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<meta name="DC.Title" content="Alexander Disease: Diagnosis with MR Imaging" />
<meta name="DC.Date" content="2001-03-01" />
<meta name="DC.Publisher" content="American Journal of Neuroradiology" />
<meta name="DC.Rights" content="Copyright © American Society of Neuroradiology" />
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<meta name="DC.Description" content="BACKGROUND AND PURPOSE: To date, the demonstration of Rosenthal fibers on brain biopsy or autopsy specimens is considered a prerequisite for a definitive diagnosis of Alexander disease. We initiated a multiinstitutional survey of MR abnormalities in both presumed and confirmed cases of Alexander disease to assess the possibility of an MR-based diagnosis.

METHODS: MR imaging studies in three patients with an autopsy-based diagnosis of Alexander disease were analyzed to define MR criteria for the diagnosis. These criteria were then applied to 217 children with leukoencephalopathy of unknown origin.

RESULTS: Five MR imaging criteria were defined: extensive cerebral white matter changes with frontal predominance, a periventricular rim with high signal on T1-weighted images and low signal on T2-weighted images, abnormalities of basal ganglia and thalami, brain stem abnormalities, and contrast enhancement of particular gray and white matter structures. Four of the five criteria had to be met for an MR imaging-based diagnosis. In a retrospective analysis of the MR studies of the 217 patients, 19 were found who fulfilled these criteria. No other essentially new MR abnormalities were found in these patients. In four of the 19 patients, subsequent histologic confirmation was obtained. The clinical symptomatology was the same in the patients with and without histologic confirmation and correlated well with the MR abnormalities. MR abnormalities were in close agreement with the known histopathologic findings of Alexander disease.

CONCLUSION: The defined criteria are sufficient for an in vivo MR imaging diagnosis of Alexander disease; only in atypical cases is a brain biopsy still necessary for a definitive diagnosis." />
<meta name="DC.Contributor" content="Marjo S. van der Knaap" />
<meta name="DC.Contributor" content="Sakkubai Naidu" />
<meta name="DC.Contributor" content="Steven N. Breiter" />
<meta name="DC.Contributor" content="Susan Blaser" />
<meta name="DC.Contributor" content="Hans Stroink" />
<meta name="DC.Contributor" content="Stephan Springer" />
<meta name="DC.Contributor" content="Jacobus C. Begeer" />
<meta name="DC.Contributor" content="Rudy van Coster" />
<meta name="DC.Contributor" content="Peter G. Barth" />
<meta name="DC.Contributor" content="Neil H. Thomas" />
<meta name="DC.Contributor" content="Jacob Valk" />
<meta name="DC.Contributor" content="James M. Powers" />
<meta name="article:published_time" content="2001-03-01" />
<meta name="article:section" content="PEDIATRICS" />
<meta name="citation_title" content="Alexander Disease: Diagnosis with MR Imaging" />
<meta name="citation_abstract" lang="en" content="&lt;p&gt;&lt;i&gt;BACKGROUND AND PURPOSE:&lt;/i&gt; To date, the demonstration of Rosenthal fibers on brain biopsy or autopsy specimens is considered a prerequisite for a definitive diagnosis of Alexander disease. We initiated a multiinstitutional survey of MR abnormalities in both presumed and confirmed cases of Alexander disease to assess the possibility of an MR-based diagnosis.&lt;/p&gt;&lt;p&gt;&lt;i&gt;METHODS:&lt;/i&gt; MR imaging studies in three patients with an autopsy-based diagnosis of Alexander disease were analyzed to define MR criteria for the diagnosis. These criteria were then applied to 217 children with leukoencephalopathy of unknown origin.&lt;/p&gt;&lt;p&gt;&lt;i&gt;RESULTS:&lt;/i&gt; Five MR imaging criteria were defined: extensive cerebral white matter changes with frontal predominance, a periventricular rim with high signal on T1-weighted images and low signal on T2-weighted images, abnormalities of basal ganglia and thalami, brain stem abnormalities, and contrast enhancement of particular gray and white matter structures. Four of the five criteria had to be met for an MR imaging-based diagnosis. In a retrospective analysis of the MR studies of the 217 patients, 19 were found who fulfilled these criteria. No other essentially new MR abnormalities were found in these patients. In four of the 19 patients, subsequent histologic confirmation was obtained. The clinical symptomatology was the same in the patients with and without histologic confirmation and correlated well with the MR abnormalities. MR abnormalities were in close agreement with the known histopathologic findings of Alexander disease.&lt;/p&gt;&lt;p&gt;&lt;i&gt;CONCLUSION:&lt;/i&gt; The defined criteria are sufficient for an in vivo MR imaging diagnosis of Alexander disease; only in atypical cases is a brain biopsy still necessary for a definitive diagnosis.&lt;/p&gt;" />
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<meta name="citation_publication_date" content="2001/03/01" />
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<meta name="twitter:title" content="Alexander Disease: Diagnosis with MR Imaging" />
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<meta name="twitter:description" content="BACKGROUND AND PURPOSE: To date, the demonstration of Rosenthal fibers on brain biopsy or autopsy specimens is considered a prerequisite for a definitive diagnosis of Alexander disease. We initiated a multiinstitutional survey of MR abnormalities in both presumed and confirmed cases of Alexander disease to assess the possibility of an MR-based diagnosis.

METHODS: MR imaging studies in three patients with an autopsy-based diagnosis of Alexander disease were analyzed to define MR criteria for the diagnosis. These criteria were then applied to 217 children with leukoencephalopathy of unknown origin.

RESULTS: Five MR imaging criteria were defined: extensive cerebral white matter changes with frontal predominance, a periventricular rim with high signal on T1-weighted images and low signal on T2-weighted images, abnormalities of basal ganglia and thalami, brain stem abnormalities, and contrast enhancement of particular gray and white matter structures. Four of the five criteria had to be met for an MR imaging-based diagnosis. In a retrospective analysis of the MR studies of the 217 patients, 19 were found who fulfilled these criteria. No other essentially new MR abnormalities were found in these patients. In four of the 19 patients, subsequent histologic confirmation was obtained. The clinical symptomatology was the same in the patients with and without histologic confirmation and correlated well with the MR abnormalities. MR abnormalities were in close agreement with the known histopathologic findings of Alexander disease.

CONCLUSION: The defined criteria are sufficient for an in vivo MR imaging diagnosis of Alexander disease; only in atypical cases is a brain biopsy still necessary for a definitive diagnosis." />
<meta name="og-title" property="og:title" content="Alexander Disease: Diagnosis with MR Imaging" />
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<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: To date, the demonstration of Rosenthal fibers on brain biopsy or autopsy specimens is considered a prerequisite for a definitive diagnosis of Alexander disease. We initiated a multiinstitutional survey of MR abnormalities in both presumed and confirmed cases of Alexander disease to assess the possibility of an MR-based diagnosis.

METHODS: MR imaging studies in three patients with an autopsy-based diagnosis of Alexander disease were analyzed to define MR criteria for the diagnosis. These criteria were then applied to 217 children with leukoencephalopathy of unknown origin.

RESULTS: Five MR imaging criteria were defined: extensive cerebral white matter changes with frontal predominance, a periventricular rim with high signal on T1-weighted images and low signal on T2-weighted images, abnormalities of basal ganglia and thalami, brain stem abnormalities, and contrast enhancement of particular gray and white matter structures. Four of the five criteria had to be met for an MR imaging-based diagnosis. In a retrospective analysis of the MR studies of the 217 patients, 19 were found who fulfilled these criteria. No other essentially new MR abnormalities were found in these patients. In four of the 19 patients, subsequent histologic confirmation was obtained. The clinical symptomatology was the same in the patients with and without histologic confirmation and correlated well with the MR abnormalities. MR abnormalities were in close agreement with the known histopathologic findings of Alexander disease.

CONCLUSION: The defined criteria are sufficient for an in vivo MR imaging diagnosis of Alexander disease; only in atypical cases is a brain biopsy still necessary for a definitive diagnosis." />
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      <h1 class="highwire-cite-title" id="page-title">Alexander Disease: Diagnosis with MR Imaging</h1>  
    	<div class="highwire-cite-authors"><span  class="highwire-citation-authors"><span class="highwire-citation-author first" data-delta="0">Marjo S. van der Knaap</span>, <span class="highwire-citation-author" data-delta="1">Sakkubai Naidu</span>, <span class="highwire-citation-author" data-delta="2">Steven N. Breiter</span>, <span class="highwire-citation-author" data-delta="3">Susan Blaser</span>, <span class="highwire-citation-author" data-delta="4">Hans Stroink</span>, <span class="highwire-citation-author" data-delta="5">Stephan Springer</span>, <span class="highwire-citation-author" data-delta="6">Jacobus C. Begeer</span>, <span class="highwire-citation-author" data-delta="7">Rudy van Coster</span>, <span class="highwire-citation-author" data-delta="8">Peter G. Barth</span>, <span class="highwire-citation-author" data-delta="9">Neil H. Thomas</span>, <span class="highwire-citation-author" data-delta="10">Jacob Valk</span> and <span class="highwire-citation-author" data-delta="11">James M. Powers</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">March 2001,  </span><span  class="highwire-cite-metadata-volume highwire-cite-metadata">22 </span><span  class="highwire-cite-metadata-issue highwire-cite-metadata">(3) </span><span  class="highwire-cite-metadata-pages highwire-cite-metadata">541-552; </span></div>
  
  
  
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<div id="hw-article-author-popups-top-node-2431--6610148733" style="display: none;"><div class="author-tooltip-0"><div class="author-tooltip-name">Marjo S. van der Knaap </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=Marjo%2BS.%2Bvan%2Bder%2BKnaap%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%20Knaap%20MS&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%3AMarjo%2BS.%2Bvan%2Bder%2BKnaap%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">Sakkubai Naidu </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=Sakkubai%2BNaidu%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=Naidu%20S&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%3ASakkubai%2BNaidu%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">Steven N. Breiter </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=Steven%2BN.%2BBreiter%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=Breiter%20SN&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%3ASteven%2BN.%2BBreiter%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">Susan Blaser </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=Susan%2BBlaser%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=Blaser%20S&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%3ASusan%2BBlaser%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">Hans Stroink </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=Hans%2BStroink%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=Stroink%20H&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%3AHans%2BStroink%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-5"><div class="author-tooltip-name">Stephan Springer </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=Stephan%2BSpringer%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=Springer%20S&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%3AStephan%2BSpringer%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-6"><div class="author-tooltip-name">Jacobus C. 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        	<div class="highwire-cite-metadata"><span  class="highwire-cite-metadata-volume highwire-cite-metadata">Vol. 22</span>, <span  class="highwire-cite-metadata-issue highwire-cite-metadata">Issue 3 </span><div  class="highwire-cite-metadata-pubdate highwire-cite-metadata">1 Mar 2001 </div></div>
      
      
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    	<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">Mar 2001, </span><span  class="highwire-cite-metadata-volume highwire-cite-metadata">22 </span><span  class="highwire-cite-metadata-issue highwire-cite-metadata">(3) </span><span  class="highwire-cite-metadata-pages highwire-cite-metadata">541-552; </span></div>
  
  
  
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