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<meta name="DC.Title" content="Incidence of Cerebellar Tonsillar Ectopia in Idiopathic Intracranial Hypertension: A Mimic of the Chiari I Malformation" />
<meta name="DC.Identifier" content="10.3174/ajnr.A3068" />
<meta name="DC.Date" content="2012-11-01" />
<meta name="DC.Publisher" content="American Journal of Neuroradiology" />
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<meta name="DC.Description" content="BACKGROUND AND PURPOSE: IIH is a syndrome of elevated intracranial pressure without hydrocephalus, mass, or identifiable cause. Diagnosis is made by clinical presentation, intracranial pressure measurement, and supportive imaging findings. A subset of patients with IIH may have tonsillar ectopia, meeting the criteria for Chiari malformation type I but not responding to surgical decompression for Chiari I. The purpose of this study was to determine the incidence and morphology of cerebellar tonsillar ectopia in patients with IIH.

MATERIALS AND METHODS: Forty-three patients with clinically confirmed IIH and 44 age-matched controls were included. Two neuroradiologists with CAQs reviewed sagittal T1-weighted MRI in a blinded fashion and measured cerebellar tonsil and obex positions relative to the foramen magnum and prepontine cistern width at the level of the midpons.

RESULTS: Nine of 43 patients with IIH and 1/44 controls had cerebellar tonsillar ectopia of ≥5 mm. Five of 9 of patients with IIH with ectopia of ≥5 mm also had a “peglike” tonsil configuration. Patients with IIH had a significantly lower tonsillar position (2.1 ± 2.8 mm) than age-matched controls (0.7 ±1.9 mm, P &lt; .05). The obex position was significantly lower in patients with IIH versus controls (−7.9 mm [above the FM] versus −9.4 mm [above the FM], P &lt; .05). The prepontine width was not significantly different between the groups.

CONCLUSIONS: Cerebellar tonsil position in patients with IIH was significantly lower than that in age-matched controls, often times peglike, mimicking Chiari I. A significantly lower obex position suggests an inferiorly displaced brain stem and cerebellum. When tonsillar ectopia of &gt;5 mm is identified, imaging and clinical consideration of IIH are warranted to avoid misdiagnosis as Chiari I.

CAQ
:   Certificate of Added Qualification
CM
:   Chiari malformation
FM
:   foramen magnum
ICP
:   intracranial pressure
IIH
:   idiopathic intracranial hypertension" />
<meta name="DC.Contributor" content="A.H. Aiken" />
<meta name="DC.Contributor" content="J.A. Hoots" />
<meta name="DC.Contributor" content="A.M. Saindane" />
<meta name="DC.Contributor" content="P.A. Hudgins" />
<meta name="article:published_time" content="2012-11-01" />
<meta name="article:section" content="Brain" />
<meta name="citation_title" content="Incidence of Cerebellar Tonsillar Ectopia in Idiopathic Intracranial Hypertension: A Mimic of the Chiari I Malformation" />
<meta name="citation_abstract" lang="en" content="&lt;h3&gt;BACKGROUND AND PURPOSE:&lt;/h3&gt;
&lt;p&gt;IIH is a syndrome of elevated intracranial pressure without hydrocephalus, mass, or identifiable cause. Diagnosis is made by clinical presentation, intracranial pressure measurement, and supportive imaging findings. A subset of patients with IIH may have tonsillar ectopia, meeting the criteria for Chiari malformation type I but not responding to surgical decompression for Chiari I. The purpose of this study was to determine the incidence and morphology of cerebellar tonsillar ectopia in patients with IIH.&lt;/p&gt;&lt;h3&gt;MATERIALS AND METHODS:&lt;/h3&gt;
&lt;p&gt;Forty-three patients with clinically confirmed IIH and 44 age-matched controls were included. Two neuroradiologists with CAQs reviewed sagittal T1-weighted MRI in a blinded fashion and measured cerebellar tonsil and obex positions relative to the foramen magnum and prepontine cistern width at the level of the midpons.&lt;/p&gt;&lt;h3&gt;RESULTS:&lt;/h3&gt;
&lt;p&gt;Nine of 43 patients with IIH and 1/44 controls had cerebellar tonsillar ectopia of ≥5 mm. Five of 9 of patients with IIH with ectopia of ≥5 mm also had a “peglike” tonsil configuration. Patients with IIH had a significantly lower tonsillar position (2.1 ± 2.8 mm) than age-matched controls (0.7 ±1.9 mm, &lt;i&gt;P&lt;/i&gt; &amp;lt; .05). The obex position was significantly lower in patients with IIH versus controls (−7.9 mm [above the FM] versus −9.4 mm [above the FM], &lt;i&gt;P&lt;/i&gt; &amp;lt; .05). The prepontine width was not significantly different between the groups.&lt;/p&gt;&lt;h3&gt;CONCLUSIONS:&lt;/h3&gt;
&lt;p&gt;Cerebellar tonsil position in patients with IIH was significantly lower than that in age-matched controls, often times peglike, mimicking Chiari I. A significantly lower obex position suggests an inferiorly displaced brain stem and cerebellum. When tonsillar ectopia of &amp;gt;5 mm is identified, imaging and clinical consideration of IIH are warranted to avoid misdiagnosis as Chiari I.&lt;/p&gt;" />
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<meta name="twitter:title" content="Incidence of Cerebellar Tonsillar Ectopia in Idiopathic Intracranial Hypertension: A Mimic of the Chiari I Malformation" />
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<meta name="twitter:description" content="BACKGROUND AND PURPOSE: IIH is a syndrome of elevated intracranial pressure without hydrocephalus, mass, or identifiable cause. Diagnosis is made by clinical presentation, intracranial pressure measurement, and supportive imaging findings. A subset of patients with IIH may have tonsillar ectopia, meeting the criteria for Chiari malformation type I but not responding to surgical decompression for Chiari I. The purpose of this study was to determine the incidence and morphology of cerebellar tonsillar ectopia in patients with IIH.

MATERIALS AND METHODS: Forty-three patients with clinically confirmed IIH and 44 age-matched controls were included. Two neuroradiologists with CAQs reviewed sagittal T1-weighted MRI in a blinded fashion and measured cerebellar tonsil and obex positions relative to the foramen magnum and prepontine cistern width at the level of the midpons.

RESULTS: Nine of 43 patients with IIH and 1/44 controls had cerebellar tonsillar ectopia of ≥5 mm. Five of 9 of patients with IIH with ectopia of ≥5 mm also had a “peglike” tonsil configuration. Patients with IIH had a significantly lower tonsillar position (2.1 ± 2.8 mm) than age-matched controls (0.7 ±1.9 mm, P &lt; .05). The obex position was significantly lower in patients with IIH versus controls (−7.9 mm [above the FM] versus −9.4 mm [above the FM], P &lt; .05). The prepontine width was not significantly different between the groups.

CONCLUSIONS: Cerebellar tonsil position in patients with IIH was significantly lower than that in age-matched controls, often times peglike, mimicking Chiari I. A significantly lower obex position suggests an inferiorly displaced brain stem and cerebellum. When tonsillar ectopia of &gt;5 mm is identified, imaging and clinical consideration of IIH are warranted to avoid misdiagnosis as Chiari I.

CAQ
:   Certificate of Added Qualification
CM
:   Chiari malformation
FM
:   foramen magnum
ICP
:   intracranial pressure
IIH
:   idiopathic intracranial hypertension" />
<meta name="og-title" property="og:title" content="Incidence of Cerebellar Tonsillar Ectopia in Idiopathic Intracranial Hypertension: A Mimic of the Chiari I Malformation" />
<meta name="og-url" property="og:url" content="http://ajnr-stage2.stage.highwire.org/content/33/10/1901" />
<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: IIH is a syndrome of elevated intracranial pressure without hydrocephalus, mass, or identifiable cause. Diagnosis is made by clinical presentation, intracranial pressure measurement, and supportive imaging findings. A subset of patients with IIH may have tonsillar ectopia, meeting the criteria for Chiari malformation type I but not responding to surgical decompression for Chiari I. The purpose of this study was to determine the incidence and morphology of cerebellar tonsillar ectopia in patients with IIH.

MATERIALS AND METHODS: Forty-three patients with clinically confirmed IIH and 44 age-matched controls were included. Two neuroradiologists with CAQs reviewed sagittal T1-weighted MRI in a blinded fashion and measured cerebellar tonsil and obex positions relative to the foramen magnum and prepontine cistern width at the level of the midpons.

RESULTS: Nine of 43 patients with IIH and 1/44 controls had cerebellar tonsillar ectopia of ≥5 mm. Five of 9 of patients with IIH with ectopia of ≥5 mm also had a “peglike” tonsil configuration. Patients with IIH had a significantly lower tonsillar position (2.1 ± 2.8 mm) than age-matched controls (0.7 ±1.9 mm, P &lt; .05). The obex position was significantly lower in patients with IIH versus controls (−7.9 mm [above the FM] versus −9.4 mm [above the FM], P &lt; .05). The prepontine width was not significantly different between the groups.

CONCLUSIONS: Cerebellar tonsil position in patients with IIH was significantly lower than that in age-matched controls, often times peglike, mimicking Chiari I. A significantly lower obex position suggests an inferiorly displaced brain stem and cerebellum. When tonsillar ectopia of &gt;5 mm is identified, imaging and clinical consideration of IIH are warranted to avoid misdiagnosis as Chiari I.

CAQ
:   Certificate of Added Qualification
CM
:   Chiari malformation
FM
:   foramen magnum
ICP
:   intracranial pressure
IIH
:   idiopathic intracranial hypertension" />
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      <h1 class="highwire-cite-title" id="page-title">Incidence of Cerebellar Tonsillar Ectopia in Idiopathic Intracranial Hypertension: A Mimic of the Chiari I Malformation</h1>  
    	<div class="highwire-cite-authors"><span  class="highwire-citation-authors"><span class="highwire-citation-author first" data-delta="0">A.H. Aiken</span>, <span class="highwire-citation-author" data-delta="1">J.A. Hoots</span>, <span class="highwire-citation-author" data-delta="2">A.M. Saindane</span> and <span class="highwire-citation-author" data-delta="3">P.A. Hudgins</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 2012,  </span><span  class="highwire-cite-metadata-volume highwire-cite-metadata">33 </span><span  class="highwire-cite-metadata-issue highwire-cite-metadata">(10) </span><span  class="highwire-cite-metadata-pages highwire-cite-metadata">1901-1906; </span><span  class="highwire-cite-metadata-doi highwire-cite-metadata">DOI: https://doi.org/10.3174/ajnr.A3068 </span></div>
  
  
  
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<div id="hw-article-author-popups-top-node-12158--6208777129" style="display: none;"><div class="author-tooltip-0"><div class="author-tooltip-name">A.H. Aiken </div><div class="author-tooltip-affiliation"><span class="author-tooltip-text"><div class='author-affiliation'><span class='nlm-sup'>a</span>From the Department of Radiology and Imaging Sciences, Emory University, Atlanta, Georgia.</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.H.%2BAiken%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=Aiken%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.H.%2BAiken%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">J.A. Hoots </div><div class="author-tooltip-affiliation"><span class="author-tooltip-text"><div class='author-affiliation'><span class='nlm-sup'>a</span>From the Department of Radiology and Imaging Sciences, Emory University, Atlanta, Georgia.</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=J.A.%2BHoots%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=Hoots%20J&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%3AJ.A.%2BHoots%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">A.M. Saindane </div><div class="author-tooltip-affiliation"><span class="author-tooltip-text"><div class='author-affiliation'><span class='nlm-sup'>a</span>From the Department of Radiology and Imaging Sciences, Emory University, Atlanta, Georgia.</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.M.%2BSaindane%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=Saindane%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.M.%2BSaindane%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">P.A. Hudgins </div><div class="author-tooltip-affiliation"><span class="author-tooltip-text"><div class='author-affiliation'><span class='nlm-sup'>a</span>From the Department of Radiology and Imaging Sciences, Emory University, Atlanta, Georgia.</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=P.A.%2BHudgins%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=Hudgins%20P&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%3AP.A.%2BHudgins%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-2"><strong>BACKGROUND AND PURPOSE:</strong> IIH is a syndrome of elevated intracranial pressure without hydrocephalus, mass, or identifiable cause. Diagnosis is made by clinical presentation, intracranial pressure measurement, and supportive imaging findings. A subset of patients with IIH may have tonsillar ectopia, meeting the criteria for Chiari malformation type I but not responding to surgical decompression for Chiari I. The purpose of this study was to determine the incidence and morphology of cerebellar tonsillar ectopia in patients with IIH.</p>
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<p id="p-3"><strong>MATERIALS AND METHODS:</strong> Forty-three patients with clinically confirmed IIH and 44 age-matched controls were included. Two neuroradiologists with CAQs reviewed sagittal T1-weighted MRI in a blinded fashion and measured cerebellar tonsil and obex positions relative to the foramen magnum and prepontine cistern width at the level of the midpons.</p>
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<p id="p-4"><strong>RESULTS:</strong> Nine of 43 patients with IIH and 1/44 controls had cerebellar tonsillar ectopia of ≥5 mm. Five of 9 of patients with IIH with ectopia of ≥5 mm also had a “peglike” tonsil configuration. Patients with IIH had a significantly lower tonsillar position (2.1 ± 2.8 mm) than age-matched controls (0.7 ±1.9 mm, <em>P</em> &lt; .05). The obex position was significantly lower in patients with IIH versus controls (−7.9 mm [above the FM] versus −9.4 mm [above the FM], <em>P</em> &lt; .05). The prepontine width was not significantly different between the groups.</p>
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<p id="p-5"><strong>CONCLUSIONS:</strong> Cerebellar tonsil position in patients with IIH was significantly lower than that in age-matched controls, often times peglike, mimicking Chiari I. A significantly lower obex position suggests an inferiorly displaced brain stem and cerebellum. When tonsillar ectopia of &gt;5 mm is identified, imaging and clinical consideration of IIH are warranted to avoid misdiagnosis as Chiari I.</p>
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</div><div class="section glossary" id="glossary-1"><h2>ABBREVIATIONS:</h2>
<dl id="def-list-1"><dt id="def-item-1">CAQ<span class="def-item-dt-sep"></span></dt><dd>Certificate of Added Qualification<span class="def-item-dd-sep"></span></dd><dt id="def-item-2">CM<span class="def-item-dt-sep"></span></dt><dd>Chiari malformation<span class="def-item-dd-sep"></span></dd><dt id="def-item-3">FM<span class="def-item-dt-sep"></span></dt><dd>foramen magnum<span class="def-item-dd-sep"></span></dd><dt id="def-item-4">ICP<span class="def-item-dt-sep"></span></dt><dd>intracranial pressure<span class="def-item-dd-sep"></span></dd><dt id="def-item-5">IIH<span class="def-item-dt-sep"></span></dt><dd>idiopathic intracranial hypertension</dd></dl>
</div><ul class="copyright-statement"><li class="fn" id="copyright-statement-1">© 2012 by American Journal of Neuroradiology</li></ul><span class="highwire-journal-article-marker-end"></span></div><span class="related-urls"></span></div><a href="http://ajnr-stage2.stage.highwire.org/content/33/10/1901.full" class="hw-link hw-link-article-full-text" data-icon-position="" data-hide-link-title="0">View Full Text</a></div>  </div>

  
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        	<div class="highwire-cite-metadata"><span  class="highwire-cite-metadata-volume highwire-cite-metadata">Vol. 33</span>, <span  class="highwire-cite-metadata-issue highwire-cite-metadata">Issue 10 </span><div  class="highwire-cite-metadata-pubdate highwire-cite-metadata">1 Nov 2012 </div></div>
      
      
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      <div class="highwire-cite-title">Incidence of Cerebellar Tonsillar Ectopia in Idiopathic Intracranial Hypertension: A Mimic of the Chiari I Malformation</div>  
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