Corpus Callosum Atrophy in Patients with Hereditary Diffuse Leukoencephalopathy with Neuroaxonal Spheroids: An MRI-based Study
Objective Hereditary diffuse leukoencephalopathy with neuroaxonal spheroids (HDLS) is an adult-onset white matter disease that presents clinically with cognitive, mental and motor dysfunction. Several autopsy reports have indicated that the corpus callosum (CC), the largest bundle of white matter, i...
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| Veröffentlicht in: | Internal Medicine 2014, Vol.53(1), pp.21-27 |
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| creator | Kinoshita, Michiaki Kondo, Yasufumi Yoshida, Kunihiro Fukushima, Kazuhiro Hoshi, Ken-ichi Ishizawa, Keisuke Araki, Nobuo Yazawa, Ikuru Washimi, Yukihiko Saitoh, Banyu Kira, Jun-ichi Ikeda, Shu-ichi |
| description | Objective Hereditary diffuse leukoencephalopathy with neuroaxonal spheroids (HDLS) is an adult-onset white matter disease that presents clinically with cognitive, mental and motor dysfunction. Several autopsy reports have indicated that the corpus callosum (CC), the largest bundle of white matter, is severely affected in patients with HDLS. The aim of this study was to evaluate corpus callosum atrophy (CCA) quantitatively in HDLS patients. Methods We assessed CCA in six genetically-proven HDLS patients (HDLS group), in comparison with that observed in 20 patients with vascular dementia (VaD group) and 24 age-matched patients without organic central nervous system (CNS) disease (non-CNS group). Using midsagittal MR images, five measurements of the CC were obtained: the width of the rostrum (aa'), body (bb') and splenium (cc'), the anterior to posterior length (ab) and the maximum height (cd). Next, the corpus callosum index (CCI) was calculated as (aa' + bb' + cc')/ab. Results All HDLS patients had white matter lesions in the CC and frontoparietal lobes on the initial MRI scans. Compared with that observed in the VaD and age-matched non-CNS groups, the CCI was significantly decreased in the HDLS group (with VaD group, p |
| doi_str_mv | 10.2169/internalmedicine.53.0863 |
| format | Article |
| fullrecord | <record><control><sourceid>proquest_cross</sourceid><recordid>TN_cdi_proquest_miscellaneous_1622613084</recordid><sourceformat>XML</sourceformat><sourcesystem>PC</sourcesystem><sourcerecordid>1490743475</sourcerecordid><originalsourceid>FETCH-LOGICAL-c603t-eef8625b53c5634e0b55d509cbf67e96c08501b5f56a75b3ba938828d3dd5ab53</originalsourceid><addsrcrecordid>eNqFkc1u1DAUhS1ERYfCKyAv2WRw7Nix2Y2GQisNLaKwthznhrhk4uAfwWx4djLMdBYIqZt7F_c751g-COGSLGkp1Bs3JgijGbbQOutGWHK2JFKwJ2hRskoVNWX8KVoQVcqCzuMcPY_xnhAma0WfoXNaMUU4ZQv0e-3DlCNem2HwMW_xKgU_9TvsRvzJJAdjivinSz2-gjCnJRN2-J3ruhwBbyB_9zBamHoz-MmkWfeXvYEcvPnl5yfiu6mH4F0b3-LViD9-vi4aE6HFdym3uxforDNDhJfHfYG-vr_8sr4qNrcfrterTWEFYakA6KSgvOHMcsEqIA3nLSfKNp2oQQlLJCdlwzsuTM0b1hjFpKSyZW3LzSy7QK8PvlPwPzLEpLcuWhgGM4LPUZeCUlEyIqvH0UqRumJVvXeVB9QGH2OATk_Bbecf0iXR-6L0v0VpzvS-qFn66piSm_l4Ej40MwM3B-A-JvMNToAJydkB_utc7scx4QTa3gQNI_sDqLCzEA</addsrcrecordid><sourcetype>Aggregation Database</sourcetype><iscdi>true</iscdi><recordtype>article</recordtype><pqid>1490743475</pqid></control><display><type>article</type><title>Corpus Callosum Atrophy in Patients with Hereditary Diffuse Leukoencephalopathy with Neuroaxonal Spheroids: An MRI-based Study</title><source>MEDLINE</source><source>J-STAGE (Japan Science & Technology Information Aggregator, Electronic) Freely Available Titles - Japanese</source><creator>Kinoshita, Michiaki ; Kondo, Yasufumi ; Yoshida, Kunihiro ; Fukushima, Kazuhiro ; Hoshi, Ken-ichi ; Ishizawa, Keisuke ; Araki, Nobuo ; Yazawa, Ikuru ; Washimi, Yukihiko ; Saitoh, Banyu ; Kira, Jun-ichi ; Ikeda, Shu-ichi</creator><creatorcontrib>Kinoshita, Michiaki ; Kondo, Yasufumi ; Yoshida, Kunihiro ; Fukushima, Kazuhiro ; Hoshi, Ken-ichi ; Ishizawa, Keisuke ; Araki, Nobuo ; Yazawa, Ikuru ; Washimi, Yukihiko ; Saitoh, Banyu ; Kira, Jun-ichi ; Ikeda, Shu-ichi</creatorcontrib><description>Objective Hereditary diffuse leukoencephalopathy with neuroaxonal spheroids (HDLS) is an adult-onset white matter disease that presents clinically with cognitive, mental and motor dysfunction. Several autopsy reports have indicated that the corpus callosum (CC), the largest bundle of white matter, is severely affected in patients with HDLS. The aim of this study was to evaluate corpus callosum atrophy (CCA) quantitatively in HDLS patients. Methods We assessed CCA in six genetically-proven HDLS patients (HDLS group), in comparison with that observed in 20 patients with vascular dementia (VaD group) and 24 age-matched patients without organic central nervous system (CNS) disease (non-CNS group). Using midsagittal MR images, five measurements of the CC were obtained: the width of the rostrum (aa'), body (bb') and splenium (cc'), the anterior to posterior length (ab) and the maximum height (cd). Next, the corpus callosum index (CCI) was calculated as (aa' + bb' + cc')/ab. Results All HDLS patients had white matter lesions in the CC and frontoparietal lobes on the initial MRI scans. Compared with that observed in the VaD and age-matched non-CNS groups, the CCI was significantly decreased in the HDLS group (with VaD group, p<0.01; with non-CNS group, p<0.01). Conclusion This study showed significant atrophy of the CC in all HDLS patients on the initial MRI scans obtained 6-36 months after onset. We propose that the early appearance of CCA, frequently accompanied by high-intensity in the genu and/or splenium, on T2 images is an important diagnostic clue to HDLS.</description><identifier>ISSN: 0918-2918</identifier><identifier>EISSN: 1349-7235</identifier><identifier>DOI: 10.2169/internalmedicine.53.0863</identifier><identifier>PMID: 24390523</identifier><language>eng</language><publisher>Japan: The Japanese Society of Internal Medicine</publisher><subject>Adult ; Aged ; Atrophy - pathology ; colony stimulating factor 1 receptor (CSF1R) ; Corpus Callosum - pathology ; corpus callosum atrophy (CCA) ; corpus callosum index (CCI) ; Female ; hereditary diffuse leukoencephalopathy with neuroaxonal spheroids (HDLS) ; Humans ; Leukoencephalopathies - diagnosis ; Leukoencephalopathies - epidemiology ; Magnetic Resonance Imaging - methods ; Male ; Middle Aged ; Nerve Fibers, Myelinated - pathology ; Retrospective Studies ; white matter lesions (WMLs)</subject><ispartof>Internal Medicine, 2014, Vol.53(1), pp.21-27</ispartof><rights>2014 by The Japanese Society of Internal Medicine</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c603t-eef8625b53c5634e0b55d509cbf67e96c08501b5f56a75b3ba938828d3dd5ab53</citedby><cites>FETCH-LOGICAL-c603t-eef8625b53c5634e0b55d509cbf67e96c08501b5f56a75b3ba938828d3dd5ab53</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>314,777,781,1877,27905,27906</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/24390523$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Kinoshita, Michiaki</creatorcontrib><creatorcontrib>Kondo, Yasufumi</creatorcontrib><creatorcontrib>Yoshida, Kunihiro</creatorcontrib><creatorcontrib>Fukushima, Kazuhiro</creatorcontrib><creatorcontrib>Hoshi, Ken-ichi</creatorcontrib><creatorcontrib>Ishizawa, Keisuke</creatorcontrib><creatorcontrib>Araki, Nobuo</creatorcontrib><creatorcontrib>Yazawa, Ikuru</creatorcontrib><creatorcontrib>Washimi, Yukihiko</creatorcontrib><creatorcontrib>Saitoh, Banyu</creatorcontrib><creatorcontrib>Kira, Jun-ichi</creatorcontrib><creatorcontrib>Ikeda, Shu-ichi</creatorcontrib><title>Corpus Callosum Atrophy in Patients with Hereditary Diffuse Leukoencephalopathy with Neuroaxonal Spheroids: An MRI-based Study</title><title>Internal Medicine</title><addtitle>Intern. Med.</addtitle><description>Objective Hereditary diffuse leukoencephalopathy with neuroaxonal spheroids (HDLS) is an adult-onset white matter disease that presents clinically with cognitive, mental and motor dysfunction. Several autopsy reports have indicated that the corpus callosum (CC), the largest bundle of white matter, is severely affected in patients with HDLS. The aim of this study was to evaluate corpus callosum atrophy (CCA) quantitatively in HDLS patients. Methods We assessed CCA in six genetically-proven HDLS patients (HDLS group), in comparison with that observed in 20 patients with vascular dementia (VaD group) and 24 age-matched patients without organic central nervous system (CNS) disease (non-CNS group). Using midsagittal MR images, five measurements of the CC were obtained: the width of the rostrum (aa'), body (bb') and splenium (cc'), the anterior to posterior length (ab) and the maximum height (cd). Next, the corpus callosum index (CCI) was calculated as (aa' + bb' + cc')/ab. Results All HDLS patients had white matter lesions in the CC and frontoparietal lobes on the initial MRI scans. Compared with that observed in the VaD and age-matched non-CNS groups, the CCI was significantly decreased in the HDLS group (with VaD group, p<0.01; with non-CNS group, p<0.01). Conclusion This study showed significant atrophy of the CC in all HDLS patients on the initial MRI scans obtained 6-36 months after onset. We propose that the early appearance of CCA, frequently accompanied by high-intensity in the genu and/or splenium, on T2 images is an important diagnostic clue to HDLS.</description><subject>Adult</subject><subject>Aged</subject><subject>Atrophy - pathology</subject><subject>colony stimulating factor 1 receptor (CSF1R)</subject><subject>Corpus Callosum - pathology</subject><subject>corpus callosum atrophy (CCA)</subject><subject>corpus callosum index (CCI)</subject><subject>Female</subject><subject>hereditary diffuse leukoencephalopathy with neuroaxonal spheroids (HDLS)</subject><subject>Humans</subject><subject>Leukoencephalopathies - diagnosis</subject><subject>Leukoencephalopathies - epidemiology</subject><subject>Magnetic Resonance Imaging - methods</subject><subject>Male</subject><subject>Middle Aged</subject><subject>Nerve Fibers, Myelinated - pathology</subject><subject>Retrospective Studies</subject><subject>white matter lesions (WMLs)</subject><issn>0918-2918</issn><issn>1349-7235</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2014</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNqFkc1u1DAUhS1ERYfCKyAv2WRw7Nix2Y2GQisNLaKwthznhrhk4uAfwWx4djLMdBYIqZt7F_c751g-COGSLGkp1Bs3JgijGbbQOutGWHK2JFKwJ2hRskoVNWX8KVoQVcqCzuMcPY_xnhAma0WfoXNaMUU4ZQv0e-3DlCNem2HwMW_xKgU_9TvsRvzJJAdjivinSz2-gjCnJRN2-J3ruhwBbyB_9zBamHoz-MmkWfeXvYEcvPnl5yfiu6mH4F0b3-LViD9-vi4aE6HFdym3uxforDNDhJfHfYG-vr_8sr4qNrcfrterTWEFYakA6KSgvOHMcsEqIA3nLSfKNp2oQQlLJCdlwzsuTM0b1hjFpKSyZW3LzSy7QK8PvlPwPzLEpLcuWhgGM4LPUZeCUlEyIqvH0UqRumJVvXeVB9QGH2OATk_Bbecf0iXR-6L0v0VpzvS-qFn66piSm_l4Ej40MwM3B-A-JvMNToAJydkB_utc7scx4QTa3gQNI_sDqLCzEA</recordid><startdate>20140101</startdate><enddate>20140101</enddate><creator>Kinoshita, Michiaki</creator><creator>Kondo, Yasufumi</creator><creator>Yoshida, Kunihiro</creator><creator>Fukushima, Kazuhiro</creator><creator>Hoshi, Ken-ichi</creator><creator>Ishizawa, Keisuke</creator><creator>Araki, Nobuo</creator><creator>Yazawa, Ikuru</creator><creator>Washimi, Yukihiko</creator><creator>Saitoh, Banyu</creator><creator>Kira, Jun-ichi</creator><creator>Ikeda, Shu-ichi</creator><general>The Japanese Society of Internal Medicine</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>7TK</scope><scope>8FD</scope><scope>FR3</scope><scope>P64</scope><scope>RC3</scope></search><sort><creationdate>20140101</creationdate><title>Corpus Callosum Atrophy in Patients with Hereditary Diffuse Leukoencephalopathy with Neuroaxonal Spheroids: An MRI-based Study</title><author>Kinoshita, Michiaki ; Kondo, Yasufumi ; Yoshida, Kunihiro ; Fukushima, Kazuhiro ; Hoshi, Ken-ichi ; Ishizawa, Keisuke ; Araki, Nobuo ; Yazawa, Ikuru ; Washimi, Yukihiko ; Saitoh, Banyu ; Kira, Jun-ichi ; Ikeda, Shu-ichi</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c603t-eef8625b53c5634e0b55d509cbf67e96c08501b5f56a75b3ba938828d3dd5ab53</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2014</creationdate><topic>Adult</topic><topic>Aged</topic><topic>Atrophy - pathology</topic><topic>colony stimulating factor 1 receptor (CSF1R)</topic><topic>Corpus Callosum - pathology</topic><topic>corpus callosum atrophy (CCA)</topic><topic>corpus callosum index (CCI)</topic><topic>Female</topic><topic>hereditary diffuse leukoencephalopathy with neuroaxonal spheroids (HDLS)</topic><topic>Humans</topic><topic>Leukoencephalopathies - diagnosis</topic><topic>Leukoencephalopathies - epidemiology</topic><topic>Magnetic Resonance Imaging - methods</topic><topic>Male</topic><topic>Middle Aged</topic><topic>Nerve Fibers, Myelinated - pathology</topic><topic>Retrospective Studies</topic><topic>white matter lesions (WMLs)</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Kinoshita, Michiaki</creatorcontrib><creatorcontrib>Kondo, Yasufumi</creatorcontrib><creatorcontrib>Yoshida, Kunihiro</creatorcontrib><creatorcontrib>Fukushima, Kazuhiro</creatorcontrib><creatorcontrib>Hoshi, Ken-ichi</creatorcontrib><creatorcontrib>Ishizawa, Keisuke</creatorcontrib><creatorcontrib>Araki, Nobuo</creatorcontrib><creatorcontrib>Yazawa, Ikuru</creatorcontrib><creatorcontrib>Washimi, Yukihiko</creatorcontrib><creatorcontrib>Saitoh, Banyu</creatorcontrib><creatorcontrib>Kira, Jun-ichi</creatorcontrib><creatorcontrib>Ikeda, Shu-ichi</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>Neurosciences Abstracts</collection><collection>Technology Research Database</collection><collection>Engineering Research Database</collection><collection>Biotechnology and BioEngineering Abstracts</collection><collection>Genetics Abstracts</collection><jtitle>Internal Medicine</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Kinoshita, Michiaki</au><au>Kondo, Yasufumi</au><au>Yoshida, Kunihiro</au><au>Fukushima, Kazuhiro</au><au>Hoshi, Ken-ichi</au><au>Ishizawa, Keisuke</au><au>Araki, Nobuo</au><au>Yazawa, Ikuru</au><au>Washimi, Yukihiko</au><au>Saitoh, Banyu</au><au>Kira, Jun-ichi</au><au>Ikeda, Shu-ichi</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Corpus Callosum Atrophy in Patients with Hereditary Diffuse Leukoencephalopathy with Neuroaxonal Spheroids: An MRI-based Study</atitle><jtitle>Internal Medicine</jtitle><addtitle>Intern. Med.</addtitle><date>2014-01-01</date><risdate>2014</risdate><volume>53</volume><issue>1</issue><spage>21</spage><epage>27</epage><pages>21-27</pages><issn>0918-2918</issn><eissn>1349-7235</eissn><abstract>Objective Hereditary diffuse leukoencephalopathy with neuroaxonal spheroids (HDLS) is an adult-onset white matter disease that presents clinically with cognitive, mental and motor dysfunction. Several autopsy reports have indicated that the corpus callosum (CC), the largest bundle of white matter, is severely affected in patients with HDLS. The aim of this study was to evaluate corpus callosum atrophy (CCA) quantitatively in HDLS patients. Methods We assessed CCA in six genetically-proven HDLS patients (HDLS group), in comparison with that observed in 20 patients with vascular dementia (VaD group) and 24 age-matched patients without organic central nervous system (CNS) disease (non-CNS group). Using midsagittal MR images, five measurements of the CC were obtained: the width of the rostrum (aa'), body (bb') and splenium (cc'), the anterior to posterior length (ab) and the maximum height (cd). Next, the corpus callosum index (CCI) was calculated as (aa' + bb' + cc')/ab. Results All HDLS patients had white matter lesions in the CC and frontoparietal lobes on the initial MRI scans. Compared with that observed in the VaD and age-matched non-CNS groups, the CCI was significantly decreased in the HDLS group (with VaD group, p<0.01; with non-CNS group, p<0.01). Conclusion This study showed significant atrophy of the CC in all HDLS patients on the initial MRI scans obtained 6-36 months after onset. We propose that the early appearance of CCA, frequently accompanied by high-intensity in the genu and/or splenium, on T2 images is an important diagnostic clue to HDLS.</abstract><cop>Japan</cop><pub>The Japanese Society of Internal Medicine</pub><pmid>24390523</pmid><doi>10.2169/internalmedicine.53.0863</doi><tpages>7</tpages><oa>free_for_read</oa></addata></record> |
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| subjects | Adult Aged Atrophy - pathology colony stimulating factor 1 receptor (CSF1R) Corpus Callosum - pathology corpus callosum atrophy (CCA) corpus callosum index (CCI) Female hereditary diffuse leukoencephalopathy with neuroaxonal spheroids (HDLS) Humans Leukoencephalopathies - diagnosis Leukoencephalopathies - epidemiology Magnetic Resonance Imaging - methods Male Middle Aged Nerve Fibers, Myelinated - pathology Retrospective Studies white matter lesions (WMLs) |
| title | Corpus Callosum Atrophy in Patients with Hereditary Diffuse Leukoencephalopathy with Neuroaxonal Spheroids: An MRI-based Study |
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