copyright © American Society of Neuroradiology" />
<meta name="DC.AccessRights" content="restricted" />
<meta name="DC.Description" content="BACKGROUND AND PURPOSE: Upper motor neuron degeneration varies in different phenotypes of MND. We used single-voxel MR spectroscopy of the primary motor cortex to detect corticomotoneuron degeneration and glial hyperactivity in different phenotypes of MND with a relatively short disease duration, contributing to further delineation of the phenotypes.

MATERIALS AND METHODS: We prospectively included patients with ALS-B, ALS-L, and PMA and compared their data with those of patients with PLS and healthy controls. Each cohort consisted of 12 individuals. Disease duration was &lt;1 year in ALS and PMA, but longer in PLS by definition. Follow-up examination was at 6 months. We measured ALSFRS-R, finger- and foot-tapping speed, and levels of the following: 1) NAAx, 2) mIns, and 3) Glx in the primary motor cortex.

RESULTS: At baseline, we found significantly decreased NAAx levels and increased mIns levels in PLS. Levels of NAAx and mIns in patients with ALS-L and ALS-B were not significantly different from those in controls, but NAAx levels were significantly lower compared with those in PMA. At follow-up, only in PMA was a decrease of NAAx demonstrated. Glx levels varied widely in all groups. Levels of NAAx and mIns correlated well with clinical variables.

CONCLUSIONS: Metabolite changes suggest neuronal dysfunction and active glial involvement in PLS. The corticomotoneuron is affected in early ALS-B and ALS-L, but at a later stage also in PMA. MR spectroscopy data are useful to obtain insight into the disease process at the level of the upper motor neuron in various phenotypes of MND.

ALS
:   amyotrophic lateral sclerosis
ALS-B
:   bulbar onset ALS
ALSFRS-R
:   Revised ALS Functional Rating Scale
ALS-L
:   limb-onset ALS
CI
:   confidence interval
Glx
:   glutamate + glutamine
L
:   left
mIns
:   myo-inositol
MND
:   motor neuron disease
MRS
:   MR spectroscopy
NA
:   not applicable
NAA
:   N -acetylaspartate
NAAx
:   N -acetylaspartate + N -acetyl aspartylglutamate
NS
:   not significant
PLS
:   primary lateral sclerosis
PMA
:   progressive muscular atrophy
R
:   right
ROC
:   receiver operating characteristic analysis
VC
:   vital capacity" />
<meta name="DC.Contributor" content="M.M. van der Graaff" />
<meta name="DC.Contributor" content="C. Lavini" />
<meta name="DC.Contributor" content="E.M. Akkerman" />
<meta name="DC.Contributor" content="Ch.B. Majoie" />
<meta name="DC.Contributor" content="A.J. Nederveen" />
<meta name="DC.Contributor" content="A.H. Zwinderman" />
<meta name="DC.Contributor" content="F. Brugman" />
<meta name="DC.Contributor" content="L.H. van den Berg" />
<meta name="DC.Contributor" content="J.M.B.V. de Jong" />
<meta name="DC.Contributor" content="M. de Visser" />
<meta name="article:published_time" content="2010-11-01" />
<meta name="article:section" content="Functional" />
<meta name="citation_title" content="MR Spectroscopy Findings in Early Stages of Motor Neuron Disease" />
<meta name="citation_abstract" lang="en" content="&lt;h3&gt;BACKGROUND AND PURPOSE:&lt;/h3&gt;
&lt;p&gt;Upper motor neuron degeneration varies in different phenotypes of MND. We used single-voxel MR spectroscopy of the primary motor cortex to detect corticomotoneuron degeneration and glial hyperactivity in different phenotypes of MND with a relatively short disease duration, contributing to further delineation of the phenotypes.&lt;/p&gt;&lt;h3&gt;MATERIALS AND METHODS:&lt;/h3&gt;
&lt;p&gt;We prospectively included patients with ALS-B, ALS-L, and PMA and compared their data with those of patients with PLS and healthy controls. Each cohort consisted of 12 individuals. Disease duration was &amp;lt;1 year in ALS and PMA, but longer in PLS by definition. Follow-up examination was at 6 months. We measured ALSFRS-R, finger- and foot-tapping speed, and levels of the following: 1) NAAx, 2) mIns, and 3) Glx in the primary motor cortex.&lt;/p&gt;&lt;h3&gt;RESULTS:&lt;/h3&gt;
&lt;p&gt;At baseline, we found significantly decreased NAAx levels and increased mIns levels in PLS. Levels of NAAx and mIns in patients with ALS-L and ALS-B were not significantly different from those in controls, but NAAx levels were significantly lower compared with those in PMA. At follow-up, only in PMA was a decrease of NAAx demonstrated. Glx levels varied widely in all groups. Levels of NAAx and mIns correlated well with clinical variables.&lt;/p&gt;&lt;h3&gt;CONCLUSIONS:&lt;/h3&gt;
&lt;p&gt;Metabolite changes suggest neuronal dysfunction and active glial involvement in PLS. The corticomotoneuron is affected in early ALS-B and ALS-L, but at a later stage also in PMA. MR spectroscopy data are useful to obtain insight into the disease process at the level of the upper motor neuron in various phenotypes of MND.&lt;/p&gt;" />
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<meta name="twitter:title" content="MR Spectroscopy Findings in Early Stages of Motor Neuron Disease" />
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<meta name="twitter:image" content="http://ajnr-stage2.stage.highwire.org/sites/default/files/highwire/ajnr/31/10.cover.gif" />
<meta name="twitter:description" content="BACKGROUND AND PURPOSE: Upper motor neuron degeneration varies in different phenotypes of MND. We used single-voxel MR spectroscopy of the primary motor cortex to detect corticomotoneuron degeneration and glial hyperactivity in different phenotypes of MND with a relatively short disease duration, contributing to further delineation of the phenotypes.

MATERIALS AND METHODS: We prospectively included patients with ALS-B, ALS-L, and PMA and compared their data with those of patients with PLS and healthy controls. Each cohort consisted of 12 individuals. Disease duration was &lt;1 year in ALS and PMA, but longer in PLS by definition. Follow-up examination was at 6 months. We measured ALSFRS-R, finger- and foot-tapping speed, and levels of the following: 1) NAAx, 2) mIns, and 3) Glx in the primary motor cortex.

RESULTS: At baseline, we found significantly decreased NAAx levels and increased mIns levels in PLS. Levels of NAAx and mIns in patients with ALS-L and ALS-B were not significantly different from those in controls, but NAAx levels were significantly lower compared with those in PMA. At follow-up, only in PMA was a decrease of NAAx demonstrated. Glx levels varied widely in all groups. Levels of NAAx and mIns correlated well with clinical variables.

CONCLUSIONS: Metabolite changes suggest neuronal dysfunction and active glial involvement in PLS. The corticomotoneuron is affected in early ALS-B and ALS-L, but at a later stage also in PMA. MR spectroscopy data are useful to obtain insight into the disease process at the level of the upper motor neuron in various phenotypes of MND.

ALS
:   amyotrophic lateral sclerosis
ALS-B
:   bulbar onset ALS
ALSFRS-R
:   Revised ALS Functional Rating Scale
ALS-L
:   limb-onset ALS
CI
:   confidence interval
Glx
:   glutamate + glutamine
L
:   left
mIns
:   myo-inositol
MND
:   motor neuron disease
MRS
:   MR spectroscopy
NA
:   not applicable
NAA
:   N -acetylaspartate
NAAx
:   N -acetylaspartate + N -acetyl aspartylglutamate
NS
:   not significant
PLS
:   primary lateral sclerosis
PMA
:   progressive muscular atrophy
R
:   right
ROC
:   receiver operating characteristic analysis
VC
:   vital capacity" />
<meta name="og-title" property="og:title" content="MR Spectroscopy Findings in Early Stages of Motor Neuron Disease" />
<meta name="og-url" property="og:url" content="http://ajnr-stage2.stage.highwire.org/content/31/10/1799" />
<meta name="og-site-name" property="og:site_name" content="American Journal of Neuroradiology" />
<meta name="og-description" property="og:description" content="BACKGROUND AND PURPOSE: Upper motor neuron degeneration varies in different phenotypes of MND. We used single-voxel MR spectroscopy of the primary motor cortex to detect corticomotoneuron degeneration and glial hyperactivity in different phenotypes of MND with a relatively short disease duration, contributing to further delineation of the phenotypes.

MATERIALS AND METHODS: We prospectively included patients with ALS-B, ALS-L, and PMA and compared their data with those of patients with PLS and healthy controls. Each cohort consisted of 12 individuals. Disease duration was &lt;1 year in ALS and PMA, but longer in PLS by definition. Follow-up examination was at 6 months. We measured ALSFRS-R, finger- and foot-tapping speed, and levels of the following: 1) NAAx, 2) mIns, and 3) Glx in the primary motor cortex.

RESULTS: At baseline, we found significantly decreased NAAx levels and increased mIns levels in PLS. Levels of NAAx and mIns in patients with ALS-L and ALS-B were not significantly different from those in controls, but NAAx levels were significantly lower compared with those in PMA. At follow-up, only in PMA was a decrease of NAAx demonstrated. Glx levels varied widely in all groups. Levels of NAAx and mIns correlated well with clinical variables.

CONCLUSIONS: Metabolite changes suggest neuronal dysfunction and active glial involvement in PLS. The corticomotoneuron is affected in early ALS-B and ALS-L, but at a later stage also in PMA. MR spectroscopy data are useful to obtain insight into the disease process at the level of the upper motor neuron in various phenotypes of MND.

ALS
:   amyotrophic lateral sclerosis
ALS-B
:   bulbar onset ALS
ALSFRS-R
:   Revised ALS Functional Rating Scale
ALS-L
:   limb-onset ALS
CI
:   confidence interval
Glx
:   glutamate + glutamine
L
:   left
mIns
:   myo-inositol
MND
:   motor neuron disease
MRS
:   MR spectroscopy
NA
:   not applicable
NAA
:   N -acetylaspartate
NAAx
:   N -acetylaspartate + N -acetyl aspartylglutamate
NS
:   not significant
PLS
:   primary lateral sclerosis
PMA
:   progressive muscular atrophy
R
:   right
ROC
:   receiver operating characteristic analysis
VC
:   vital capacity" />
<meta name="og-type" property="og:type" content="article" />
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      <h1 class="highwire-cite-title" id="page-title">MR Spectroscopy Findings in Early Stages of Motor Neuron Disease</h1>  
    	<div class="highwire-cite-authors"><span  class="highwire-citation-authors"><span class="highwire-citation-author first" data-delta="0">M.M. van der Graaff</span>, <span class="highwire-citation-author" data-delta="1">C. Lavini</span>, <span class="highwire-citation-author" data-delta="2">E.M. Akkerman</span>, <span class="highwire-citation-author" data-delta="3">Ch.B. Majoie</span>, <span class="highwire-citation-author" data-delta="4">A.J. Nederveen</span>, <span class="highwire-citation-author" data-delta="5">A.H. Zwinderman</span>, <span class="highwire-citation-author" data-delta="6">F. Brugman</span>, <span class="highwire-citation-author" data-delta="7">L.H. van den Berg</span>, <span class="highwire-citation-author" data-delta="8">J.M.B.V. de Jong</span> and <span class="highwire-citation-author" data-delta="9">M. de Visser</span></span></div>
  
    	<div class="highwire-cite-metadata"><span  class="highwire-cite-metadata-journal highwire-cite-metadata">American Journal of Neuroradiology </span><span  class="highwire-cite-metadata-date highwire-cite-metadata">November 2010,  </span><span  class="highwire-cite-metadata-volume highwire-cite-metadata">31 </span><span  class="highwire-cite-metadata-issue highwire-cite-metadata">(10) </span><span  class="highwire-cite-metadata-pages highwire-cite-metadata">1799-1806; </span><span  class="highwire-cite-metadata-doi highwire-cite-metadata">DOI: https://doi.org/10.3174/ajnr.A2217 </span></div>
  
  
  
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<div id="hw-article-author-popups-top-node-9726--81945299382" style="display: none;"><div class="author-tooltip-0"><div class="author-tooltip-name">M.M. van der Graaff </div><ul class="author-tooltip-find-more"><li class="author-tooltip-gs-link first"><a href="/lookup/google-scholar?link_type=googlescholar&amp;gs_type=author&amp;author%5B0%5D=M.M.%2Bvan%2Bder%2BGraaff%2B" target="_blank" class="" data-icon-position="" data-hide-link-title="0">Find this author on Google Scholar</a></li><li class="author-tooltip-pubmed-link"><a href="/lookup/external-ref?access_num=van%20der%20Graaff%20M&amp;link_type=AUTHORSEARCH" target="_blank" class="" data-icon-position="" data-hide-link-title="0">Find this author on PubMed</a></li><li class="author-site-search-link last"><a href="/search/author1%3AM.M.%2Bvan%2Bder%2BGraaff%2B" rel="nofollow" class="" data-icon-position="" data-hide-link-title="0">Search for this author on this site</a></li></ul></div><div class="author-tooltip-1"><div class="author-tooltip-name">C. Lavini </div><ul class="author-tooltip-find-more"><li class="author-tooltip-gs-link first"><a href="/lookup/google-scholar?link_type=googlescholar&amp;gs_type=author&amp;author%5B0%5D=C.%2BLavini%2B" target="_blank" class="" data-icon-position="" data-hide-link-title="0">Find this author on Google Scholar</a></li><li class="author-tooltip-pubmed-link"><a href="/lookup/external-ref?access_num=Lavini%20C&amp;link_type=AUTHORSEARCH" target="_blank" class="" data-icon-position="" data-hide-link-title="0">Find this author on PubMed</a></li><li class="author-site-search-link last"><a href="/search/author1%3AC.%2BLavini%2B" rel="nofollow" class="" data-icon-position="" data-hide-link-title="0">Search for this author on this site</a></li></ul></div><div class="author-tooltip-2"><div class="author-tooltip-name">E.M. Akkerman </div><ul class="author-tooltip-find-more"><li class="author-tooltip-gs-link first"><a href="/lookup/google-scholar?link_type=googlescholar&amp;gs_type=author&amp;author%5B0%5D=E.M.%2BAkkerman%2B" target="_blank" class="" data-icon-position="" data-hide-link-title="0">Find this author on Google Scholar</a></li><li class="author-tooltip-pubmed-link"><a href="/lookup/external-ref?access_num=Akkerman%20E&amp;link_type=AUTHORSEARCH" target="_blank" class="" data-icon-position="" data-hide-link-title="0">Find this author on PubMed</a></li><li class="author-site-search-link last"><a href="/search/author1%3AE.M.%2BAkkerman%2B" rel="nofollow" class="" data-icon-position="" data-hide-link-title="0">Search for this author on this site</a></li></ul></div><div class="author-tooltip-3"><div class="author-tooltip-name">Ch.B. Majoie </div><ul class="author-tooltip-find-more"><li class="author-tooltip-gs-link first"><a href="/lookup/google-scholar?link_type=googlescholar&amp;gs_type=author&amp;author%5B0%5D=Ch.B.%2BMajoie%2B" target="_blank" class="" data-icon-position="" data-hide-link-title="0">Find this author on Google Scholar</a></li><li class="author-tooltip-pubmed-link"><a href="/lookup/external-ref?access_num=Majoie%20C&amp;link_type=AUTHORSEARCH" target="_blank" class="" data-icon-position="" data-hide-link-title="0">Find this author on PubMed</a></li><li class="author-site-search-link last"><a href="/search/author1%3ACh.B.%2BMajoie%2B" rel="nofollow" class="" data-icon-position="" data-hide-link-title="0">Search for this author on this site</a></li></ul></div><div class="author-tooltip-4"><div class="author-tooltip-name">A.J. 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    	<div class="highwire-cite-authors"><span  class="highwire-citation-authors"><span class="highwire-citation-author first" data-delta="0"><span class="nlm-given-names">M.M.</span> <span class="nlm-surname">van der Graaff</span></span>, <span class="highwire-citation-author" data-delta="1"><span class="nlm-given-names">C.</span> <span class="nlm-surname">Lavini</span></span>, <span class="highwire-citation-author" data-delta="2"><span class="nlm-given-names">E.M.</span> <span class="nlm-surname">Akkerman</span></span>, <span class="highwire-citation-author" data-delta="3"><span class="nlm-given-names">Ch.B.</span> <span class="nlm-surname">Majoie</span></span>, <span class="highwire-citation-author" data-delta="4"><span class="nlm-given-names">A.J.</span> <span class="nlm-surname">Nederveen</span></span>, <span class="highwire-citation-author" data-delta="5"><span class="nlm-given-names">A.H.</span> <span class="nlm-surname">Zwinderman</span></span>, <span class="highwire-citation-author" data-delta="6"><span class="nlm-given-names">F.</span> <span class="nlm-surname">Brugman</span></span>, <span class="highwire-citation-author" data-delta="7"><span class="nlm-given-names">L.H.</span> <span class="nlm-surname">van den Berg</span></span>, <span class="highwire-citation-author" data-delta="8"><span class="nlm-given-names">J.M.B.V.</span> <span class="nlm-surname">de Jong</span></span>, <span class="highwire-citation-author" data-delta="9"><span class="nlm-given-names">M.</span> <span class="nlm-surname">de Visser</span></span></span></div>
  
    	<div class="highwire-cite-metadata"><span  class="highwire-cite-metadata-journal highwire-cite-metadata">American Journal of Neuroradiology </span><span  class="highwire-cite-metadata-date highwire-cite-metadata">Nov 2010, </span><span  class="highwire-cite-metadata-volume highwire-cite-metadata">31 </span><span  class="highwire-cite-metadata-issue highwire-cite-metadata">(10) </span><span  class="highwire-cite-metadata-pages highwire-cite-metadata">1799-1806; </span><span  class="highwire-cite-metadata-doi highwire-cite-metadata"><span class="label">DOI:</span> 10.3174/ajnr.A2217 </span></div>
  
  
  
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      <div class="highwire-cite-title">MR Spectroscopy Findings in Early Stages of Motor Neuron Disease</div>  
    	<div class="highwire-cite-authors"><span  class="highwire-citation-authors"><span class="highwire-citation-author first" data-delta="0"><span class="nlm-given-names">M.M.</span> <span class="nlm-surname">van der Graaff</span></span>, <span class="highwire-citation-author" data-delta="1"><span class="nlm-given-names">C.</span> <span class="nlm-surname">Lavini</span></span>, <span class="highwire-citation-author" data-delta="2"><span class="nlm-given-names">E.M.</span> <span class="nlm-surname">Akkerman</span></span>, <span class="highwire-citation-author" data-delta="3"><span class="nlm-given-names">Ch.B.</span> <span class="nlm-surname">Majoie</span></span>, <span class="highwire-citation-author" data-delta="4"><span class="nlm-given-names">A.J.</span> <span class="nlm-surname">Nederveen</span></span>, <span class="highwire-citation-author" data-delta="5"><span class="nlm-given-names">A.H.</span> <span class="nlm-surname">Zwinderman</span></span>, <span class="highwire-citation-author" data-delta="6"><span class="nlm-given-names">F.</span> <span class="nlm-surname">Brugman</span></span>, <span class="highwire-citation-author" data-delta="7"><span class="nlm-given-names">L.H.</span> <span class="nlm-surname">van den Berg</span></span>, <span class="highwire-citation-author" data-delta="8"><span class="nlm-given-names">J.M.B.V.</span> <span class="nlm-surname">de Jong</span></span>, <span class="highwire-citation-author" data-delta="9"><span class="nlm-given-names">M.</span> <span class="nlm-surname">de Visser</span></span></span></div>
  
    	<div class="highwire-cite-metadata"><span  class="highwire-cite-metadata-journal highwire-cite-metadata">American Journal of Neuroradiology </span><span  class="highwire-cite-metadata-date highwire-cite-metadata">Nov 2010, </span><span  class="highwire-cite-metadata-volume highwire-cite-metadata">31 </span><span  class="highwire-cite-metadata-issue highwire-cite-metadata">(10) </span><span  class="highwire-cite-metadata-pages highwire-cite-metadata">1799-1806; </span><span  class="highwire-cite-metadata-doi highwire-cite-metadata"><span class="label">DOI:</span> 10.3174/ajnr.A2217 </span></div>
  
  
  
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