Brain edema with clasmatodendrosis complicating ataxia telangiectasia
Abstract Ataxia-telangiectasia is a chronic progressive disorder affecting the nervous and immune systems, caused by a genetic defect in the ATM protein. Clasmatodendrosis, a distinct form of astroglial death, has rarely been reported in ataxia-telangiectasia. Neuropathology of our patient disclosed...
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| Veröffentlicht in: | Brain & development (Tokyo. 1979) 2017-08, Vol.39 (7), p.629-632 |
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| container_title | Brain & development (Tokyo. 1979) |
| container_volume | 39 |
| creator | Shimoda, Konomi Mimaki, Masakazu Fujino, Shuhei Takeuchi, Masato Hino, Rumi Uozaki, Hiroshi Hayashi, Masaharu Oka, Akira Mizuguchi, Masashi |
| description | Abstract Ataxia-telangiectasia is a chronic progressive disorder affecting the nervous and immune systems, caused by a genetic defect in the ATM protein. Clasmatodendrosis, a distinct form of astroglial death, has rarely been reported in ataxia-telangiectasia. Neuropathology of our patient disclosed diffuse edema of the cerebral and cerebellar white matter with prominent clasmatodendrosis, implicating ATM in the regulation of astroglial cell death. |
| doi_str_mv | 10.1016/j.braindev.2017.02.007 |
| format | Article |
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Clasmatodendrosis, a distinct form of astroglial death, has rarely been reported in ataxia-telangiectasia. Neuropathology of our patient disclosed diffuse edema of the cerebral and cerebellar white matter with prominent clasmatodendrosis, implicating ATM in the regulation of astroglial cell death.</description><identifier>ISSN: 0387-7604</identifier><identifier>EISSN: 1872-7131</identifier><identifier>DOI: 10.1016/j.braindev.2017.02.007</identifier><identifier>PMID: 28351596</identifier><language>eng</language><publisher>Netherlands: Elsevier B.V</publisher><subject>Adult ; Astrocytes - pathology ; Ataxia Telangiectasia - complications ; Ataxia Telangiectasia - pathology ; Ataxia Telangiectasia - physiopathology ; Ataxia Telangiectasia - therapy ; Ataxia-telangiectasia ; Brain - pathology ; Brain Edema - complications ; Brain Edema - pathology ; Brain Edema - physiopathology ; Brain Edema - therapy ; Clasmatodendrosis ; Fatal Outcome ; Humans ; Male ; Neurology ; NF-kappa B ; Tumor suppressor protein p53</subject><ispartof>Brain & development (Tokyo. 1979), 2017-08, Vol.39 (7), p.629-632</ispartof><rights>The Japanese Society of Child Neurology</rights><rights>2017 The Japanese Society of Child Neurology</rights><rights>Copyright © 2017 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c447t-6cfe45e2d08d09093fbef61642403afa2bd8a5a4ae55e9e7dfe384c0159946953</citedby><cites>FETCH-LOGICAL-c447t-6cfe45e2d08d09093fbef61642403afa2bd8a5a4ae55e9e7dfe384c0159946953</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.2017.02.007$$EHTML$$P50$$Gelsevier$$H</linktohtml><link.rule.ids>314,780,784,3548,27922,27923,45993</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/28351596$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Shimoda, Konomi</creatorcontrib><creatorcontrib>Mimaki, Masakazu</creatorcontrib><creatorcontrib>Fujino, Shuhei</creatorcontrib><creatorcontrib>Takeuchi, Masato</creatorcontrib><creatorcontrib>Hino, Rumi</creatorcontrib><creatorcontrib>Uozaki, Hiroshi</creatorcontrib><creatorcontrib>Hayashi, Masaharu</creatorcontrib><creatorcontrib>Oka, Akira</creatorcontrib><creatorcontrib>Mizuguchi, Masashi</creatorcontrib><title>Brain edema with clasmatodendrosis complicating ataxia telangiectasia</title><title>Brain & development (Tokyo. 1979)</title><addtitle>Brain Dev</addtitle><description>Abstract Ataxia-telangiectasia is a chronic progressive disorder affecting the nervous and immune systems, caused by a genetic defect in the ATM protein. Clasmatodendrosis, a distinct form of astroglial death, has rarely been reported in ataxia-telangiectasia. Neuropathology of our patient disclosed diffuse edema of the cerebral and cerebellar white matter with prominent clasmatodendrosis, implicating ATM in the regulation of astroglial cell death.</description><subject>Adult</subject><subject>Astrocytes - pathology</subject><subject>Ataxia Telangiectasia - complications</subject><subject>Ataxia Telangiectasia - pathology</subject><subject>Ataxia Telangiectasia - physiopathology</subject><subject>Ataxia Telangiectasia - therapy</subject><subject>Ataxia-telangiectasia</subject><subject>Brain - pathology</subject><subject>Brain Edema - complications</subject><subject>Brain Edema - pathology</subject><subject>Brain Edema - physiopathology</subject><subject>Brain Edema - therapy</subject><subject>Clasmatodendrosis</subject><subject>Fatal Outcome</subject><subject>Humans</subject><subject>Male</subject><subject>Neurology</subject><subject>NF-kappa B</subject><subject>Tumor suppressor protein p53</subject><issn>0387-7604</issn><issn>1872-7131</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2017</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNqFkc1P3DAQxa2qCLaUfwHlyCXp-COxc6mgCGglpB6gZ2vWnlBv87HYWVr-exwt9NBLT3N5783M7zF2yqHiwJtPm2odMYyenioBXFcgKgD9jq240aLUXPL3bAXS6FI3oI7Yh5Q2AMAFh0N2JIysed02K3b1ZYkpyNOAxe8w_yxcj2nAefI0-jilkAo3Dds-OJzD-FDgjH8CFjP1OD4EcjOmgB_ZQYd9opPXecx-XF_dX34tb7_ffLu8uC2dUnouG9eRqkl4MB5aaGW3pq7hjRIKJHYo1t5gjQqprqkl7TuSRjnIp7aqaWt5zM72uds4Pe4ozXYIyVGfb6Fplyw3RoARyrRZ2uylLj-RInV2G8OA8dlysAtCu7FvCO2C0IKwGWE2nr7u2K0H8n9tb8yy4HwvoPzpU6Bokws0OvIhZh7WT-H_Oz7_E-H6MGbE_S96prSZdnHMHC23KRvs3VLk0iPXEkAqLV8AXuWbQA</recordid><startdate>20170801</startdate><enddate>20170801</enddate><creator>Shimoda, Konomi</creator><creator>Mimaki, Masakazu</creator><creator>Fujino, Shuhei</creator><creator>Takeuchi, Masato</creator><creator>Hino, Rumi</creator><creator>Uozaki, Hiroshi</creator><creator>Hayashi, Masaharu</creator><creator>Oka, Akira</creator><creator>Mizuguchi, Masashi</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></search><sort><creationdate>20170801</creationdate><title>Brain edema with clasmatodendrosis complicating ataxia telangiectasia</title><author>Shimoda, Konomi ; Mimaki, Masakazu ; Fujino, Shuhei ; Takeuchi, Masato ; Hino, Rumi ; Uozaki, Hiroshi ; Hayashi, Masaharu ; Oka, Akira ; Mizuguchi, Masashi</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c447t-6cfe45e2d08d09093fbef61642403afa2bd8a5a4ae55e9e7dfe384c0159946953</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2017</creationdate><topic>Adult</topic><topic>Astrocytes - pathology</topic><topic>Ataxia Telangiectasia - complications</topic><topic>Ataxia Telangiectasia - pathology</topic><topic>Ataxia Telangiectasia - physiopathology</topic><topic>Ataxia Telangiectasia - therapy</topic><topic>Ataxia-telangiectasia</topic><topic>Brain - pathology</topic><topic>Brain Edema - complications</topic><topic>Brain Edema - pathology</topic><topic>Brain Edema - physiopathology</topic><topic>Brain Edema - therapy</topic><topic>Clasmatodendrosis</topic><topic>Fatal Outcome</topic><topic>Humans</topic><topic>Male</topic><topic>Neurology</topic><topic>NF-kappa B</topic><topic>Tumor suppressor protein p53</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Shimoda, Konomi</creatorcontrib><creatorcontrib>Mimaki, Masakazu</creatorcontrib><creatorcontrib>Fujino, Shuhei</creatorcontrib><creatorcontrib>Takeuchi, Masato</creatorcontrib><creatorcontrib>Hino, Rumi</creatorcontrib><creatorcontrib>Uozaki, Hiroshi</creatorcontrib><creatorcontrib>Hayashi, Masaharu</creatorcontrib><creatorcontrib>Oka, Akira</creatorcontrib><creatorcontrib>Mizuguchi, Masashi</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>Brain & development (Tokyo. 1979)</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Shimoda, Konomi</au><au>Mimaki, Masakazu</au><au>Fujino, Shuhei</au><au>Takeuchi, Masato</au><au>Hino, Rumi</au><au>Uozaki, Hiroshi</au><au>Hayashi, Masaharu</au><au>Oka, Akira</au><au>Mizuguchi, Masashi</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Brain edema with clasmatodendrosis complicating ataxia telangiectasia</atitle><jtitle>Brain & development (Tokyo. 1979)</jtitle><addtitle>Brain Dev</addtitle><date>2017-08-01</date><risdate>2017</risdate><volume>39</volume><issue>7</issue><spage>629</spage><epage>632</epage><pages>629-632</pages><issn>0387-7604</issn><eissn>1872-7131</eissn><abstract>Abstract Ataxia-telangiectasia is a chronic progressive disorder affecting the nervous and immune systems, caused by a genetic defect in the ATM protein. 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| fulltext | fulltext |
| identifier | ISSN: 0387-7604 |
| ispartof | Brain & development (Tokyo. 1979), 2017-08, Vol.39 (7), p.629-632 |
| issn | 0387-7604 1872-7131 |
| language | eng |
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| source | MEDLINE; ScienceDirect Journals (5 years ago - present) |
| subjects | Adult Astrocytes - pathology Ataxia Telangiectasia - complications Ataxia Telangiectasia - pathology Ataxia Telangiectasia - physiopathology Ataxia Telangiectasia - therapy Ataxia-telangiectasia Brain - pathology Brain Edema - complications Brain Edema - pathology Brain Edema - physiopathology Brain Edema - therapy Clasmatodendrosis Fatal Outcome Humans Male Neurology NF-kappa B Tumor suppressor protein p53 |
| title | Brain edema with clasmatodendrosis complicating ataxia telangiectasia |
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