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<meta name="DC.Title" content="Isolated Brain Stem Lesion in Children: Is It Acute Disseminated Encephalomyelitis or Not?" />
<meta name="DC.Identifier" content="10.3174/ajnr.A3176" />
<meta name="DC.Date" content="2013-01-01" />
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<meta name="DC.Description" content="SUMMARY: Isolated brain stem lesions presenting with acute neurologic findings create a major diagnostic dilemma in children. Although the brain stem is frequently involved in ADEM, solitary brain stem lesions are unusual. We performed a retrospective review in 6 children who presented with an inflammatory lesion confined to the brain stem. Two children were diagnosed with connective tissue disorder, CNS lupus, and localized scleroderma. The etiology could not be determined in 1, and clinical features suggested monophasic demyelination in 3. In these 3 children, initial lesions demonstrated vasogenic edema; all showed dramatic response to high-dose corticosteroids and made a full clinical recovery. Follow-up MRI showed complete resolution of lesions, and none had relapses at &gt;2 years of follow-up. In retrospect, these cases are best regarded as a localized form of ADEM. We conclude that though ADEM is typically a disseminated disease with multifocal lesions, it rarely presents with monofocal demyelination confined to the brain stem.

ADEM
:   acute disseminated encephalomyelitis
CIS
:   clinically isolated syndrome
CPM
:   central pontine myelinolysis
IPMSSG
:   International Pediatric Multiple Sclerosis Study Group
POLG 
:   polymerase subunit-gamma" />
<meta name="DC.Contributor" content="G. Alper" />
<meta name="DC.Contributor" content="G. Sreedher" />
<meta name="DC.Contributor" content="G. Zuccoli" />
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<meta name="citation_abstract" lang="en" content="&lt;h3&gt;SUMMARY:&lt;/h3&gt;
&lt;p&gt;Isolated brain stem lesions presenting with acute neurologic findings create a major diagnostic dilemma in children. Although the brain stem is frequently involved in ADEM, solitary brain stem lesions are unusual. We performed a retrospective review in 6 children who presented with an inflammatory lesion confined to the brain stem. Two children were diagnosed with connective tissue disorder, CNS lupus, and localized scleroderma. The etiology could not be determined in 1, and clinical features suggested monophasic demyelination in 3. In these 3 children, initial lesions demonstrated vasogenic edema; all showed dramatic response to high-dose corticosteroids and made a full clinical recovery. Follow-up MRI showed complete resolution of lesions, and none had relapses at &amp;gt;2 years of follow-up. In retrospect, these cases are best regarded as a localized form of ADEM. We conclude that though ADEM is typically a disseminated disease with multifocal lesions, it rarely presents with monofocal demyelination confined to the brain stem.&lt;/p&gt;" />
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<meta name="twitter:title" content="Isolated Brain Stem Lesion in Children: Is It Acute Disseminated Encephalomyelitis or Not?" />
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<meta name="twitter:description" content="SUMMARY: Isolated brain stem lesions presenting with acute neurologic findings create a major diagnostic dilemma in children. Although the brain stem is frequently involved in ADEM, solitary brain stem lesions are unusual. We performed a retrospective review in 6 children who presented with an inflammatory lesion confined to the brain stem. Two children were diagnosed with connective tissue disorder, CNS lupus, and localized scleroderma. The etiology could not be determined in 1, and clinical features suggested monophasic demyelination in 3. In these 3 children, initial lesions demonstrated vasogenic edema; all showed dramatic response to high-dose corticosteroids and made a full clinical recovery. Follow-up MRI showed complete resolution of lesions, and none had relapses at &gt;2 years of follow-up. In retrospect, these cases are best regarded as a localized form of ADEM. We conclude that though ADEM is typically a disseminated disease with multifocal lesions, it rarely presents with monofocal demyelination confined to the brain stem.

ADEM
:   acute disseminated encephalomyelitis
CIS
:   clinically isolated syndrome
CPM
:   central pontine myelinolysis
IPMSSG
:   International Pediatric Multiple Sclerosis Study Group
POLG 
:   polymerase subunit-gamma" />
<meta name="og-title" property="og:title" content="Isolated Brain Stem Lesion in Children: Is It Acute Disseminated Encephalomyelitis or Not?" />
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<meta name="og-description" property="og:description" content="SUMMARY: Isolated brain stem lesions presenting with acute neurologic findings create a major diagnostic dilemma in children. Although the brain stem is frequently involved in ADEM, solitary brain stem lesions are unusual. We performed a retrospective review in 6 children who presented with an inflammatory lesion confined to the brain stem. Two children were diagnosed with connective tissue disorder, CNS lupus, and localized scleroderma. The etiology could not be determined in 1, and clinical features suggested monophasic demyelination in 3. In these 3 children, initial lesions demonstrated vasogenic edema; all showed dramatic response to high-dose corticosteroids and made a full clinical recovery. Follow-up MRI showed complete resolution of lesions, and none had relapses at &gt;2 years of follow-up. In retrospect, these cases are best regarded as a localized form of ADEM. We conclude that though ADEM is typically a disseminated disease with multifocal lesions, it rarely presents with monofocal demyelination confined to the brain stem.

ADEM
:   acute disseminated encephalomyelitis
CIS
:   clinically isolated syndrome
CPM
:   central pontine myelinolysis
IPMSSG
:   International Pediatric Multiple Sclerosis Study Group
POLG 
:   polymerase subunit-gamma" />
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      <h1 class="highwire-cite-title" id="page-title">Isolated Brain Stem Lesion in Children: Is It Acute Disseminated Encephalomyelitis or Not?</h1>  
    	<div class="highwire-cite-authors"><span  class="highwire-citation-authors"><span class="highwire-citation-author first" data-delta="0">G. Alper</span>, <span class="highwire-citation-author" data-delta="1">G. Sreedher</span> and <span class="highwire-citation-author" data-delta="2">G. Zuccoli</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">January 2013,  </span><span  class="highwire-cite-metadata-volume highwire-cite-metadata">34 </span><span  class="highwire-cite-metadata-issue highwire-cite-metadata">(1) </span><span  class="highwire-cite-metadata-pages highwire-cite-metadata">217-220; </span><span  class="highwire-cite-metadata-doi highwire-cite-metadata">DOI: https://doi.org/10.3174/ajnr.A3176 </span></div>
  
  
  
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<div id="hw-article-author-popups-top-node-12421--61601257611" style="display: none;"><div class="author-tooltip-0"><div class="author-tooltip-name">G. Alper </div><div class="author-tooltip-affiliation"><span class="author-tooltip-text"><div class='author-affiliation'><span class='nlm-sup'>a</span>From the Division of Child Neurology (G.A.), Department of Pediatrics</div></span></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=G.%2BAlper%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=Alper%20G&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%3AG.%2BAlper%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">G. Sreedher </div><div class="author-tooltip-affiliation"><span class="author-tooltip-text"><div class='author-affiliation'><span class='nlm-sup'>b</span>Department of Radiology (G.S., G.Z.), University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania.</div></span></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=G.%2BSreedher%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=Sreedher%20G&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%3AG.%2BSreedher%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">G. Zuccoli </div><div class="author-tooltip-affiliation"><span class="author-tooltip-text"><div class='author-affiliation'><span class='nlm-sup'>b</span>Department of Radiology (G.S., G.Z.), University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania.</div></span></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=G.%2BZuccoli%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=Zuccoli%20G&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%3AG.%2BZuccoli%2B" rel="nofollow" class="" data-icon-position="" data-hide-link-title="0">Search for this author on this site</a></li></ul></div></div></div>  </div>

  
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<p id="p-1"><strong>SUMMARY:</strong> Isolated brain stem lesions presenting with acute neurologic findings create a major diagnostic dilemma in children. Although the brain stem is frequently involved in ADEM, solitary brain stem lesions are unusual. We performed a retrospective review in 6 children who presented with an inflammatory lesion confined to the brain stem. Two children were diagnosed with connective tissue disorder, CNS lupus, and localized scleroderma. The etiology could not be determined in 1, and clinical features suggested monophasic demyelination in 3. In these 3 children, initial lesions demonstrated vasogenic edema; all showed dramatic response to high-dose corticosteroids and made a full clinical recovery. Follow-up MRI showed complete resolution of lesions, and none had relapses at &gt;2 years of follow-up. In retrospect, these cases are best regarded as a localized form of ADEM. We conclude that though ADEM is typically a disseminated disease with multifocal lesions, it rarely presents with monofocal demyelination confined to the brain stem.</p>
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<h2>ABBREVIATIONS:</h2>
<dl id="def-list-1"><dt id="def-item-1">ADEM<span class="def-item-dt-sep"></span></dt><dd>acute disseminated encephalomyelitis<span class="def-item-dd-sep"></span></dd><dt id="def-item-2">CIS<span class="def-item-dt-sep"></span></dt><dd>clinically isolated syndrome<span class="def-item-dd-sep"></span></dd><dt id="def-item-3">CPM<span class="def-item-dt-sep"></span></dt><dd>central pontine myelinolysis<span class="def-item-dd-sep"></span></dd><dt id="def-item-4">IPMSSG<span class="def-item-dt-sep"></span></dt><dd>International Pediatric Multiple Sclerosis Study Group<span class="def-item-dd-sep"></span></dd><dt id="def-item-5"><em>POLG</em><span class="def-item-dt-sep"></span></dt><dd>polymerase subunit-gamma</dd></dl>
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      <div class="highwire-cite-title">Isolated Brain Stem Lesion in Children: Is It Acute Disseminated Encephalomyelitis or Not?</div>  
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