Brain metabolic impairment in non-cerebral and cerebral forms of x-linked adrenoleukodystrophy by proton MRS: Identification of metabolic patterns by discriminant analysis
Cerebral metabolism in six children with X‐linked adrenoleukodystrophy (X‐ALD) was studied using 1H magnetic resonance spectroscopy (MRS), and the status of the patients was monitored for evaluating disease progression. Spectra were abnormal even in patients with no cerebral impairment. Four differe...
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Veröffentlicht in: | Magnetic resonance in medicine 1999-06, Vol.41 (6), p.1119-1126 |
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description | Cerebral metabolism in six children with X‐linked adrenoleukodystrophy (X‐ALD) was studied using 1H magnetic resonance spectroscopy (MRS), and the status of the patients was monitored for evaluating disease progression. Spectra were abnormal even in patients with no cerebral impairment. Four different metabolic patterns were identified, and a metabolic classification of the disease was proposed, from grade 0 to grade III. The evolution of the disease toward grade II appears to be systematic, but many patients did not evolve from this grade to grade III, which is the metabolic mark of severe progressive forms. Metabolic data of X‐ALD were processed using discriminant analysis, which provides a classification accuracy of 95.2%. Proton cerebral MRS together with discriminant analysis may be useful during the follow‐up in X‐ALD for monitoring the evolution of the disease and the effects of therapy. Magn Reson Med 41:1119–1126, 1999. © 1999 Wiley‐Liss, Inc. |
doi_str_mv | 10.1002/(SICI)1522-2594(199906)41:6<1119::AID-MRM7>3.0.CO;2-F |
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Spectra were abnormal even in patients with no cerebral impairment. Four different metabolic patterns were identified, and a metabolic classification of the disease was proposed, from grade 0 to grade III. The evolution of the disease toward grade II appears to be systematic, but many patients did not evolve from this grade to grade III, which is the metabolic mark of severe progressive forms. Metabolic data of X‐ALD were processed using discriminant analysis, which provides a classification accuracy of 95.2%. Proton cerebral MRS together with discriminant analysis may be useful during the follow‐up in X‐ALD for monitoring the evolution of the disease and the effects of therapy. 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Glycogenosis ; Case-Control Studies ; Child ; Child, Preschool ; Discriminant Analysis ; Errors of metabolism ; Genetic Linkage ; Humans ; Magnetic Resonance Spectroscopy ; Male ; Medical sciences ; Metabolic diseases ; proton magnetic resonance spectroscopy ; X Chromosome ; X-ALD</subject><ispartof>Magnetic resonance in medicine, 1999-06, Vol.41 (6), p.1119-1126</ispartof><rights>Copyright © 1999 Wiley‐Liss, Inc.</rights><rights>1999 INIST-CNRS</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><cites>FETCH-LOGICAL-c5077-91917a4ec161c2e9334e3783c9cdf4bec4fc18a3e6d39999367a917387714f03</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://onlinelibrary.wiley.com/doi/pdf/10.1002%2F%28SICI%291522-2594%28199906%2941%3A6%3C1119%3A%3AAID-MRM7%3E3.0.CO%3B2-F$$EPDF$$P50$$Gwiley$$H</linktopdf><linktohtml>$$Uhttps://onlinelibrary.wiley.com/doi/full/10.1002%2F%28SICI%291522-2594%28199906%2941%3A6%3C1119%3A%3AAID-MRM7%3E3.0.CO%3B2-F$$EHTML$$P50$$Gwiley$$H</linktohtml><link.rule.ids>314,780,784,1417,1433,27924,27925,45574,45575,46409,46833</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=1844973$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/10371443$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Salvan, Anne-Marie</creatorcontrib><creatorcontrib>Confort-Gouny, Sylviane</creatorcontrib><creatorcontrib>Chabrol, Brigitte</creatorcontrib><creatorcontrib>Cozzone, Patrick J.</creatorcontrib><creatorcontrib>Vion-Dury, Jean</creatorcontrib><title>Brain metabolic impairment in non-cerebral and cerebral forms of x-linked adrenoleukodystrophy by proton MRS: Identification of metabolic patterns by discriminant analysis</title><title>Magnetic resonance in medicine</title><addtitle>Magn. Reson. Med</addtitle><description>Cerebral metabolism in six children with X‐linked adrenoleukodystrophy (X‐ALD) was studied using 1H magnetic resonance spectroscopy (MRS), and the status of the patients was monitored for evaluating disease progression. Spectra were abnormal even in patients with no cerebral impairment. Four different metabolic patterns were identified, and a metabolic classification of the disease was proposed, from grade 0 to grade III. The evolution of the disease toward grade II appears to be systematic, but many patients did not evolve from this grade to grade III, which is the metabolic mark of severe progressive forms. Metabolic data of X‐ALD were processed using discriminant analysis, which provides a classification accuracy of 95.2%. Proton cerebral MRS together with discriminant analysis may be useful during the follow‐up in X‐ALD for monitoring the evolution of the disease and the effects of therapy. Magn Reson Med 41:1119–1126, 1999. © 1999 Wiley‐Liss, Inc.</description><subject>Adolescent</subject><subject>Adrenoleukodystrophy - diagnosis</subject><subject>Adrenoleukodystrophy - genetics</subject><subject>Adrenoleukodystrophy - metabolism</subject><subject>Biological and medical sciences</subject><subject>Brain - metabolism</subject><subject>brain metabolic grading</subject><subject>Carbohydrates (enzymatic deficiencies). Glycogenosis</subject><subject>Case-Control Studies</subject><subject>Child</subject><subject>Child, Preschool</subject><subject>Discriminant Analysis</subject><subject>Errors of metabolism</subject><subject>Genetic Linkage</subject><subject>Humans</subject><subject>Magnetic Resonance Spectroscopy</subject><subject>Male</subject><subject>Medical sciences</subject><subject>Metabolic diseases</subject><subject>proton magnetic resonance spectroscopy</subject><subject>X Chromosome</subject><subject>X-ALD</subject><issn>0740-3194</issn><issn>1522-2594</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>1999</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNqFkd1u0zAYhiMEYmVwC8gHCG0HKXbsxHEHSFugo9K6St0Qh59cxxGmiVPsVCzXxE3i0KpFAokj_-j9Xj_yE0XvCB4TjJM3Z3ezYnZO0iSJk1SwMyKEwNk5I5PsLSFETCaXsw_xfDnn7-kYj4vFRRJPH0Wjw8TjaIQ5wzElgp1Ez7z_hjEWgrOn0QnBlBPG6Cj6eeWksajRnVy1tVHINBtpXKNth8K9bW2stNMrJ2skbYkOh6p1jUdthR7i2ti1LpEsnbZtrbfrtux959rN1x6terRxbddaNF_eTdCsDMWmMkp2JtyF8ePLG9l12lk_zJTGK2caY2XgkFbWvTf-efSkkrXXL_braXQ__XhffIpvFtez4vImVinmPBZEEC6ZViQjKtGCUqYpz6kSqqzYSitWKZJLqrOShk8VNOMyTNCchz-pMD2NXu9qA_j3rfYdNIFG17W0ut16yESe4JynIfh5F1Su9d7pCjaBWboeCIZBIsAgEQYlMCiBnURgBDIYJAIEiTBIBAoYigUkMA29L_cA21Wjyz9ad9ZC4NU-IL2SdeWkVcYfczljgtMj3w9T6_4vuP-x_QPt9zn0xrte4zv9cOiVbg0ZpzyFL7fXcEuvOCHpMmx-AaYv1qI</recordid><startdate>199906</startdate><enddate>199906</enddate><creator>Salvan, Anne-Marie</creator><creator>Confort-Gouny, Sylviane</creator><creator>Chabrol, Brigitte</creator><creator>Cozzone, Patrick J.</creator><creator>Vion-Dury, Jean</creator><general>John Wiley & Sons, Inc</general><general>Williams & Wilkins</general><scope>BSCLL</scope><scope>IQODW</scope><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></search><sort><creationdate>199906</creationdate><title>Brain metabolic impairment in non-cerebral and cerebral forms of x-linked adrenoleukodystrophy by proton MRS: Identification of metabolic patterns by discriminant analysis</title><author>Salvan, Anne-Marie ; Confort-Gouny, Sylviane ; Chabrol, Brigitte ; Cozzone, Patrick J. ; Vion-Dury, Jean</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c5077-91917a4ec161c2e9334e3783c9cdf4bec4fc18a3e6d39999367a917387714f03</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>1999</creationdate><topic>Adolescent</topic><topic>Adrenoleukodystrophy - diagnosis</topic><topic>Adrenoleukodystrophy - genetics</topic><topic>Adrenoleukodystrophy - metabolism</topic><topic>Biological and medical sciences</topic><topic>Brain - metabolism</topic><topic>brain metabolic grading</topic><topic>Carbohydrates (enzymatic deficiencies). Glycogenosis</topic><topic>Case-Control Studies</topic><topic>Child</topic><topic>Child, Preschool</topic><topic>Discriminant Analysis</topic><topic>Errors of metabolism</topic><topic>Genetic Linkage</topic><topic>Humans</topic><topic>Magnetic Resonance Spectroscopy</topic><topic>Male</topic><topic>Medical sciences</topic><topic>Metabolic diseases</topic><topic>proton magnetic resonance spectroscopy</topic><topic>X Chromosome</topic><topic>X-ALD</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Salvan, Anne-Marie</creatorcontrib><creatorcontrib>Confort-Gouny, Sylviane</creatorcontrib><creatorcontrib>Chabrol, Brigitte</creatorcontrib><creatorcontrib>Cozzone, Patrick J.</creatorcontrib><creatorcontrib>Vion-Dury, Jean</creatorcontrib><collection>Istex</collection><collection>Pascal-Francis</collection><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><jtitle>Magnetic resonance in medicine</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Salvan, Anne-Marie</au><au>Confort-Gouny, Sylviane</au><au>Chabrol, Brigitte</au><au>Cozzone, Patrick J.</au><au>Vion-Dury, Jean</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Brain metabolic impairment in non-cerebral and cerebral forms of x-linked adrenoleukodystrophy by proton MRS: Identification of metabolic patterns by discriminant analysis</atitle><jtitle>Magnetic resonance in medicine</jtitle><addtitle>Magn. Reson. Med</addtitle><date>1999-06</date><risdate>1999</risdate><volume>41</volume><issue>6</issue><spage>1119</spage><epage>1126</epage><pages>1119-1126</pages><issn>0740-3194</issn><eissn>1522-2594</eissn><coden>MRMEEN</coden><abstract>Cerebral metabolism in six children with X‐linked adrenoleukodystrophy (X‐ALD) was studied using 1H magnetic resonance spectroscopy (MRS), and the status of the patients was monitored for evaluating disease progression. Spectra were abnormal even in patients with no cerebral impairment. Four different metabolic patterns were identified, and a metabolic classification of the disease was proposed, from grade 0 to grade III. The evolution of the disease toward grade II appears to be systematic, but many patients did not evolve from this grade to grade III, which is the metabolic mark of severe progressive forms. Metabolic data of X‐ALD were processed using discriminant analysis, which provides a classification accuracy of 95.2%. Proton cerebral MRS together with discriminant analysis may be useful during the follow‐up in X‐ALD for monitoring the evolution of the disease and the effects of therapy. Magn Reson Med 41:1119–1126, 1999. © 1999 Wiley‐Liss, Inc.</abstract><cop>New York</cop><pub>John Wiley & Sons, Inc</pub><pmid>10371443</pmid><doi>10.1002/(SICI)1522-2594(199906)41:6<1119::AID-MRM7>3.0.CO;2-F</doi><tpages>8</tpages><oa>free_for_read</oa></addata></record> |
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subjects | Adolescent Adrenoleukodystrophy - diagnosis Adrenoleukodystrophy - genetics Adrenoleukodystrophy - metabolism Biological and medical sciences Brain - metabolism brain metabolic grading Carbohydrates (enzymatic deficiencies). Glycogenosis Case-Control Studies Child Child, Preschool Discriminant Analysis Errors of metabolism Genetic Linkage Humans Magnetic Resonance Spectroscopy Male Medical sciences Metabolic diseases proton magnetic resonance spectroscopy X Chromosome X-ALD |
title | Brain metabolic impairment in non-cerebral and cerebral forms of x-linked adrenoleukodystrophy by proton MRS: Identification of metabolic patterns by discriminant analysis |
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