Age-Related Changes in a Patient With Pelizaeus-Merzbacher Disease Determined by Repeated 1H-Magnetic Resonance Spectroscopy
A boy with Pelizaeus-Merzbacher disease underwent repeated evaluations by 3-Tesla 1H-magnetic resonance spectroscopy (MRS). The patient showed overlap of the PLP1. Individuals selected as normal controls for 1H-magnetic resonance spectroscopy consisted of healthy age-matched children. For 1H-magneti...
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| Veröffentlicht in: | Journal of child neurology 2014-02, Vol.29 (2), p.283-288 |
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| container_title | Journal of child neurology |
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| creator | Mori, Tatsuo Mori, Kenji Ito, Hiromichi Goji, Aya Miyazaki, Masahito Harada, Masafumi Kurosawa, Kenji Kagami, Shoji |
| description | A boy with Pelizaeus-Merzbacher disease underwent repeated evaluations by 3-Tesla 1H-magnetic resonance spectroscopy (MRS). The patient showed overlap of the PLP1. Individuals selected as normal controls for 1H-magnetic resonance spectroscopy consisted of healthy age-matched children. For 1H-magnetic resonance spectroscopy, the center of a voxel was positioned in the right parietal lobe. 1H-magnetic resonance spectroscopy was performed when the patient was 2, 6, 14, and 25 months old. γ-Aminobutyric acid concentration in early childhood was increased compared with that in normal controls. However, the γ-aminobutyric acid concentration in the Pelizaeus-Merzbacher disease patient was normalized at 14 and 25 months. No remarkable changes were observed in choline-containing compounds concentration at any time. These results suggest that the changes in metabolite concentrations during growth can reflect the pathological condition of Pelizaeus-Merzbacher disease. Furthermore, the lack of change in the choline-containing compounds concentration can be useful for differentiating Pelizaeus-Merzbacher disease from other white matter disorders. |
| doi_str_mv | 10.1177/0883073813499635 |
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The patient showed overlap of the PLP1. Individuals selected as normal controls for 1H-magnetic resonance spectroscopy consisted of healthy age-matched children. For 1H-magnetic resonance spectroscopy, the center of a voxel was positioned in the right parietal lobe. 1H-magnetic resonance spectroscopy was performed when the patient was 2, 6, 14, and 25 months old. γ-Aminobutyric acid concentration in early childhood was increased compared with that in normal controls. However, the γ-aminobutyric acid concentration in the Pelizaeus-Merzbacher disease patient was normalized at 14 and 25 months. No remarkable changes were observed in choline-containing compounds concentration at any time. These results suggest that the changes in metabolite concentrations during growth can reflect the pathological condition of Pelizaeus-Merzbacher disease. Furthermore, the lack of change in the choline-containing compounds concentration can be useful for differentiating Pelizaeus-Merzbacher disease from other white matter disorders.</description><identifier>ISSN: 0883-0738</identifier><identifier>EISSN: 1708-8283</identifier><identifier>DOI: 10.1177/0883073813499635</identifier><identifier>PMID: 24056155</identifier><language>eng</language><publisher>Los Angeles, CA: SAGE Publications</publisher><subject>Age ; Aging ; Aspartic Acid - analogs & derivatives ; Aspartic Acid - metabolism ; Case-Control Studies ; Child, Preschool ; Creatine - metabolism ; gamma-Aminobutyric Acid - metabolism ; Glutamic Acid - metabolism ; Humans ; Infant ; Inositol - metabolism ; Magnetic Resonance Spectroscopy ; Male ; Parietal Lobe - growth & development ; Parietal Lobe - metabolism ; Pelizaeus-Merzbacher Disease - metabolism</subject><ispartof>Journal of child neurology, 2014-02, Vol.29 (2), p.283-288</ispartof><rights>The Author(s) 2013</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://journals.sagepub.com/doi/pdf/10.1177/0883073813499635$$EPDF$$P50$$Gsage$$H</linktopdf><linktohtml>$$Uhttps://journals.sagepub.com/doi/10.1177/0883073813499635$$EHTML$$P50$$Gsage$$H</linktohtml><link.rule.ids>314,780,784,21818,27923,27924,43620,43621</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/24056155$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Mori, Tatsuo</creatorcontrib><creatorcontrib>Mori, Kenji</creatorcontrib><creatorcontrib>Ito, Hiromichi</creatorcontrib><creatorcontrib>Goji, Aya</creatorcontrib><creatorcontrib>Miyazaki, Masahito</creatorcontrib><creatorcontrib>Harada, Masafumi</creatorcontrib><creatorcontrib>Kurosawa, Kenji</creatorcontrib><creatorcontrib>Kagami, Shoji</creatorcontrib><title>Age-Related Changes in a Patient With Pelizaeus-Merzbacher Disease Determined by Repeated 1H-Magnetic Resonance Spectroscopy</title><title>Journal of child neurology</title><addtitle>J Child Neurol</addtitle><description>A boy with Pelizaeus-Merzbacher disease underwent repeated evaluations by 3-Tesla 1H-magnetic resonance spectroscopy (MRS). The patient showed overlap of the PLP1. Individuals selected as normal controls for 1H-magnetic resonance spectroscopy consisted of healthy age-matched children. For 1H-magnetic resonance spectroscopy, the center of a voxel was positioned in the right parietal lobe. 1H-magnetic resonance spectroscopy was performed when the patient was 2, 6, 14, and 25 months old. γ-Aminobutyric acid concentration in early childhood was increased compared with that in normal controls. However, the γ-aminobutyric acid concentration in the Pelizaeus-Merzbacher disease patient was normalized at 14 and 25 months. No remarkable changes were observed in choline-containing compounds concentration at any time. These results suggest that the changes in metabolite concentrations during growth can reflect the pathological condition of Pelizaeus-Merzbacher disease. Furthermore, the lack of change in the choline-containing compounds concentration can be useful for differentiating Pelizaeus-Merzbacher disease from other white matter disorders.</description><subject>Age</subject><subject>Aging</subject><subject>Aspartic Acid - analogs & derivatives</subject><subject>Aspartic Acid - metabolism</subject><subject>Case-Control Studies</subject><subject>Child, Preschool</subject><subject>Creatine - metabolism</subject><subject>gamma-Aminobutyric Acid - metabolism</subject><subject>Glutamic Acid - metabolism</subject><subject>Humans</subject><subject>Infant</subject><subject>Inositol - metabolism</subject><subject>Magnetic Resonance Spectroscopy</subject><subject>Male</subject><subject>Parietal Lobe - growth & development</subject><subject>Parietal Lobe - metabolism</subject><subject>Pelizaeus-Merzbacher Disease - metabolism</subject><issn>0883-0738</issn><issn>1708-8283</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2014</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNqNkU1P3DAQhi1EBQvlzgn5yMUwjjexfURLW5BARbSIYzRxJrtGWSfEzmERP75ZPi6cehpp3kePNPMydizhTEqtz8EYBVoZqebWFirfYTOpwQiTGbXLZttYbPN9dhDjEwCY3MIe28_mkBcyz2fs9WJJ4p5aTFTzxQrDkiL3gSO_w-QpJP7o04rfUetfkMYobml4qdCtaOCXPhJG4peUaFj7MBmqDb-nnt5s8krc4jJQ8m5axi5gcMT_9OTS0EXX9Zvv7FuDbaSjj3nIHn7--Lu4Eje_f10vLm5EL61OwjYSCBqEoimKhrTNHVJTI7gCzbwmU2dgnMkrV0OhMLNNZirjHClZS0eNOmSn795-6J5Hiqlc--iobTFQN8ZSzm1hDGij_wcFLS1k-YSefKBjtaa67Ae_xmFTfj53AsQ7EHFJ5VM3DmG6spRQbssrv5an_gHQkopl</recordid><startdate>201402</startdate><enddate>201402</enddate><creator>Mori, Tatsuo</creator><creator>Mori, Kenji</creator><creator>Ito, Hiromichi</creator><creator>Goji, Aya</creator><creator>Miyazaki, Masahito</creator><creator>Harada, Masafumi</creator><creator>Kurosawa, Kenji</creator><creator>Kagami, Shoji</creator><general>SAGE Publications</general><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>7X8</scope><scope>7TK</scope></search><sort><creationdate>201402</creationdate><title>Age-Related Changes in a Patient With Pelizaeus-Merzbacher Disease Determined by Repeated 1H-Magnetic Resonance Spectroscopy</title><author>Mori, Tatsuo ; Mori, Kenji ; Ito, Hiromichi ; Goji, Aya ; Miyazaki, Masahito ; Harada, Masafumi ; Kurosawa, Kenji ; Kagami, Shoji</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-p197t-9f10e0fa06f66fe795caefda0c6a84de8d208c85bcd063a29f28b8cce31d1cef3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2014</creationdate><topic>Age</topic><topic>Aging</topic><topic>Aspartic Acid - analogs & derivatives</topic><topic>Aspartic Acid - metabolism</topic><topic>Case-Control Studies</topic><topic>Child, Preschool</topic><topic>Creatine - metabolism</topic><topic>gamma-Aminobutyric Acid - metabolism</topic><topic>Glutamic Acid - metabolism</topic><topic>Humans</topic><topic>Infant</topic><topic>Inositol - metabolism</topic><topic>Magnetic Resonance Spectroscopy</topic><topic>Male</topic><topic>Parietal Lobe - growth & development</topic><topic>Parietal Lobe - metabolism</topic><topic>Pelizaeus-Merzbacher Disease - metabolism</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Mori, Tatsuo</creatorcontrib><creatorcontrib>Mori, Kenji</creatorcontrib><creatorcontrib>Ito, Hiromichi</creatorcontrib><creatorcontrib>Goji, Aya</creatorcontrib><creatorcontrib>Miyazaki, Masahito</creatorcontrib><creatorcontrib>Harada, Masafumi</creatorcontrib><creatorcontrib>Kurosawa, Kenji</creatorcontrib><creatorcontrib>Kagami, Shoji</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>MEDLINE - Academic</collection><collection>Neurosciences Abstracts</collection><jtitle>Journal of child neurology</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Mori, Tatsuo</au><au>Mori, Kenji</au><au>Ito, Hiromichi</au><au>Goji, Aya</au><au>Miyazaki, Masahito</au><au>Harada, Masafumi</au><au>Kurosawa, Kenji</au><au>Kagami, Shoji</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Age-Related Changes in a Patient With Pelizaeus-Merzbacher Disease Determined by Repeated 1H-Magnetic Resonance Spectroscopy</atitle><jtitle>Journal of child neurology</jtitle><addtitle>J Child Neurol</addtitle><date>2014-02</date><risdate>2014</risdate><volume>29</volume><issue>2</issue><spage>283</spage><epage>288</epage><pages>283-288</pages><issn>0883-0738</issn><eissn>1708-8283</eissn><abstract>A boy with Pelizaeus-Merzbacher disease underwent repeated evaluations by 3-Tesla 1H-magnetic resonance spectroscopy (MRS). The patient showed overlap of the PLP1. Individuals selected as normal controls for 1H-magnetic resonance spectroscopy consisted of healthy age-matched children. For 1H-magnetic resonance spectroscopy, the center of a voxel was positioned in the right parietal lobe. 1H-magnetic resonance spectroscopy was performed when the patient was 2, 6, 14, and 25 months old. γ-Aminobutyric acid concentration in early childhood was increased compared with that in normal controls. However, the γ-aminobutyric acid concentration in the Pelizaeus-Merzbacher disease patient was normalized at 14 and 25 months. No remarkable changes were observed in choline-containing compounds concentration at any time. These results suggest that the changes in metabolite concentrations during growth can reflect the pathological condition of Pelizaeus-Merzbacher disease. 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| source | MEDLINE; SAGE Complete A-Z List |
| subjects | Age Aging Aspartic Acid - analogs & derivatives Aspartic Acid - metabolism Case-Control Studies Child, Preschool Creatine - metabolism gamma-Aminobutyric Acid - metabolism Glutamic Acid - metabolism Humans Infant Inositol - metabolism Magnetic Resonance Spectroscopy Male Parietal Lobe - growth & development Parietal Lobe - metabolism Pelizaeus-Merzbacher Disease - metabolism |
| title | Age-Related Changes in a Patient With Pelizaeus-Merzbacher Disease Determined by Repeated 1H-Magnetic Resonance Spectroscopy |
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