MR spectroscopy of the brain in Leigh syndrome
Abstract Brain magnetic resonance spectroscopy in two patients with Leigh syndrome revealed the presence of lactate in gray and white matter brain tissue and relatively high choline levels in the white matter. The latter observation, most probably related to an ongoing demyelination process, underli...
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| Veröffentlicht in: | Brain & development (Tokyo. 1979) 2008-10, Vol.30 (9), p.579-583 |
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| container_title | Brain & development (Tokyo. 1979) |
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| creator | Sijens, P.E Smit, G.P.A Rödiger, L.A van Spronsen, F.J Oudkerk, M Rodenburg, R.J Lunsing, R.J |
| description | Abstract Brain magnetic resonance spectroscopy in two patients with Leigh syndrome revealed the presence of lactate in gray and white matter brain tissue and relatively high choline levels in the white matter. The latter observation, most probably related to an ongoing demyelination process, underlines specific involvement of white matter metabolism in Leigh syndrome even in cases without involvement of the white matter as visualized on MRI. Magnetic resonance spectroscopy might thus be of help in differentiating Leigh syndrome from a range of other mitochondrial diseases, such as ophthalmoplegia and Kearns-Sayre syndrome, showing lack of lactate in brain tissues appearing normal on MRI. |
| doi_str_mv | 10.1016/j.braindev.2008.01.011 |
| format | Article |
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The latter observation, most probably related to an ongoing demyelination process, underlines specific involvement of white matter metabolism in Leigh syndrome even in cases without involvement of the white matter as visualized on MRI. Magnetic resonance spectroscopy might thus be of help in differentiating Leigh syndrome from a range of other mitochondrial diseases, such as ophthalmoplegia and Kearns-Sayre syndrome, showing lack of lactate in brain tissues appearing normal on MRI.</description><identifier>ISSN: 0387-7604</identifier><identifier>EISSN: 1872-7131</identifier><identifier>DOI: 10.1016/j.braindev.2008.01.011</identifier><identifier>PMID: 18329833</identifier><language>eng</language><publisher>Netherlands: Elsevier B.V</publisher><subject>Brain - metabolism ; Brain - pathology ; Choline - metabolism ; Diagnosis, Differential ; Female ; Humans ; Infant ; Kearns-Sayre Syndrome - metabolism ; Kearns-Sayre Syndrome - pathology ; Lactates - metabolism ; Leigh Disease - metabolism ; Leigh Disease - pathology ; Leigh syndrome ; Magnetic resonance spectroscopy ; Magnetic Resonance Spectroscopy - methods ; Male ; Mitochondrial diseases ; Neurology</subject><ispartof>Brain & development (Tokyo. 1979), 2008-10, Vol.30 (9), p.579-583</ispartof><rights>Elsevier B.V.</rights><rights>2008 Elsevier B.V.</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c474t-c594e86e90c9d10038871e71012c24893f41d486a121ed5330935f04fa0d405b3</citedby><cites>FETCH-LOGICAL-c474t-c594e86e90c9d10038871e71012c24893f41d486a121ed5330935f04fa0d405b3</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktohtml>$$Uhttps://dx.doi.org/10.1016/j.braindev.2008.01.011$$EHTML$$P50$$Gelsevier$$H</linktohtml><link.rule.ids>314,777,781,3537,27905,27906,45976</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/18329833$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Sijens, P.E</creatorcontrib><creatorcontrib>Smit, G.P.A</creatorcontrib><creatorcontrib>Rödiger, L.A</creatorcontrib><creatorcontrib>van Spronsen, F.J</creatorcontrib><creatorcontrib>Oudkerk, M</creatorcontrib><creatorcontrib>Rodenburg, R.J</creatorcontrib><creatorcontrib>Lunsing, R.J</creatorcontrib><title>MR spectroscopy of the brain in Leigh syndrome</title><title>Brain & development (Tokyo. 1979)</title><addtitle>Brain Dev</addtitle><description>Abstract Brain magnetic resonance spectroscopy in two patients with Leigh syndrome revealed the presence of lactate in gray and white matter brain tissue and relatively high choline levels in the white matter. The latter observation, most probably related to an ongoing demyelination process, underlines specific involvement of white matter metabolism in Leigh syndrome even in cases without involvement of the white matter as visualized on MRI. Magnetic resonance spectroscopy might thus be of help in differentiating Leigh syndrome from a range of other mitochondrial diseases, such as ophthalmoplegia and Kearns-Sayre syndrome, showing lack of lactate in brain tissues appearing normal on MRI.</description><subject>Brain - metabolism</subject><subject>Brain - pathology</subject><subject>Choline - metabolism</subject><subject>Diagnosis, Differential</subject><subject>Female</subject><subject>Humans</subject><subject>Infant</subject><subject>Kearns-Sayre Syndrome - metabolism</subject><subject>Kearns-Sayre Syndrome - pathology</subject><subject>Lactates - metabolism</subject><subject>Leigh Disease - metabolism</subject><subject>Leigh Disease - pathology</subject><subject>Leigh syndrome</subject><subject>Magnetic resonance spectroscopy</subject><subject>Magnetic Resonance Spectroscopy - methods</subject><subject>Male</subject><subject>Mitochondrial diseases</subject><subject>Neurology</subject><issn>0387-7604</issn><issn>1872-7131</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2008</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNqFUctOwzAQtBCIlscvoJy4JezabuJcEAjxkoqQeJyt1N5QlzQpdorUv8elRUhckEbay-zs7AxjJwgZAuZns2ziK9da-sw4gMoAI3CHDVEVPC1Q4C4bglBFWuQgB-wghBkAIEfYZwNUgpdKiCHLHp6SsCDT-y6YbrFKujrpp5R8qycRY3Jv0ySsWuu7OR2xvbpqAh1v5yF7vbl-ubpLx4-391eX49TIQvapGZWSVE4lmNIiRB-qQCqic264VKWoJVqp8ir6ITsSAkoxqkHWFVgJo4k4ZKcb3YXvPpYUej13wVDTVC11y6DzUuaKK4jEfEM08YHgqdYL7-aVX2kEvU5Kz_RPUnqdlAaMwLh4sr2wnMzJ_q5to4mEiw2B4p-fjrwOxlFryDof89K2c__fOP8jYRrXOlM177SiMOuWvo0patSBa9DP677WdYGKVQng4gvKc49R</recordid><startdate>20081001</startdate><enddate>20081001</enddate><creator>Sijens, P.E</creator><creator>Smit, G.P.A</creator><creator>Rödiger, L.A</creator><creator>van Spronsen, F.J</creator><creator>Oudkerk, M</creator><creator>Rodenburg, R.J</creator><creator>Lunsing, R.J</creator><general>Elsevier B.V</general><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7X8</scope><scope>8BM</scope></search><sort><creationdate>20081001</creationdate><title>MR spectroscopy of the brain in Leigh syndrome</title><author>Sijens, P.E ; Smit, G.P.A ; Rödiger, L.A ; van Spronsen, F.J ; Oudkerk, M ; Rodenburg, R.J ; Lunsing, R.J</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c474t-c594e86e90c9d10038871e71012c24893f41d486a121ed5330935f04fa0d405b3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2008</creationdate><topic>Brain - metabolism</topic><topic>Brain - pathology</topic><topic>Choline - metabolism</topic><topic>Diagnosis, Differential</topic><topic>Female</topic><topic>Humans</topic><topic>Infant</topic><topic>Kearns-Sayre Syndrome - metabolism</topic><topic>Kearns-Sayre Syndrome - pathology</topic><topic>Lactates - metabolism</topic><topic>Leigh Disease - metabolism</topic><topic>Leigh Disease - pathology</topic><topic>Leigh syndrome</topic><topic>Magnetic resonance spectroscopy</topic><topic>Magnetic Resonance Spectroscopy - methods</topic><topic>Male</topic><topic>Mitochondrial diseases</topic><topic>Neurology</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Sijens, P.E</creatorcontrib><creatorcontrib>Smit, G.P.A</creatorcontrib><creatorcontrib>Rödiger, L.A</creatorcontrib><creatorcontrib>van Spronsen, F.J</creatorcontrib><creatorcontrib>Oudkerk, M</creatorcontrib><creatorcontrib>Rodenburg, R.J</creatorcontrib><creatorcontrib>Lunsing, R.J</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><collection>ComDisDome</collection><jtitle>Brain & development (Tokyo. 1979)</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Sijens, P.E</au><au>Smit, G.P.A</au><au>Rödiger, L.A</au><au>van Spronsen, F.J</au><au>Oudkerk, M</au><au>Rodenburg, R.J</au><au>Lunsing, R.J</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>MR spectroscopy of the brain in Leigh syndrome</atitle><jtitle>Brain & development (Tokyo. 1979)</jtitle><addtitle>Brain Dev</addtitle><date>2008-10-01</date><risdate>2008</risdate><volume>30</volume><issue>9</issue><spage>579</spage><epage>583</epage><pages>579-583</pages><issn>0387-7604</issn><eissn>1872-7131</eissn><abstract>Abstract Brain magnetic resonance spectroscopy in two patients with Leigh syndrome revealed the presence of lactate in gray and white matter brain tissue and relatively high choline levels in the white matter. The latter observation, most probably related to an ongoing demyelination process, underlines specific involvement of white matter metabolism in Leigh syndrome even in cases without involvement of the white matter as visualized on MRI. Magnetic resonance spectroscopy might thus be of help in differentiating Leigh syndrome from a range of other mitochondrial diseases, such as ophthalmoplegia and Kearns-Sayre syndrome, showing lack of lactate in brain tissues appearing normal on MRI.</abstract><cop>Netherlands</cop><pub>Elsevier B.V</pub><pmid>18329833</pmid><doi>10.1016/j.braindev.2008.01.011</doi><tpages>5</tpages></addata></record> |
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| ispartof | Brain & development (Tokyo. 1979), 2008-10, Vol.30 (9), p.579-583 |
| issn | 0387-7604 1872-7131 |
| language | eng |
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| source | MEDLINE; Elsevier ScienceDirect Journals |
| subjects | Brain - metabolism Brain - pathology Choline - metabolism Diagnosis, Differential Female Humans Infant Kearns-Sayre Syndrome - metabolism Kearns-Sayre Syndrome - pathology Lactates - metabolism Leigh Disease - metabolism Leigh Disease - pathology Leigh syndrome Magnetic resonance spectroscopy Magnetic Resonance Spectroscopy - methods Male Mitochondrial diseases Neurology |
| title | MR spectroscopy of the brain in Leigh syndrome |
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