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      <h1 class="highwire-cite-title" id="page-title">Optic Canal Narrowing in Hunter Syndrome (Mucopolysaccharidosis II)</h1>  
    	<div class="highwire-cite-authors"><span  class="highwire-citation-authors"><span class="highwire-citation-author first" data-delta="0">A. Sen</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 2013,  </span><span  class="highwire-cite-metadata-volume highwire-cite-metadata">34 </span><span  class="highwire-cite-metadata-issue highwire-cite-metadata">(3) </span><span  class="highwire-cite-metadata-pages highwire-cite-metadata">E34-E35; </span><span  class="highwire-cite-metadata-doi highwire-cite-metadata">DOI: https://doi.org/10.3174/ajnr.A3499 </span></div>
  
  
  
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<div id="hw-article-author-popups-top-node-12275--61280377962" style="display: none;"><div class="author-tooltip-0"><div class="author-tooltip-name">A. Sen </div><div class="author-tooltip-affiliation"><span class="author-tooltip-text"><div class='author-affiliation'><span class='nlm-sup'>a</span>Radiodiagnosis
Devi Scans
Kerala, India</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.%2BSen%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=Sen%20A&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.%2BSen%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-markup"><div xmlns="http://www.w3.org/1999/xhtml" data-highwire-cite-ref-tooltip-instance="highwire_reflinks_tooltip" class="content-block-markup" xmlns:xhtml="http://www.w3.org/1999/xhtml"><div class="article fulltext-view "><span class="highwire-journal-article-marker-start"></span><p id="p-1">I read with interest the article “Brain and Spinal MR Imaging Findings in Mucopolysaccharidoses: A Review”<sup><a id="xref-ref-1-1" class="xref-bibr" href="#ref-1">1</a></sup> and would like to illustrate optic canal narrowing in a patient with Hunter syndrome (mucopolysaccharidosis [MPS]-II).</p><p id="p-2">A 6-year-old boy presented with significant loss of vision. He had been evaluated (at 3 years of age) for mucopolysaccharidosis; clinical phenotype, heparin sulfate in urine electrophoresis, and plasma enzyme levels (iduronate 2-sulfatase level: 4.2 nmol/h by fluorimetric assay by using 4-methyl umbelliferone; normal range: 167–475 nmol/h; control: 194.3) were suggestive of Hunter syndrome.</p><p id="p-3">MR imaging of the brain showed periventricular white matter hyperintensities (<a id="xref-fig-1-1" class="xref-fig" href="#F1">Fig 1</a><em>A–C</em>), enlarged Virchow-Robin spaces (<a id="xref-fig-1-2" class="xref-fig" href="#F1">Fig 1</a><em>A</em>, <em>-B</em>), ventriculomegaly, and atrophy (<a id="xref-fig-1-3" class="xref-fig" href="#F1">Fig 1</a><em>A–C</em>, <em>-E</em>, <em>-F</em>), known features of mucopolysaccharidosis. The bilateral optic canals were symmetrically narrowed (<a id="xref-fig-1-4" class="xref-fig" href="#F1">Fig 1</a><em>D–F</em>), and hyperintensities were noted around both optic nerves (<a id="xref-fig-1-5" class="xref-fig" href="#F1">Fig 1</a><em>C–F</em>).</p><div id="F1" class="fig pos-float  odd"><div class="highwire-figure"><div class="fig-inline-img-wrapper"><div class="fig-inline-img"><a href="http://ajnr-stage2.stage.highwire.org/content/ajnr/34/3/E34/F1.large.jpg?width=800&amp;height=600&amp;carousel=1" title="Axial T2 (A) and axial T2 FLAIR (B) images show periventricular white matter hyperintensities (arrows), prominent Virchow-Robin spaces (arrowheads), ventriculomegaly, and brain atrophy. C, Coronal T2 image shows hyperintensities in periventricular white matter (arrows) and perioptic spaces (arrowheads), ventriculomegaly, and brain atrophy. Axial T2 (D) and right (E) and left (F) sagittal T2 images show narrowing of the optic canals (arrows), hyperintensities in perioptic spaces, and brain atrophy." class="highwire-fragment fragment-images colorbox-load" rel="gallery-fragment-images-1844228413" data-figure-caption="&lt;div class=&quot;highwire-markup&quot;&gt;&lt;div xmlns=&quot;http://www.w3.org/1999/xhtml&quot;&gt;Axial T2 (&lt;em&gt;A&lt;/em&gt;) and axial T2 FLAIR (&lt;em&gt;B&lt;/em&gt;) images show periventricular white matter hyperintensities (&lt;em&gt;arrows&lt;/em&gt;), prominent Virchow-Robin spaces (&lt;em&gt;arrowheads&lt;/em&gt;), ventriculomegaly, and brain atrophy. &lt;em&gt;C&lt;/em&gt;, Coronal T2 image shows hyperintensities in periventricular white matter (&lt;em&gt;arrows&lt;/em&gt;) and perioptic spaces (&lt;em&gt;arrowheads&lt;/em&gt;), ventriculomegaly, and brain atrophy. Axial T2 (&lt;em&gt;D&lt;/em&gt;) and right (&lt;em&gt;E&lt;/em&gt;) and left (&lt;em&gt;F&lt;/em&gt;) sagittal T2 images show narrowing of the optic canals (&lt;em&gt;arrows&lt;/em&gt;), hyperintensities in perioptic spaces, and brain atrophy.&lt;/div&gt;&lt;/div&gt;" data-icon-position="" data-hide-link-title="0"><span class="hw-responsive-img"><img class="highwire-fragment fragment-image lazyload" alt="Fig. 1." src="data:image/gif;base64,R0lGODlhAQABAIAAAAAAAP///yH5BAEAAAAALAAAAAABAAEAAAIBRAA7" data-src="http://ajnr-stage2.stage.highwire.org/content/ajnr/34/3/E34/F1.medium.gif" width="360" height="440"/><noscript><img class="highwire-fragment fragment-image" alt="Fig. 1." src="http://ajnr-stage2.stage.highwire.org/content/ajnr/34/3/E34/F1.medium.gif" width="360" height="440"/></noscript></span></a></div></div><ul class="highwire-figure-links inline"><li class="download-fig first"><a href="http://ajnr-stage2.stage.highwire.org/content/ajnr/34/3/E34/F1.large.jpg?download=true" class="highwire-figure-link highwire-figure-link-download" title="Download Fig. 1." data-icon-position="" data-hide-link-title="0">Download figure</a></li><li class="new-tab"><a href="http://ajnr-stage2.stage.highwire.org/content/ajnr/34/3/E34/F1.large.jpg" class="highwire-figure-link highwire-figure-link-newtab" target="_blank" data-icon-position="" data-hide-link-title="0">Open in new tab</a></li><li class="download-ppt last"><a href="/highwire/powerpoint/39780" class="highwire-figure-link highwire-figure-link-ppt" data-icon-position="" data-hide-link-title="0">Download powerpoint</a></li></ul></div><div class="fig-caption" xmlns:xhtml="http://www.w3.org/1999/xhtml"><span class="fig-label">Fig. 1.</span> <p id="p-4" class="first-child">Axial T2 (<em>A</em>) and axial T2 FLAIR (<em>B</em>) images show periventricular white matter hyperintensities (<em>arrows</em>), prominent Virchow-Robin spaces (<em>arrowheads</em>), ventriculomegaly, and brain atrophy. <em>C</em>, Coronal T2 image shows hyperintensities in periventricular white matter (<em>arrows</em>) and perioptic spaces (<em>arrowheads</em>), ventriculomegaly, and brain atrophy. Axial T2 (<em>D</em>) and right (<em>E</em>) and left (<em>F</em>) sagittal T2 images show narrowing of the optic canals (<em>arrows</em>), hyperintensities in perioptic spaces, and brain atrophy.</p><div class="sb-div caption-clear"></div></div></div><p id="p-5">Hunter syndrome, or mucopolysaccharidosis II, is an X-linked progressive lysosomal storage disease due to deficiency of the lysosomal enzyme iduronate-2-sulfatase. Known ocular manifestations of Hunter syndrome include exophthalmos, hypertelorism, pigmentary retinopathy, uveal effusion, optic nerve head swelling (disc edema,<sup><a id="xref-ref-2-1" class="xref-bibr" href="#ref-2">2</a></sup> possibly due to compression by scleral deposits), and optic atrophy.</p><p id="p-6">Optic canal narrowing is a known feature of osteopetrosis,<sup><a id="xref-ref-3-1" class="xref-bibr" href="#ref-3">3</a></sup> and fibrous dysplasia and has been described in familial hypophosphatemic rickets, carbonic anhydrase type 2 deficiency (which includes osteopetrosis), and MPS-4.<sup><a id="xref-ref-4-1" class="xref-bibr" href="#ref-4">4</a></sup> It has not been previously reported in Hunter syndrome. Perhaps optic canal narrowing is underdiagnosed in Hunter syndrome because intellectually impaired children may not be able to describe visual loss.</p><div class="section ref-list" id="ref-list-1"><h2>References</h2><ol class="cit-list ref-use-labels"><li><span class="ref-label">1.</span><a class="rev-xref-ref" href="#xref-ref-1-1" title="View reference 1. in text" id="ref-1">↵</a>

<div class="cit ref-cit ref-journal" id="cit-34.3.E34.1" data-doi="10.3174/ajnr.A2832"><div class="cit-metadata"><ol class="cit-auth-list"><li><span class="cit-auth"><span class="cit-name-surname">Zafeiriou</span>  <span class="cit-name-given-names">DI</span></span>, </li><li><span class="cit-auth"><span class="cit-name-surname">Batzios</span>  <span class="cit-name-given-names">SP</span></span></li></ol><cite>. <span class="cit-article-title">Brain and spinal MR imaging findings in mucopolysaccharidoses: a review</span>. <abbr class="cit-jnl-abbrev">AJNR Am J Neuroradiol</abbr> <span class="cit-pub-date">2013</span>;<span class="cit-vol">34</span>:<span class="cit-fpage">5</span>–<span class="cit-lpage">13</span></cite></div><div class="cit-extra"><a href="/lookup/ijlink/YTozOntzOjQ6InBhdGgiO3M6MTQ6Ii9sb29rdXAvaWpsaW5rIjtzOjU6InF1ZXJ5IjthOjQ6e3M6ODoibGlua1R5cGUiO3M6NDoiQUJTVCI7czoxMToiam91cm5hbENvZGUiO3M6NDoiYWpuciI7czo1OiJyZXNpZCI7czo2OiIzNC8xLzUiO3M6NDoiYXRvbSI7czoxOToiL2FqbnIvMzQvMy9FMzQuYXRvbSI7fXM6ODoiZnJhZ21lbnQiO3M6MDoiIjt9" class="cit-ref-sprinkles cit-ref-sprinkles-ijlink"><span><span class="cit-reflinks-abstract">Abstract</span><span class="cit-sep cit-reflinks-variant-name-sep">/</span><span class="cit-reflinks-full-text"><span class="free-full-text">FREE </span>Full Text</span></span></a></div></div></li><li><span class="ref-label">2.</span><a class="rev-xref-ref" href="#xref-ref-2-1" title="View reference 2. in text" id="ref-2">↵</a>

<div class="cit ref-cit ref-journal" id="cit-34.3.E34.2" data-doi="10.1136/bjo.68.8.590"><div class="cit-metadata"><ol class="cit-auth-list"><li><span class="cit-auth"><span class="cit-name-surname">Beck</span>  <span class="cit-name-given-names">M</span></span>, </li><li><span class="cit-auth"><span class="cit-name-surname">Cole</span>  <span class="cit-name-given-names">G</span></span></li></ol><cite>. <span class="cit-article-title">Disc oedema in association with Hunter's syndrome: ocular histopathological findings</span>. <abbr class="cit-jnl-abbrev">Br J Ophthalmol</abbr> <span class="cit-pub-date">1984</span>;<span class="cit-vol">68</span>:<span class="cit-fpage">590</span>–<span class="cit-lpage">94</span></cite></div><div class="cit-extra"><a href="/lookup/ijlink/YTozOntzOjQ6InBhdGgiO3M6MTQ6Ii9sb29rdXAvaWpsaW5rIjtzOjU6InF1ZXJ5IjthOjQ6e3M6ODoibGlua1R5cGUiO3M6NDoiQUJTVCI7czoxMToiam91cm5hbENvZGUiO3M6MTI6ImJqb3BodGhhbG1vbCI7czo1OiJyZXNpZCI7czo4OiI2OC84LzU5MCI7czo0OiJhdG9tIjtzOjE5OiIvYWpuci8zNC8zL0UzNC5hdG9tIjt9czo4OiJmcmFnbWVudCI7czowOiIiO30=" class="cit-ref-sprinkles cit-ref-sprinkles-ijlink"><span><span class="cit-reflinks-abstract">Abstract</span><span class="cit-sep cit-reflinks-variant-name-sep">/</span><span class="cit-reflinks-full-text"><span class="free-full-text">FREE </span>Full Text</span></span></a></div></div></li><li><span class="ref-label">3.</span><a class="rev-xref-ref" href="#xref-ref-3-1" title="View reference 3. in text" id="ref-3">↵</a>

<div class="cit ref-cit ref-journal" id="cit-34.3.E34.3"><div class="cit-metadata"><ol class="cit-auth-list"><li><span class="cit-auth"><span class="cit-name-surname">Curé</span>  <span class="cit-name-given-names">JK</span></span>, </li><li><span class="cit-auth"><span class="cit-name-surname">Key</span>  <span class="cit-name-given-names">LL</span></span>, </li><li><span class="cit-auth"><span class="cit-name-surname">Goltra</span>  <span class="cit-name-given-names">DD</span></span>, </li><li><span class="cit-etal">et al</span></li></ol><cite>. <span class="cit-article-title">Cranial MR imaging of osteopetrosis</span>. <abbr class="cit-jnl-abbrev">AJNR Am J Neuroradiol</abbr> <span class="cit-pub-date">2000</span>;<span class="cit-vol">21</span>:<span class="cit-fpage">1110</span>–<span class="cit-lpage">15</span></cite></div><div class="cit-extra"><a href="/lookup/ijlink/YTozOntzOjQ6InBhdGgiO3M6MTQ6Ii9sb29rdXAvaWpsaW5rIjtzOjU6InF1ZXJ5IjthOjQ6e3M6ODoibGlua1R5cGUiO3M6NDoiQUJTVCI7czoxMToiam91cm5hbENvZGUiO3M6NDoiYWpuciI7czo1OiJyZXNpZCI7czo5OiIyMS82LzExMTAiO3M6NDoiYXRvbSI7czoxOToiL2FqbnIvMzQvMy9FMzQuYXRvbSI7fXM6ODoiZnJhZ21lbnQiO3M6MDoiIjt9" class="cit-ref-sprinkles cit-ref-sprinkles-ijlink"><span><span class="cit-reflinks-abstract">Abstract</span><span class="cit-sep cit-reflinks-variant-name-sep">/</span><span class="cit-reflinks-full-text"><span class="free-full-text">FREE </span>Full Text</span></span></a></div></div></li><li><span class="ref-label">4.</span><a class="rev-xref-ref" href="#xref-ref-4-1" title="View reference 4. in text" id="ref-4">↵</a>

<div class="cit ref-cit ref-journal" id="cit-34.3.E34.4" data-doi="10.1159/000050383"><div class="cit-metadata"><ol class="cit-auth-list"><li><span class="cit-auth"><span class="cit-name-surname">Vougioukas</span>  <span class="cit-name-given-names">VI</span></span>, </li><li><span class="cit-auth"><span class="cit-name-surname">Berlis</span>  <span class="cit-name-given-names">A</span></span>, </li><li><span class="cit-auth"><span class="cit-name-surname">Kopp</span>  <span class="cit-name-given-names">MV</span></span>, </li><li><span class="cit-etal">et al</span></li></ol><cite>. <span class="cit-article-title">Neurosurgical interventions in children with Maroteaux-Lamy syndrome: case report and review of the literature</span>. <abbr class="cit-jnl-abbrev">Pediatr Neurosurg</abbr> <span class="cit-pub-date">2001</span>;<span class="cit-vol">35</span>:<span class="cit-fpage">35</span>–<span class="cit-lpage">38</span></cite></div><div class="cit-extra"><a href="/lookup/external-ref?access_num=10.1159/000050383&amp;link_type=DOI" class="cit-ref-sprinkles cit-ref-sprinkles-doi cit-ref-sprinkles-crossref"><span>CrossRef</span></a><a href="/lookup/external-ref?access_num=11490189&amp;link_type=MED&amp;atom=%2Fajnr%2F34%2F3%2FE34.atom" class="cit-ref-sprinkles cit-ref-sprinkles-medline"><span>PubMed</span></a></div></div></li></ol></div><ul class="copyright-statement"><li class="fn" id="copyright-statement-1">© 2013 by American Journal of Neuroradiology</li></ul><span class="highwire-journal-article-marker-end"></span></div><span class="related-urls"></span></div></div>  </div>

  
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