The Proteus syndrome: CNS manifestations
Proteus syndrome is a complex hamartomatous disorder characterized by multiple, diverse, somatic manifestations. We present a case in which severe, evolving CNS abnormalities were also exhibited. Imaging findings at presentation included hemimegalencephaly, subependymal calcified nodules, and perive...
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| Veröffentlicht in: | American journal of neuroradiology 1998-05, Vol.19 (5), p.987-990 |
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| container_title | American journal of neuroradiology |
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| creator | Dietrich, RB Glidden, DE Roth, GM Martin, RA Demo, DS |
| description | Proteus syndrome is a complex hamartomatous disorder characterized by multiple, diverse, somatic manifestations. We present a case in which severe, evolving CNS abnormalities were also exhibited. Imaging findings at presentation included hemimegalencephaly, subependymal calcified nodules, and periventricular cysts. Subsequently, dural sinus thrombosis developed. Eight previously reported patients may also have had hemimegalencephaly. When neuroimaging studies show hemimegalencephaly in a child with pigmented skin lesions, Proteus syndrome should be considered in the differential diagnosis. |
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When neuroimaging studies show hemimegalencephaly in a child with pigmented skin lesions, Proteus syndrome should be considered in the differential diagnosis.</description><identifier>ISSN: 0195-6108</identifier><identifier>EISSN: 1936-959X</identifier><identifier>PMID: 9613526</identifier><identifier>CODEN: AAJNDL</identifier><language>eng</language><publisher>Oak Brook, IL: Am Soc Neuroradiology</publisher><subject>Biological and medical sciences ; Brain Diseases - diagnosis ; Brain Diseases - etiology ; Case Reports ; Cerebral Angiography ; Child, Preschool ; Complex syndromes ; Humans ; Magnetic Resonance Imaging ; Male ; Medical genetics ; Medical sciences ; Proteus Syndrome - complications ; Review ; Tomography, X-Ray Computed</subject><ispartof>American journal of neuroradiology, 1998-05, Vol.19 (5), p.987-990</ispartof><rights>1998 INIST-CNRS</rights><rights>Copyright © American Society of Neuroradiology</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://www.ncbi.nlm.nih.gov/pmc/articles/PMC8337570/pdf/$$EPDF$$P50$$Gpubmedcentral$$H</linktopdf><linktohtml>$$Uhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC8337570/$$EHTML$$P50$$Gpubmedcentral$$H</linktohtml><link.rule.ids>230,309,310,314,723,776,780,785,786,881,23909,23910,25118,53766,53768</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=2245804$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/9613526$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Dietrich, RB</creatorcontrib><creatorcontrib>Glidden, DE</creatorcontrib><creatorcontrib>Roth, GM</creatorcontrib><creatorcontrib>Martin, RA</creatorcontrib><creatorcontrib>Demo, DS</creatorcontrib><title>The Proteus syndrome: CNS manifestations</title><title>American journal of neuroradiology</title><addtitle>AJNR Am J Neuroradiol</addtitle><description>Proteus syndrome is a complex hamartomatous disorder characterized by multiple, diverse, somatic manifestations. We present a case in which severe, evolving CNS abnormalities were also exhibited. Imaging findings at presentation included hemimegalencephaly, subependymal calcified nodules, and periventricular cysts. Subsequently, dural sinus thrombosis developed. Eight previously reported patients may also have had hemimegalencephaly. When neuroimaging studies show hemimegalencephaly in a child with pigmented skin lesions, Proteus syndrome should be considered in the differential diagnosis.</description><subject>Biological and medical sciences</subject><subject>Brain Diseases - diagnosis</subject><subject>Brain Diseases - etiology</subject><subject>Case Reports</subject><subject>Cerebral Angiography</subject><subject>Child, Preschool</subject><subject>Complex syndromes</subject><subject>Humans</subject><subject>Magnetic Resonance Imaging</subject><subject>Male</subject><subject>Medical genetics</subject><subject>Medical sciences</subject><subject>Proteus Syndrome - complications</subject><subject>Review</subject><subject>Tomography, X-Ray Computed</subject><issn>0195-6108</issn><issn>1936-959X</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>1998</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNpVkEtLxDAUhYMo4zj6E4QuVNwU8miSxoUggy8YVHAEd-FOmk4jfYxJa5l_b2HKoKu7OB_fOdwDNCWKiVhx9XmIppgoHguC02N0EsIXxpgrSSdoogRhnIopul4WNnrzTWu7EIVtnfmmsjfR_OU9qqB2uQ0ttK6pwyk6yqEM9my8M_TxcL-cP8WL18fn-d0iLhjFbZyvSCIIERlNMJMcWyZwQhXDlGJCqDUYqBSEW2AsTcCYnBsqZJ4CZNhklM3Q7c676VaVzYytWw-l3nhXgd_qBpz-n9Su0OvmR6ds6JN4EFyNAt98d8N-XblgbFlCbZsuaKkUoSzlA3j-t2lfMf5myC_GHIKBMvdQGxf2GKUJT3EyYJc7rHDronfe6lBBWQ5Sovu-J0pzrVLJfgHYfHn1</recordid><startdate>19980501</startdate><enddate>19980501</enddate><creator>Dietrich, RB</creator><creator>Glidden, DE</creator><creator>Roth, GM</creator><creator>Martin, RA</creator><creator>Demo, DS</creator><general>Am Soc Neuroradiology</general><general>American Society of Neuroradiology</general><scope>IQODW</scope><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>7X8</scope><scope>5PM</scope></search><sort><creationdate>19980501</creationdate><title>The Proteus syndrome: CNS manifestations</title><author>Dietrich, RB ; Glidden, DE ; Roth, GM ; Martin, RA ; Demo, DS</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-h320t-fb146116d2403750e36042930220112ec0a27615ea3384accf5c267f8aad0cd23</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>1998</creationdate><topic>Biological and medical sciences</topic><topic>Brain Diseases - diagnosis</topic><topic>Brain Diseases - etiology</topic><topic>Case Reports</topic><topic>Cerebral Angiography</topic><topic>Child, Preschool</topic><topic>Complex syndromes</topic><topic>Humans</topic><topic>Magnetic Resonance Imaging</topic><topic>Male</topic><topic>Medical genetics</topic><topic>Medical sciences</topic><topic>Proteus Syndrome - complications</topic><topic>Review</topic><topic>Tomography, X-Ray Computed</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Dietrich, RB</creatorcontrib><creatorcontrib>Glidden, DE</creatorcontrib><creatorcontrib>Roth, GM</creatorcontrib><creatorcontrib>Martin, RA</creatorcontrib><creatorcontrib>Demo, DS</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>MEDLINE - Academic</collection><collection>PubMed Central (Full Participant titles)</collection><jtitle>American journal of neuroradiology</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Dietrich, RB</au><au>Glidden, DE</au><au>Roth, GM</au><au>Martin, RA</au><au>Demo, DS</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>The Proteus syndrome: CNS manifestations</atitle><jtitle>American journal of neuroradiology</jtitle><addtitle>AJNR Am J Neuroradiol</addtitle><date>1998-05-01</date><risdate>1998</risdate><volume>19</volume><issue>5</issue><spage>987</spage><epage>990</epage><pages>987-990</pages><issn>0195-6108</issn><eissn>1936-959X</eissn><coden>AAJNDL</coden><abstract>Proteus syndrome is a complex hamartomatous disorder characterized by multiple, diverse, somatic manifestations. We present a case in which severe, evolving CNS abnormalities were also exhibited. Imaging findings at presentation included hemimegalencephaly, subependymal calcified nodules, and periventricular cysts. Subsequently, dural sinus thrombosis developed. Eight previously reported patients may also have had hemimegalencephaly. When neuroimaging studies show hemimegalencephaly in a child with pigmented skin lesions, Proteus syndrome should be considered in the differential diagnosis.</abstract><cop>Oak Brook, IL</cop><pub>Am Soc Neuroradiology</pub><pmid>9613526</pmid><tpages>4</tpages></addata></record> |
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| ispartof | American journal of neuroradiology, 1998-05, Vol.19 (5), p.987-990 |
| issn | 0195-6108 1936-959X |
| language | eng |
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| source | MEDLINE; Elektronische Zeitschriftenbibliothek - Frei zugängliche E-Journals; PubMed Central |
| subjects | Biological and medical sciences Brain Diseases - diagnosis Brain Diseases - etiology Case Reports Cerebral Angiography Child, Preschool Complex syndromes Humans Magnetic Resonance Imaging Male Medical genetics Medical sciences Proteus Syndrome - complications Review Tomography, X-Ray Computed |
| title | The Proteus syndrome: CNS manifestations |
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