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<meta name="DC.Title" content="CT and MR of the brain in disorders of the propionate and methylmalonate metabolism." />
<meta name="DC.Date" content="1994-09-01" />
<meta name="DC.Publisher" content="American Journal of Neuroradiology" />
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<meta name="DC.Description" content="PURPOSE To present the CT and MR findings in children with propionic and methylmalonic acidemia.

METHODS Twenty-three new patients with methylmalonic and 20 with propionic acidemia were examined with CT and/or MR of the brain. In total 52 CT and 55 MR studies were done. Twenty-six previously published cases were also reviewed.

RESULTS The findings were similar in the two syndromes. During the first month of life the examinations were either normal or showed white matter attenuation. Later during the first year moderate or even severe widening of sulci and fissures was seen, especially in infants with propionic acidemia. During therapy, these changes often resolved, especially in the patients with methylmalonic acidemia. Mild to moderate delay in myelination was also a common finding in both disorders. Basal ganglia changes, predominately in the globus pallidus, were seen in five patients with methylmalonic acidemia and in two children with propionic acidemia; in two patients these changes were transient.

CONCLUSION Children who have methylmalonic or propionic acidemia, in addition to widening of cerebrospinal fluid spaces and some delay in myelination, also often show symmetric involvement of the basal ganglia." />
<meta name="DC.Contributor" content="J Brismar" />
<meta name="DC.Contributor" content="P T Ozand" />
<meta name="article:published_time" content="1994-09-01" />
<meta name="citation_title" content="CT and MR of the brain in disorders of the propionate and methylmalonate metabolism." />
<meta name="citation_abstract" lang="en" content="&lt;h3&gt;PURPOSE&lt;/h3&gt;
&lt;p&gt;To present the CT and MR findings in children with propionic and methylmalonic acidemia.&lt;/p&gt;&lt;h3&gt;METHODS&lt;/h3&gt;
&lt;p&gt;Twenty-three new patients with methylmalonic and 20 with propionic acidemia were examined with CT and/or MR of the brain. In total 52 CT and 55 MR studies were done. Twenty-six previously published cases were also reviewed.&lt;/p&gt;&lt;h3&gt;RESULTS&lt;/h3&gt;
&lt;p&gt;The findings were similar in the two syndromes. During the first month of life the examinations were either normal or showed white matter attenuation. Later during the first year moderate or even severe widening of sulci and fissures was seen, especially in infants with propionic acidemia. During therapy, these changes often resolved, especially in the patients with methylmalonic acidemia. Mild to moderate delay in myelination was also a common finding in both disorders. Basal ganglia changes, predominately in the globus pallidus, were seen in five patients with methylmalonic acidemia and in two children with propionic acidemia; in two patients these changes were transient.&lt;/p&gt;&lt;h3&gt;CONCLUSION&lt;/h3&gt;
&lt;p&gt;Children who have methylmalonic or propionic acidemia, in addition to widening of cerebrospinal fluid spaces and some delay in myelination, also often show symmetric involvement of the basal ganglia.&lt;/p&gt;" />
<meta name="citation_journal_title" content="American Journal of Neuroradiology" />
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<meta name="citation_author" content="J Brismar" />
<meta name="citation_author_institution" content="Department of Diagnostic Radiology, King Faisal Specialist Hospital and Research Centre, Riyadh, Saudi Arabia." />
<meta name="citation_author" content="P T Ozand" />
<meta name="citation_author_institution" content="Department of Diagnostic Radiology, King Faisal Specialist Hospital and Research Centre, Riyadh, Saudi Arabia." />
<meta name="twitter:title" content="CT and MR of the brain in disorders of the propionate and methylmalonate metabolism." />
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<meta name="twitter:description" content="PURPOSE To present the CT and MR findings in children with propionic and methylmalonic acidemia.

METHODS Twenty-three new patients with methylmalonic and 20 with propionic acidemia were examined with CT and/or MR of the brain. In total 52 CT and 55 MR studies were done. Twenty-six previously published cases were also reviewed.

RESULTS The findings were similar in the two syndromes. During the first month of life the examinations were either normal or showed white matter attenuation. Later during the first year moderate or even severe widening of sulci and fissures was seen, especially in infants with propionic acidemia. During therapy, these changes often resolved, especially in the patients with methylmalonic acidemia. Mild to moderate delay in myelination was also a common finding in both disorders. Basal ganglia changes, predominately in the globus pallidus, were seen in five patients with methylmalonic acidemia and in two children with propionic acidemia; in two patients these changes were transient.

CONCLUSION Children who have methylmalonic or propionic acidemia, in addition to widening of cerebrospinal fluid spaces and some delay in myelination, also often show symmetric involvement of the basal ganglia." />
<meta name="og-title" property="og:title" content="CT and MR of the brain in disorders of the propionate and methylmalonate metabolism." />
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<meta name="og-description" property="og:description" content="PURPOSE To present the CT and MR findings in children with propionic and methylmalonic acidemia.

METHODS Twenty-three new patients with methylmalonic and 20 with propionic acidemia were examined with CT and/or MR of the brain. In total 52 CT and 55 MR studies were done. Twenty-six previously published cases were also reviewed.

RESULTS The findings were similar in the two syndromes. During the first month of life the examinations were either normal or showed white matter attenuation. Later during the first year moderate or even severe widening of sulci and fissures was seen, especially in infants with propionic acidemia. During therapy, these changes often resolved, especially in the patients with methylmalonic acidemia. Mild to moderate delay in myelination was also a common finding in both disorders. Basal ganglia changes, predominately in the globus pallidus, were seen in five patients with methylmalonic acidemia and in two children with propionic acidemia; in two patients these changes were transient.

CONCLUSION Children who have methylmalonic or propionic acidemia, in addition to widening of cerebrospinal fluid spaces and some delay in myelination, also often show symmetric involvement of the basal ganglia." />
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      <h1 class="highwire-cite-title" id="page-title">CT and MR of the brain in disorders of the propionate and methylmalonate metabolism.</h1>  
    	<div class="highwire-cite-authors"><span  class="highwire-citation-authors"><span class="highwire-citation-author first" data-delta="0">J Brismar</span> and <span class="highwire-citation-author" data-delta="1">P T Ozand</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 1994,  </span><span  class="highwire-cite-metadata-volume highwire-cite-metadata">15 </span><span  class="highwire-cite-metadata-issue highwire-cite-metadata">(8) </span><span  class="highwire-cite-metadata-pages highwire-cite-metadata">1459-1473; </span></div>
  
  
  
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<div id="hw-article-author-popups-top-node-6130--6723659959" style="display: none;"><div class="author-tooltip-0"><div class="author-tooltip-name">J Brismar </div><div class="author-tooltip-affiliation"><span class="author-tooltip-text"><div class='author-affiliation'>Department of Diagnostic Radiology, King Faisal Specialist Hospital and Research Centre, Riyadh, Saudi Arabia.</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%2BBrismar%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=Brismar%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%2BBrismar%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">P T Ozand </div><div class="author-tooltip-affiliation"><span class="author-tooltip-text"><div class='author-affiliation'>Department of Diagnostic Radiology, King Faisal Specialist Hospital and Research Centre, Riyadh, Saudi Arabia.</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%2BT%2BOzand%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=Ozand%20PT&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%2BT%2BOzand%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>PURPOSE</strong> To present the CT and MR findings in children with propionic and methylmalonic acidemia.</p>
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          <p id="p-2"><strong>METHODS</strong> Twenty-three new patients with methylmalonic and 20 with propionic acidemia were examined with CT and/or MR of the brain. In total 52 CT and 55 MR studies were done. Twenty-six previously published cases were also reviewed.</p>
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          <p id="p-3"><strong>RESULTS</strong> The findings were similar in the two syndromes. During the first month of life the examinations were either normal or showed white matter attenuation. Later during the first year moderate or even severe widening of sulci and fissures was seen, especially in infants with propionic acidemia. During therapy, these changes often resolved, especially in the patients with methylmalonic acidemia. Mild to moderate delay in myelination was also a common finding in both disorders. Basal ganglia changes, predominately in the globus pallidus, were seen in five patients with methylmalonic acidemia and in two children with propionic acidemia; in two patients these changes were transient.</p>
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          <p id="p-4"><strong>CONCLUSION</strong> Children who have methylmalonic or propionic acidemia, in addition to widening of cerebrospinal fluid spaces and some delay in myelination, also often show symmetric involvement of the basal ganglia.</p>
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        	<div class="highwire-cite-metadata"><span  class="highwire-cite-metadata-volume highwire-cite-metadata">Vol. 15</span>, <span  class="highwire-cite-metadata-issue highwire-cite-metadata">Issue 8 </span><div  class="highwire-cite-metadata-pubdate highwire-cite-metadata">1 Sep 1994 </div></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">J</span> <span class="nlm-surname">Brismar</span></span>, <span class="highwire-citation-author" data-delta="1"><span class="nlm-given-names">P T</span> <span class="nlm-surname">Ozand</span></span></span></div>
  
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      <div class="highwire-cite-title">CT and MR of the brain in disorders of the propionate and methylmalonate metabolism.</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">J</span> <span class="nlm-surname">Brismar</span></span>, <span class="highwire-citation-author" data-delta="1"><span class="nlm-given-names">P T</span> <span class="nlm-surname">Ozand</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 1994, </span><span  class="highwire-cite-metadata-volume highwire-cite-metadata">15 </span><span  class="highwire-cite-metadata-issue highwire-cite-metadata">(8) </span><span  class="highwire-cite-metadata-pages highwire-cite-metadata">1459-1473; </span></div>
  
  
  
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