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<meta name="DC.Description" content="We describe the clinical and MR findings in three cases of rhombencephalosynapsis, a rare congenital malformation of the posterior fossa consisting of vermian agenesis or severe hypogenesis, fusion of the cerebellar hemispheres, and apposition or fusion of the dentate nuclei. Associated anomalies include hydrocephalus, fusion of the inferior colliculi, deficiency or absence of the septum pellucidum, and hypoplasia of the anterior commissure. Fourteen previous cases of rhombencephalosynapsis have been reported including Obersteiner's first report in 1914. The clinical presentation is variable, ranging from early death to variable degrees of cerebellar dysfunction and developmental delay. Patients may reach young adulthood. We report three additional cases and provide radiographic (MR) images of this unusual anomaly detected during life. Diagnoses in three children with rhombencephalosynapsis were made on the basis of MR findings. To our knowledge, this is the first report of this disorder being diagnosed in living patients." />
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      <h1 class="highwire-cite-title" id="page-title">MR imaging of rhombencephalosynapsis: report of three cases and review of the literature.</h1>  
    	<div class="highwire-cite-authors"><span  class="highwire-citation-authors"><span class="highwire-citation-author first" data-delta="0">C L Truwit</span>, <span class="highwire-citation-author" data-delta="1">A J Barkovich</span>, <span class="highwire-citation-author" data-delta="2">R Shanahan</span> and <span class="highwire-citation-author" data-delta="3">T V Maroldo</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">September 1991,  </span><span  class="highwire-cite-metadata-volume highwire-cite-metadata">12 </span><span  class="highwire-cite-metadata-issue highwire-cite-metadata">(5) </span><span  class="highwire-cite-metadata-pages highwire-cite-metadata">957-965; </span></div>
  
  
  
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<div id="hw-article-author-popups-top-node-5447--61612905913" style="display: none;"><div class="author-tooltip-0"><div class="author-tooltip-name">C L Truwit </div><div class="author-tooltip-affiliation"><span class="author-tooltip-text"><div class='author-affiliation'>Department of Radiology, University of California, San Francisco 94143-0628.</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=C%2BL%2BTruwit%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=Truwit%20CL&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%2BL%2BTruwit%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">A J Barkovich </div><div class="author-tooltip-affiliation"><span class="author-tooltip-text"><div class='author-affiliation'>Department of Radiology, University of California, San Francisco 94143-0628.</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=A%2BJ%2BBarkovich%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=Barkovich%20AJ&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%3AA%2BJ%2BBarkovich%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">R Shanahan </div><div class="author-tooltip-affiliation"><span class="author-tooltip-text"><div class='author-affiliation'>Department of Radiology, University of California, San Francisco 94143-0628.</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=R%2BShanahan%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=Shanahan%20R&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%3AR%2BShanahan%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">T V Maroldo </div><div class="author-tooltip-affiliation"><span class="author-tooltip-text"><div class='author-affiliation'>Department of Radiology, University of California, San Francisco 94143-0628.</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=T%2BV%2BMaroldo%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=Maroldo%20TV&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%3AT%2BV%2BMaroldo%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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      <div class="highwire-cite-title">MR imaging of rhombencephalosynapsis: report of three cases and review of the literature.</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">C L</span> <span class="nlm-surname">Truwit</span></span>, <span class="highwire-citation-author" data-delta="1"><span class="nlm-given-names">A J</span> <span class="nlm-surname">Barkovich</span></span>, <span class="highwire-citation-author" data-delta="2"><span class="nlm-given-names">R</span> <span class="nlm-surname">Shanahan</span></span>, <span class="highwire-citation-author" data-delta="3"><span class="nlm-given-names">T V</span> <span class="nlm-surname">Maroldo</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">Sep 1991, </span><span  class="highwire-cite-metadata-volume highwire-cite-metadata">12 </span><span  class="highwire-cite-metadata-issue highwire-cite-metadata">(5) </span><span  class="highwire-cite-metadata-pages highwire-cite-metadata">957-965; </span></div>
  
  
  
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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">C L</span> <span class="nlm-surname">Truwit</span></span>, <span class="highwire-citation-author" data-delta="1"><span class="nlm-given-names">A J</span> <span class="nlm-surname">Barkovich</span></span>, <span class="highwire-citation-author" data-delta="2"><span class="nlm-given-names">R</span> <span class="nlm-surname">Shanahan</span></span>, <span class="highwire-citation-author" data-delta="3"><span class="nlm-given-names">T V</span> <span class="nlm-surname">Maroldo</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">Sep 1991, </span><span  class="highwire-cite-metadata-volume highwire-cite-metadata">12 </span><span  class="highwire-cite-metadata-issue highwire-cite-metadata">(5) </span><span  class="highwire-cite-metadata-pages highwire-cite-metadata">957-965; </span></div>
  
  
  
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