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<TEI>
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<fileDesc>
<titleStmt>
<title xml:lang="en">Pontine Tegmental Cap Dysplasia: MR Imaging and Diffusion Tensor Imaging Features of Impaired Axonal Navigation</title>
<author>
<name sortKey="Jissendi Tchofo, P" sort="Jissendi Tchofo, P" uniqKey="Jissendi Tchofo P" first="P." last="Jissendi-Tchofo">P. Jissendi-Tchofo</name>
</author>
<author>
<name sortKey="Doherty, D" sort="Doherty, D" uniqKey="Doherty D" first="D." last="Doherty">D. Doherty</name>
</author>
<author>
<name sortKey="Mcgillivray, G" sort="Mcgillivray, G" uniqKey="Mcgillivray G" first="G." last="Mcgillivray">G. Mcgillivray</name>
</author>
<author>
<name sortKey="Hevner, R" sort="Hevner, R" uniqKey="Hevner R" first="R." last="Hevner">R. Hevner</name>
</author>
<author>
<name sortKey="Shaw, D" sort="Shaw, D" uniqKey="Shaw D" first="D." last="Shaw">D. Shaw</name>
</author>
<author>
<name sortKey="Ishak, G" sort="Ishak, G" uniqKey="Ishak G" first="G." last="Ishak">G. Ishak</name>
</author>
<author>
<name sortKey="Leventer, R" sort="Leventer, R" uniqKey="Leventer R" first="R." last="Leventer">R. Leventer</name>
</author>
<author>
<name sortKey="Barkovich, A J" sort="Barkovich, A J" uniqKey="Barkovich A" first="A. J." last="Barkovich">A. J. Barkovich</name>
</author>
</titleStmt>
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<idno type="wicri:source">PMC</idno>
<idno type="pmid">18842761</idno>
<idno type="pmc">3919876</idno>
<idno type="url">http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3919876</idno>
<idno type="RBID">PMC:3919876</idno>
<idno type="doi">10.3174/ajnr.A1305</idno>
<date when="2008">2008</date>
<idno type="wicri:Area/Pmc/Corpus">001F88</idno>
<idno type="wicri:explorRef" wicri:stream="Pmc" wicri:step="Corpus" wicri:corpus="PMC">001F88</idno>
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<biblStruct>
<analytic>
<title xml:lang="en" level="a" type="main">Pontine Tegmental Cap Dysplasia: MR Imaging and Diffusion Tensor Imaging Features of Impaired Axonal Navigation</title>
<author>
<name sortKey="Jissendi Tchofo, P" sort="Jissendi Tchofo, P" uniqKey="Jissendi Tchofo P" first="P." last="Jissendi-Tchofo">P. Jissendi-Tchofo</name>
</author>
<author>
<name sortKey="Doherty, D" sort="Doherty, D" uniqKey="Doherty D" first="D." last="Doherty">D. Doherty</name>
</author>
<author>
<name sortKey="Mcgillivray, G" sort="Mcgillivray, G" uniqKey="Mcgillivray G" first="G." last="Mcgillivray">G. Mcgillivray</name>
</author>
<author>
<name sortKey="Hevner, R" sort="Hevner, R" uniqKey="Hevner R" first="R." last="Hevner">R. Hevner</name>
</author>
<author>
<name sortKey="Shaw, D" sort="Shaw, D" uniqKey="Shaw D" first="D." last="Shaw">D. Shaw</name>
</author>
<author>
<name sortKey="Ishak, G" sort="Ishak, G" uniqKey="Ishak G" first="G." last="Ishak">G. Ishak</name>
</author>
<author>
<name sortKey="Leventer, R" sort="Leventer, R" uniqKey="Leventer R" first="R." last="Leventer">R. Leventer</name>
</author>
<author>
<name sortKey="Barkovich, A J" sort="Barkovich, A J" uniqKey="Barkovich A" first="A. J." last="Barkovich">A. J. Barkovich</name>
</author>
</analytic>
<series>
<title level="j">AJNR. American journal of neuroradiology</title>
<idno type="ISSN">0195-6108</idno>
<idno type="eISSN">1936-959X</idno>
<imprint>
<date when="2008">2008</date>
</imprint>
</series>
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<textClass></textClass>
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</teiHeader>
<front>
<div type="abstract" xml:lang="en">
<sec id="S1">
<title>BACKGROUND AND PURPOSE</title>
<p id="P1">Malformations of the brain stem are uncommon. We present MR imaging and diffusion tensor imaging (DTI) features of 6 patients with pontine tegmental cap dysplasia, characterized by ventral pontine hypoplasia and a dorsal “bump,” and speculate on potential mechanisms by which it forms.</p>
</sec>
<sec id="S2">
<title>MATERIALS AND METHODS</title>
<p id="P2">Birth and developmental records of 6 patients were reviewed. We reviewed MR imaging studies of all patients and DTIs of patient 3. Potential developmental causes were evaluated.</p>
</sec>
<sec id="S3">
<title>RESULTS</title>
<p id="P3">All patients were born uneventfully after normal pregnancies except patient 6 (in utero growth retardation). They presented with multiple cranial neuropathies and evidence of cerebellar dysfunction. Variable hypotonia and motor dysfunction were present. Imaging revealed ventral pontine hypoplasia and mild cerebellar vermian hypoplasia, in addition to an unusual rounded to beaklike “bump” on the dorsal surface of the pons, extending into the fourth ventricle. Color fractional anisotropy maps showed the bump to consist of a bundle of axons directed horizontally (left-right). The bump appeared, on morphologic images, to be continuous with the middle cerebellar peduncles (MCPs), which were slightly diminished in size compared with those in healthy infants. Analysis of the DTI was, however, inconclusive regarding the connections of these axons. The decussation of the MCPs, transverse pontine fibers, and longitudinal brain stem axonal pathways was also abnormal.</p>
</sec>
<sec id="S4">
<title>CONCLUSIONS</title>
<p id="P4">Our data suggest that the dorsal transverse axonal band in these disorders results from abnormal axonal pathfinding, abnormal neuronal migration, or a combination of the 2 processes.</p>
</sec>
</div>
</front>
</TEI>
<pmc article-type="research-article">
<pmc-comment>The publisher of this article does not allow downloading of the full text in XML form.</pmc-comment>
<pmc-dir>properties manuscript</pmc-dir>
<front>
<journal-meta>
<journal-id journal-id-type="nlm-journal-id">8003708</journal-id>
<journal-id journal-id-type="pubmed-jr-id">379</journal-id>
<journal-id journal-id-type="nlm-ta">AJNR Am J Neuroradiol</journal-id>
<journal-id journal-id-type="iso-abbrev">AJNR Am J Neuroradiol</journal-id>
<journal-title-group>
<journal-title>AJNR. American journal of neuroradiology</journal-title>
</journal-title-group>
<issn pub-type="ppub">0195-6108</issn>
<issn pub-type="epub">1936-959X</issn>
</journal-meta>
<article-meta>
<article-id pub-id-type="pmid">18842761</article-id>
<article-id pub-id-type="pmc">3919876</article-id>
<article-id pub-id-type="doi">10.3174/ajnr.A1305</article-id>
<article-id pub-id-type="manuscript">NIHMS267890</article-id>
<article-categories>
<subj-group subj-group-type="heading">
<subject>Article</subject>
</subj-group>
</article-categories>
<title-group>
<article-title>Pontine Tegmental Cap Dysplasia: MR Imaging and Diffusion Tensor Imaging Features of Impaired Axonal Navigation</article-title>
</title-group>
<contrib-group>
<contrib contrib-type="author">
<name>
<surname>Jissendi-Tchofo</surname>
<given-names>P.</given-names>
</name>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Doherty</surname>
<given-names>D.</given-names>
</name>
</contrib>
<contrib contrib-type="author">
<name>
<surname>McGillivray</surname>
<given-names>G.</given-names>
</name>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Hevner</surname>
<given-names>R.</given-names>
</name>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Shaw</surname>
<given-names>D.</given-names>
</name>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Ishak</surname>
<given-names>G.</given-names>
</name>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Leventer</surname>
<given-names>R.</given-names>
</name>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Barkovich</surname>
<given-names>A.J.</given-names>
</name>
</contrib>
<aff id="A1">Neuroradiology section (P.J.-T., A.J.B.), Department of Radiology, University of California, San Francisco, Calif; Service de Neuroradiologie (P.J.-T.), Hôpital Salengro, CHRU Lille, Lille, France; Neuroradiology section (P.J.-T.), Department of Radiology, Erasme Hospital, Brussels, Belgium; Division of Genetics and Developmental Medicine (D.D.), University of Washington, Children's Hospital and Medical Center, Seattle, Wash; Genetic Health Services (G.M.), Murdoch Children's Research Institute, Royal Children's Hospital, Melbourne, Australia; Neurological Surgery/Neuropathology (R.H.), University of Washington School of Medicine, Seattle Children's Hospital Research Institute, Seattle, Wash; Department of Radiology (D.S., G.I.), Children's Hospital and Medical Center, Seattle, Wash; and Department of Child Neurology (R.L.), Royal Children's Hospital, Parkville, Australia</aff>
</contrib-group>
<author-notes>
<corresp id="CR1">Please address correspondence to Patrice Jissendi, MD, Radiology Department, Erasme Hospital, Route de Lennik 808, B-1070 Brussels, Belgium;
<email>jissendi@gmail.com</email>
</corresp>
</author-notes>
<pub-date pub-type="nihms-submitted">
<day>14</day>
<month>3</month>
<year>2011</year>
</pub-date>
<pub-date pub-type="epub">
<day>08</day>
<month>10</month>
<year>2008</year>
</pub-date>
<pub-date pub-type="ppub">
<month>1</month>
<year>2009</year>
</pub-date>
<pub-date pub-type="pmc-release">
<day>10</day>
<month>2</month>
<year>2014</year>
</pub-date>
<volume>30</volume>
<issue>1</issue>
<fpage>113</fpage>
<lpage>119</lpage>
<pmc-comment>elocation-id from pubmed: 10.3174/ajnr.A1305</pmc-comment>
<abstract>
<sec id="S1">
<title>BACKGROUND AND PURPOSE</title>
<p id="P1">Malformations of the brain stem are uncommon. We present MR imaging and diffusion tensor imaging (DTI) features of 6 patients with pontine tegmental cap dysplasia, characterized by ventral pontine hypoplasia and a dorsal “bump,” and speculate on potential mechanisms by which it forms.</p>
</sec>
<sec id="S2">
<title>MATERIALS AND METHODS</title>
<p id="P2">Birth and developmental records of 6 patients were reviewed. We reviewed MR imaging studies of all patients and DTIs of patient 3. Potential developmental causes were evaluated.</p>
</sec>
<sec id="S3">
<title>RESULTS</title>
<p id="P3">All patients were born uneventfully after normal pregnancies except patient 6 (in utero growth retardation). They presented with multiple cranial neuropathies and evidence of cerebellar dysfunction. Variable hypotonia and motor dysfunction were present. Imaging revealed ventral pontine hypoplasia and mild cerebellar vermian hypoplasia, in addition to an unusual rounded to beaklike “bump” on the dorsal surface of the pons, extending into the fourth ventricle. Color fractional anisotropy maps showed the bump to consist of a bundle of axons directed horizontally (left-right). The bump appeared, on morphologic images, to be continuous with the middle cerebellar peduncles (MCPs), which were slightly diminished in size compared with those in healthy infants. Analysis of the DTI was, however, inconclusive regarding the connections of these axons. The decussation of the MCPs, transverse pontine fibers, and longitudinal brain stem axonal pathways was also abnormal.</p>
</sec>
<sec id="S4">
<title>CONCLUSIONS</title>
<p id="P4">Our data suggest that the dorsal transverse axonal band in these disorders results from abnormal axonal pathfinding, abnormal neuronal migration, or a combination of the 2 processes.</p>
</sec>
</abstract>
<funding-group>
<award-group>
<funding-source country="United States">National Center for Research Resources : NCRR</funding-source>
<award-id>KL2 RR025015-02 || RR</award-id>
</award-group>
</funding-group>
</article-meta>
</front>
</pmc>
</record>

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