Diffusion-weighted image and MR spectroscopic analysis of a case of MELAS with repeated attacks
We report the clinical and MR manifestations of an 18 year-old girl with mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome. Recurrent status epilepticus caused reversible cytotoxic edema on diffusion-weighted images (DWI). Initial and one month follow...
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Veröffentlicht in: | Yonsei medical journal 2001-02, Vol.42 (1), p.128-133 |
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creator | Kim, H S Kim, D I Lee, B I Jeong, E K Choi, C Lee, J D Yoon, P H Kim, E J Kim, S H Yoon, Y K |
description | We report the clinical and MR manifestations of an 18 year-old girl with mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome. Recurrent status epilepticus caused reversible cytotoxic edema on diffusion-weighted images (DWI). Initial and one month follow-up MR spectroscopy, after seizure control, showed some discrepancies in the ratio of metabolites. N-acetylaspartate (NAA) partially recovered (NAA/creatine (Cr) ratio: 1.27-->1.84). This was because of a normalization of decreased NAA due to cellular dysfunction as a result of status epilepticus. A low ratio of NAA/Cr due to abnormal mitochondria remained in the decreased state. Reversible NAA/Cr ratios in the acute lesion suggested that NAA reflects the neuronal function as well as the level of neuronal structural damage. The altered NAA/Cr ratio better correlated with the abnormal signal intensity area of T2-weighted images (T2WI) and DWI than the lactate (Lac)/Cr ratio. With conservative treatment with anti-epileptics not accompanied by coenzyme Q or sodium dichloroacetate, lactate persistently increased (Lac/Cr ratio: 1.01-->1.21) because of the continued production of lactate in cells with respiratory deficiency, which is the main pathology of MELAS. |
doi_str_mv | 10.3349/ymj.2001.42.1.128 |
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Recurrent status epilepticus caused reversible cytotoxic edema on diffusion-weighted images (DWI). Initial and one month follow-up MR spectroscopy, after seizure control, showed some discrepancies in the ratio of metabolites. N-acetylaspartate (NAA) partially recovered (NAA/creatine (Cr) ratio: 1.27-->1.84). This was because of a normalization of decreased NAA due to cellular dysfunction as a result of status epilepticus. A low ratio of NAA/Cr due to abnormal mitochondria remained in the decreased state. Reversible NAA/Cr ratios in the acute lesion suggested that NAA reflects the neuronal function as well as the level of neuronal structural damage. The altered NAA/Cr ratio better correlated with the abnormal signal intensity area of T2-weighted images (T2WI) and DWI than the lactate (Lac)/Cr ratio. With conservative treatment with anti-epileptics not accompanied by coenzyme Q or sodium dichloroacetate, lactate persistently increased (Lac/Cr ratio: 1.01-->1.21) because of the continued production of lactate in cells with respiratory deficiency, which is the main pathology of MELAS.</description><identifier>ISSN: 0513-5796</identifier><identifier>DOI: 10.3349/ymj.2001.42.1.128</identifier><identifier>PMID: 11293491</identifier><language>eng</language><publisher>Korea (South)</publisher><subject>Adolescent ; Aspartic Acid - analogs & derivatives ; Aspartic Acid - metabolism ; Brain - metabolism ; Creatine - metabolism ; Diffusion ; Female ; Humans ; Magnetic Resonance Imaging ; Magnetic Resonance Spectroscopy ; MELAS Syndrome - diagnosis ; MELAS Syndrome - metabolism</subject><ispartof>Yonsei medical journal, 2001-02, Vol.42 (1), p.128-133</ispartof><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c340t-bde2d90e54be111b0ac6e32bc19c0f18d351669f9e3a8aafe6b2a0c60539595a3</citedby></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>314,780,784,27924,27925</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/11293491$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Kim, H S</creatorcontrib><creatorcontrib>Kim, D I</creatorcontrib><creatorcontrib>Lee, B I</creatorcontrib><creatorcontrib>Jeong, E K</creatorcontrib><creatorcontrib>Choi, C</creatorcontrib><creatorcontrib>Lee, J D</creatorcontrib><creatorcontrib>Yoon, P H</creatorcontrib><creatorcontrib>Kim, E J</creatorcontrib><creatorcontrib>Kim, S H</creatorcontrib><creatorcontrib>Yoon, Y K</creatorcontrib><title>Diffusion-weighted image and MR spectroscopic analysis of a case of MELAS with repeated attacks</title><title>Yonsei medical journal</title><addtitle>Yonsei Med J</addtitle><description>We report the clinical and MR manifestations of an 18 year-old girl with mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome. Recurrent status epilepticus caused reversible cytotoxic edema on diffusion-weighted images (DWI). Initial and one month follow-up MR spectroscopy, after seizure control, showed some discrepancies in the ratio of metabolites. N-acetylaspartate (NAA) partially recovered (NAA/creatine (Cr) ratio: 1.27-->1.84). This was because of a normalization of decreased NAA due to cellular dysfunction as a result of status epilepticus. A low ratio of NAA/Cr due to abnormal mitochondria remained in the decreased state. Reversible NAA/Cr ratios in the acute lesion suggested that NAA reflects the neuronal function as well as the level of neuronal structural damage. The altered NAA/Cr ratio better correlated with the abnormal signal intensity area of T2-weighted images (T2WI) and DWI than the lactate (Lac)/Cr ratio. With conservative treatment with anti-epileptics not accompanied by coenzyme Q or sodium dichloroacetate, lactate persistently increased (Lac/Cr ratio: 1.01-->1.21) because of the continued production of lactate in cells with respiratory deficiency, which is the main pathology of MELAS.</description><subject>Adolescent</subject><subject>Aspartic Acid - analogs & derivatives</subject><subject>Aspartic Acid - metabolism</subject><subject>Brain - metabolism</subject><subject>Creatine - metabolism</subject><subject>Diffusion</subject><subject>Female</subject><subject>Humans</subject><subject>Magnetic Resonance Imaging</subject><subject>Magnetic Resonance Spectroscopy</subject><subject>MELAS Syndrome - diagnosis</subject><subject>MELAS Syndrome - metabolism</subject><issn>0513-5796</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2001</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNpFkEtrwzAQhHVoadK0P6CXolNvdrWSZVvHkKYPSCj0cRayvE6U2rFr2YT8-9ok0NMOy8zAfITcAQuFiNTjsdqFnDEIIx5CCDy9IFMmQQQyUfGEXHu_Y4wnwPgVmQBwNYRgSvSTK4reu3ofHNBtth3m1FVmg9Tsc7r-oL5B27W1t3Xj7PA05dE7T-uCGmqNx1Gtl6v5Jz24bktbbNCMJabrjP3xN-SyMKXH2_Odke_n5dfiNVi9v7wt5qvAioh1QZYjzxVDGWUIABkzNkbBMwvKsgLSXEiIY1UoFCY1psA444bZmEmhpJJGzMjDqbdp698efacr5y2Wpdlj3XudJIzLJI0GI5yMdhjlWyx00w6D26MGpkeSeiCpR5I64hr0QHLI3J_L-6zC_D9xxij-AF9achM</recordid><startdate>20010201</startdate><enddate>20010201</enddate><creator>Kim, H S</creator><creator>Kim, D I</creator><creator>Lee, B I</creator><creator>Jeong, E K</creator><creator>Choi, C</creator><creator>Lee, J D</creator><creator>Yoon, P H</creator><creator>Kim, E J</creator><creator>Kim, S H</creator><creator>Yoon, Y K</creator><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>20010201</creationdate><title>Diffusion-weighted image and MR spectroscopic analysis of a case of MELAS with repeated attacks</title><author>Kim, H S ; Kim, D I ; Lee, B I ; Jeong, E K ; Choi, C ; Lee, J D ; Yoon, P H ; Kim, E J ; Kim, S H ; Yoon, Y K</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c340t-bde2d90e54be111b0ac6e32bc19c0f18d351669f9e3a8aafe6b2a0c60539595a3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2001</creationdate><topic>Adolescent</topic><topic>Aspartic Acid - analogs & derivatives</topic><topic>Aspartic Acid - metabolism</topic><topic>Brain - metabolism</topic><topic>Creatine - metabolism</topic><topic>Diffusion</topic><topic>Female</topic><topic>Humans</topic><topic>Magnetic Resonance Imaging</topic><topic>Magnetic Resonance Spectroscopy</topic><topic>MELAS Syndrome - diagnosis</topic><topic>MELAS Syndrome - metabolism</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Kim, H S</creatorcontrib><creatorcontrib>Kim, D I</creatorcontrib><creatorcontrib>Lee, B I</creatorcontrib><creatorcontrib>Jeong, E K</creatorcontrib><creatorcontrib>Choi, C</creatorcontrib><creatorcontrib>Lee, J D</creatorcontrib><creatorcontrib>Yoon, P H</creatorcontrib><creatorcontrib>Kim, E J</creatorcontrib><creatorcontrib>Kim, S H</creatorcontrib><creatorcontrib>Yoon, Y K</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><jtitle>Yonsei medical journal</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Kim, H S</au><au>Kim, D I</au><au>Lee, B I</au><au>Jeong, E K</au><au>Choi, C</au><au>Lee, J D</au><au>Yoon, P H</au><au>Kim, E J</au><au>Kim, S H</au><au>Yoon, Y K</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Diffusion-weighted image and MR spectroscopic analysis of a case of MELAS with repeated attacks</atitle><jtitle>Yonsei medical journal</jtitle><addtitle>Yonsei Med J</addtitle><date>2001-02-01</date><risdate>2001</risdate><volume>42</volume><issue>1</issue><spage>128</spage><epage>133</epage><pages>128-133</pages><issn>0513-5796</issn><abstract>We report the clinical and MR manifestations of an 18 year-old girl with mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome. Recurrent status epilepticus caused reversible cytotoxic edema on diffusion-weighted images (DWI). Initial and one month follow-up MR spectroscopy, after seizure control, showed some discrepancies in the ratio of metabolites. N-acetylaspartate (NAA) partially recovered (NAA/creatine (Cr) ratio: 1.27-->1.84). This was because of a normalization of decreased NAA due to cellular dysfunction as a result of status epilepticus. A low ratio of NAA/Cr due to abnormal mitochondria remained in the decreased state. Reversible NAA/Cr ratios in the acute lesion suggested that NAA reflects the neuronal function as well as the level of neuronal structural damage. The altered NAA/Cr ratio better correlated with the abnormal signal intensity area of T2-weighted images (T2WI) and DWI than the lactate (Lac)/Cr ratio. With conservative treatment with anti-epileptics not accompanied by coenzyme Q or sodium dichloroacetate, lactate persistently increased (Lac/Cr ratio: 1.01-->1.21) because of the continued production of lactate in cells with respiratory deficiency, which is the main pathology of MELAS.</abstract><cop>Korea (South)</cop><pmid>11293491</pmid><doi>10.3349/ymj.2001.42.1.128</doi><tpages>6</tpages><oa>free_for_read</oa></addata></record> |
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subjects | Adolescent Aspartic Acid - analogs & derivatives Aspartic Acid - metabolism Brain - metabolism Creatine - metabolism Diffusion Female Humans Magnetic Resonance Imaging Magnetic Resonance Spectroscopy MELAS Syndrome - diagnosis MELAS Syndrome - metabolism |
title | Diffusion-weighted image and MR spectroscopic analysis of a case of MELAS with repeated attacks |
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