Microscopic Observations of the Brain in Rett Syndrome
Abstract RETT syndrome (RS) is a clinically defined disorder which appears to be unique to females and which is associated with apparent loss of cognitive and motor skills early in life. Using the technique of gapless serial section, microscopic analysis of the brains from three cases of RS and iden...
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| Veröffentlicht in: | Neuropediatrics 1995-04, Vol.26 (2), p.105-108 |
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| container_title | Neuropediatrics |
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| creator | Bauman, M. L. Kemper, Th. L. Arin, D. M. |
| description | Abstract
RETT syndrome (RS) is a clinically defined disorder which appears to be unique to females and which is associated with apparent loss of cognitive and motor skills early in life. Using the technique of gapless serial section, microscopic analysis of the brains from three cases of RS and identically processed age-matched controls was conducted to determine the nature and extent of cerebral abnormality in this disorder. Small neuronal cell size and increased cell packing density were observed throughout the brain in all three cases, without evidence of gliosis or active degeneration. These findings are consistent with a curtailment of brain development which may begin before birth. Further, the brain abnormalities in RS appear to be more diffuse than previously appreciated and are in accord with the widespread neurological symptoms characteristic of this disorder. |
| doi_str_mv | 10.1055/s-2007-979737 |
| format | Article |
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RETT syndrome (RS) is a clinically defined disorder which appears to be unique to females and which is associated with apparent loss of cognitive and motor skills early in life. Using the technique of gapless serial section, microscopic analysis of the brains from three cases of RS and identically processed age-matched controls was conducted to determine the nature and extent of cerebral abnormality in this disorder. Small neuronal cell size and increased cell packing density were observed throughout the brain in all three cases, without evidence of gliosis or active degeneration. These findings are consistent with a curtailment of brain development which may begin before birth. Further, the brain abnormalities in RS appear to be more diffuse than previously appreciated and are in accord with the widespread neurological symptoms characteristic of this disorder.</description><identifier>ISSN: 0174-304X</identifier><identifier>EISSN: 1439-1899</identifier><identifier>DOI: 10.1055/s-2007-979737</identifier><identifier>PMID: 7566446</identifier><identifier>CODEN: NRPDDB</identifier><language>eng</language><publisher>Stuttgart: Thieme</publisher><subject>Adolescent ; Biological and medical sciences ; Brain - physiopathology ; Brain - ultrastructure ; Cerebellum - physiopathology ; Child ; Child, Preschool ; Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases ; Female ; Humans ; Medical sciences ; Movement Disorders - complications ; Nerve Degeneration ; Neurology ; Neuropathology ; Photomicrography ; Rett Syndrome - complications ; Rett Syndrome - physiopathology</subject><ispartof>Neuropediatrics, 1995-04, Vol.26 (2), p.105-108</ispartof><rights>Hippokrates Verlag GmbH Stuttgart</rights><rights>1995 INIST-CNRS</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c420t-ff9a71d3f516904cea664dca416952e96d92fb6be5658384fdda170bff9315503</citedby></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://www.thieme-connect.de/products/ejournals/pdf/10.1055/s-2007-979737.pdf$$EPDF$$P50$$Gthieme$$H</linktopdf><link.rule.ids>310,311,315,782,786,791,792,3019,3020,23937,23938,25147,27931,27932,54566</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=3561495$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/7566446$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Bauman, M. L.</creatorcontrib><creatorcontrib>Kemper, Th. L.</creatorcontrib><creatorcontrib>Arin, D. M.</creatorcontrib><title>Microscopic Observations of the Brain in Rett Syndrome</title><title>Neuropediatrics</title><addtitle>Neuropediatrics</addtitle><description>Abstract
RETT syndrome (RS) is a clinically defined disorder which appears to be unique to females and which is associated with apparent loss of cognitive and motor skills early in life. Using the technique of gapless serial section, microscopic analysis of the brains from three cases of RS and identically processed age-matched controls was conducted to determine the nature and extent of cerebral abnormality in this disorder. Small neuronal cell size and increased cell packing density were observed throughout the brain in all three cases, without evidence of gliosis or active degeneration. These findings are consistent with a curtailment of brain development which may begin before birth. Further, the brain abnormalities in RS appear to be more diffuse than previously appreciated and are in accord with the widespread neurological symptoms characteristic of this disorder.</description><subject>Adolescent</subject><subject>Biological and medical sciences</subject><subject>Brain - physiopathology</subject><subject>Brain - ultrastructure</subject><subject>Cerebellum - physiopathology</subject><subject>Child</subject><subject>Child, Preschool</subject><subject>Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases</subject><subject>Female</subject><subject>Humans</subject><subject>Medical sciences</subject><subject>Movement Disorders - complications</subject><subject>Nerve Degeneration</subject><subject>Neurology</subject><subject>Neuropathology</subject><subject>Photomicrography</subject><subject>Rett Syndrome - complications</subject><subject>Rett Syndrome - physiopathology</subject><issn>0174-304X</issn><issn>1439-1899</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>1995</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNp1kEtLxDAQgIMo67p69Cj0IJ6MJs1rc9TFF6ws-ABvIU0TttI2a9IK--_N0rI3YWAY5psZ5gPgHKMbjBi7jTBHSEAppCDiAEwxJRLiuZSHYIqwoJAg-nUMTmL8RghTifgETATjnFI-Bfy1MsFH4zeVyVZFtOFXd5VvY-Zd1q1tdh901WYp3mzXZe_btgy-safgyOk62rMxz8Dn48PH4hkuV08vi7slNDRHHXROaoFL4hjmElFjdTpbGk1TyXIreSlzV_DCMs7mZE5dWWosUJHmCGYMkRm4GvZugv_pbexUU0Vj61q31vdRCcHmiKfPZwAO4O6bGKxTm1A1OmwVRmrnSUW186QGT4m_GBf3RWPLPT2KSf3Lsa-j0bULujVV3GOE8aSSJex6wLp1ZRurvn0f2iTkn6t_Na98gg</recordid><startdate>19950401</startdate><enddate>19950401</enddate><creator>Bauman, M. L.</creator><creator>Kemper, Th. L.</creator><creator>Arin, D. M.</creator><general>Thieme</general><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>19950401</creationdate><title>Microscopic Observations of the Brain in Rett Syndrome</title><author>Bauman, M. L. ; Kemper, Th. L. ; Arin, D. M.</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c420t-ff9a71d3f516904cea664dca416952e96d92fb6be5658384fdda170bff9315503</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>1995</creationdate><topic>Adolescent</topic><topic>Biological and medical sciences</topic><topic>Brain - physiopathology</topic><topic>Brain - ultrastructure</topic><topic>Cerebellum - physiopathology</topic><topic>Child</topic><topic>Child, Preschool</topic><topic>Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases</topic><topic>Female</topic><topic>Humans</topic><topic>Medical sciences</topic><topic>Movement Disorders - complications</topic><topic>Nerve Degeneration</topic><topic>Neurology</topic><topic>Neuropathology</topic><topic>Photomicrography</topic><topic>Rett Syndrome - complications</topic><topic>Rett Syndrome - physiopathology</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Bauman, M. L.</creatorcontrib><creatorcontrib>Kemper, Th. L.</creatorcontrib><creatorcontrib>Arin, D. M.</creatorcontrib><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>Neuropediatrics</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Bauman, M. L.</au><au>Kemper, Th. L.</au><au>Arin, D. M.</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Microscopic Observations of the Brain in Rett Syndrome</atitle><jtitle>Neuropediatrics</jtitle><addtitle>Neuropediatrics</addtitle><date>1995-04-01</date><risdate>1995</risdate><volume>26</volume><issue>2</issue><spage>105</spage><epage>108</epage><pages>105-108</pages><issn>0174-304X</issn><eissn>1439-1899</eissn><coden>NRPDDB</coden><abstract>Abstract
RETT syndrome (RS) is a clinically defined disorder which appears to be unique to females and which is associated with apparent loss of cognitive and motor skills early in life. Using the technique of gapless serial section, microscopic analysis of the brains from three cases of RS and identically processed age-matched controls was conducted to determine the nature and extent of cerebral abnormality in this disorder. Small neuronal cell size and increased cell packing density were observed throughout the brain in all three cases, without evidence of gliosis or active degeneration. These findings are consistent with a curtailment of brain development which may begin before birth. Further, the brain abnormalities in RS appear to be more diffuse than previously appreciated and are in accord with the widespread neurological symptoms characteristic of this disorder.</abstract><cop>Stuttgart</cop><pub>Thieme</pub><pmid>7566446</pmid><doi>10.1055/s-2007-979737</doi><tpages>4</tpages></addata></record> |
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| source | MEDLINE; Thieme Connect Journals |
| subjects | Adolescent Biological and medical sciences Brain - physiopathology Brain - ultrastructure Cerebellum - physiopathology Child Child, Preschool Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases Female Humans Medical sciences Movement Disorders - complications Nerve Degeneration Neurology Neuropathology Photomicrography Rett Syndrome - complications Rett Syndrome - physiopathology |
| title | Microscopic Observations of the Brain in Rett Syndrome |
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