Late adult onset of Langerhans cell histiocytosis mimicking glioblastoma multiforme
Abstract Langerhans cell histiocytosis (LCH) with multiple organ involvement is a rare disorder in adults. Extrapituitary involvement of the central nervous system (CNS) is uncommon. We report the unusual case of a 55-year-old woman presenting with a left-sided hemiataxia-hemiparesis, left hemisenso...
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Veröffentlicht in: | Journal of the neurological sciences 2011-02, Vol.301 (1), p.96-99 |
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description | Abstract Langerhans cell histiocytosis (LCH) with multiple organ involvement is a rare disorder in adults. Extrapituitary involvement of the central nervous system (CNS) is uncommon. We report the unusual case of a 55-year-old woman presenting with a left-sided hemiataxia-hemiparesis, left hemisensory loss and short-lasting episodes of an alien left hand due to lesions of the internal capsule and the right thalamus, extending into the mesencephalon associated with extensive surrounding edema, without pituitary involvement. The neuroradiological image suggested glioblastoma multiforme. Brain biopsy revealed inflammatory tissue and “pseudotumoral” multiple sclerosis was suspected. Biopsy of concomitant lung and bone lesions disclosed Langerhans cell histiocytosis. The treatment with pulsed steroids in association with mycophenolate mofetil led to a sustained, clinical neurological remission. |
doi_str_mv | 10.1016/j.jns.2010.11.006 |
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Extrapituitary involvement of the central nervous system (CNS) is uncommon. We report the unusual case of a 55-year-old woman presenting with a left-sided hemiataxia-hemiparesis, left hemisensory loss and short-lasting episodes of an alien left hand due to lesions of the internal capsule and the right thalamus, extending into the mesencephalon associated with extensive surrounding edema, without pituitary involvement. The neuroradiological image suggested glioblastoma multiforme. Brain biopsy revealed inflammatory tissue and “pseudotumoral” multiple sclerosis was suspected. Biopsy of concomitant lung and bone lesions disclosed Langerhans cell histiocytosis. The treatment with pulsed steroids in association with mycophenolate mofetil led to a sustained, clinical neurological remission.</description><identifier>ISSN: 0022-510X</identifier><identifier>EISSN: 1878-5883</identifier><identifier>DOI: 10.1016/j.jns.2010.11.006</identifier><identifier>PMID: 21131007</identifier><identifier>CODEN: JNSCAG</identifier><language>eng</language><publisher>Amsterdam: Elsevier B.V</publisher><subject>Age of Onset ; Alien hand ; Alien Hand Syndrome - etiology ; Biological and medical sciences ; Biopsy ; Bone and Bones - pathology ; Brain - pathology ; Brain Diseases - complications ; Brain Diseases - diagnosis ; Brain Diseases - drug therapy ; Brain Neoplasms - diagnosis ; Cerebellar Ataxia - etiology ; CNS involvement ; Development. Senescence. Regeneration. Transplantation ; Dexamethasone - administration & dosage ; Dexamethasone - therapeutic use ; Diagnosis, Differential ; Drug Therapy, Combination ; Female ; Fundamental and applied biological sciences. Psychology ; Glioblastoma - diagnosis ; Histiocytosis, Langerhans-Cell - complications ; Histiocytosis, Langerhans-Cell - diagnosis ; Histiocytosis, Langerhans-Cell - drug therapy ; Histiocytosis, Langerhans-Cell - epidemiology ; Humans ; Langerhans cell histiocytosis ; Lung - pathology ; Magnetic Resonance Imaging ; Medical sciences ; Middle Aged ; Multiple Sclerosis - diagnosis ; Mycophenolate mofetil ; Mycophenolic Acid - administration & dosage ; Mycophenolic Acid - analogs & derivatives ; Mycophenolic Acid - therapeutic use ; Neurology ; Paresis - etiology ; Vertebrates: nervous system and sense organs</subject><ispartof>Journal of the neurological sciences, 2011-02, Vol.301 (1), p.96-99</ispartof><rights>Elsevier B.V.</rights><rights>2010 Elsevier B.V.</rights><rights>2015 INIST-CNRS</rights><rights>Copyright © 2010 Elsevier B.V. All rights reserved.</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c469t-833e9c7c41bd3e4856c5cb0281a4cd0bc47833a68fb7f670b18dfc85e0618b103</citedby><cites>FETCH-LOGICAL-c469t-833e9c7c41bd3e4856c5cb0281a4cd0bc47833a68fb7f670b18dfc85e0618b103</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktohtml>$$Uhttps://dx.doi.org/10.1016/j.jns.2010.11.006$$EHTML$$P50$$Gelsevier$$H</linktohtml><link.rule.ids>314,780,784,3548,27922,27923,45993</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=23790583$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/21131007$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Perren, F</creatorcontrib><creatorcontrib>Fankhauser, L</creatorcontrib><creatorcontrib>Thiévent, B</creatorcontrib><creatorcontrib>Pache, J.-C</creatorcontrib><creatorcontrib>Delavelle, J</creatorcontrib><creatorcontrib>Rochat, T</creatorcontrib><creatorcontrib>Landis, T</creatorcontrib><creatorcontrib>Chizzolini, C</creatorcontrib><title>Late adult onset of Langerhans cell histiocytosis mimicking glioblastoma multiforme</title><title>Journal of the neurological sciences</title><addtitle>J Neurol Sci</addtitle><description>Abstract Langerhans cell histiocytosis (LCH) with multiple organ involvement is a rare disorder in adults. Extrapituitary involvement of the central nervous system (CNS) is uncommon. We report the unusual case of a 55-year-old woman presenting with a left-sided hemiataxia-hemiparesis, left hemisensory loss and short-lasting episodes of an alien left hand due to lesions of the internal capsule and the right thalamus, extending into the mesencephalon associated with extensive surrounding edema, without pituitary involvement. The neuroradiological image suggested glioblastoma multiforme. Brain biopsy revealed inflammatory tissue and “pseudotumoral” multiple sclerosis was suspected. Biopsy of concomitant lung and bone lesions disclosed Langerhans cell histiocytosis. The treatment with pulsed steroids in association with mycophenolate mofetil led to a sustained, clinical neurological remission.</description><subject>Age of Onset</subject><subject>Alien hand</subject><subject>Alien Hand Syndrome - etiology</subject><subject>Biological and medical sciences</subject><subject>Biopsy</subject><subject>Bone and Bones - pathology</subject><subject>Brain - pathology</subject><subject>Brain Diseases - complications</subject><subject>Brain Diseases - diagnosis</subject><subject>Brain Diseases - drug therapy</subject><subject>Brain Neoplasms - diagnosis</subject><subject>Cerebellar Ataxia - etiology</subject><subject>CNS involvement</subject><subject>Development. Senescence. Regeneration. Transplantation</subject><subject>Dexamethasone - administration & dosage</subject><subject>Dexamethasone - therapeutic use</subject><subject>Diagnosis, Differential</subject><subject>Drug Therapy, Combination</subject><subject>Female</subject><subject>Fundamental and applied biological sciences. Psychology</subject><subject>Glioblastoma - diagnosis</subject><subject>Histiocytosis, Langerhans-Cell - complications</subject><subject>Histiocytosis, Langerhans-Cell - diagnosis</subject><subject>Histiocytosis, Langerhans-Cell - drug therapy</subject><subject>Histiocytosis, Langerhans-Cell - epidemiology</subject><subject>Humans</subject><subject>Langerhans cell histiocytosis</subject><subject>Lung - pathology</subject><subject>Magnetic Resonance Imaging</subject><subject>Medical sciences</subject><subject>Middle Aged</subject><subject>Multiple Sclerosis - diagnosis</subject><subject>Mycophenolate mofetil</subject><subject>Mycophenolic Acid - administration & dosage</subject><subject>Mycophenolic Acid - analogs & derivatives</subject><subject>Mycophenolic Acid - therapeutic use</subject><subject>Neurology</subject><subject>Paresis - etiology</subject><subject>Vertebrates: nervous system and sense organs</subject><issn>0022-510X</issn><issn>1878-5883</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2011</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNqFklGL1DAUhYMo7jj6A3yRvohPHe9tmjZFEGTRVRjwYRX2LaTp7Wy6bbImrTD_3pQZV_BBXxIC3zk5ObmMvUTYIWD1dtgNLu4KWM-4A6gesQ3KWuZCSv6YbQCKIhcINxfsWYwDJELK5im7KBA5AtQbdr3XM2W6W8Y58y5SWvtsr92Bwq12MTM0jtmtjbP15jj7aGM22cmaO-sO2WG0vh11nP2ksylZ2N6HiZ6zJ70eI70471v2_dPHb5ef8_3Xqy-XH_a5KatmziXn1JjalNh2nEopKiNMC4VEXZoOWlPWCdGV7Nu6r2poUXa9kYKgQtki8C17c_K9D_7HQnFWk41rYO3IL1HJpkEuAMv_k6XgDZaiSSSeSBN8jIF6dR_spMNRIai1dDWoVLpaS1eIKlWaNK_O7ks7Ufeg-N1yAl6fAR2NHvugnbHxD8frBkR665a9O3GUWvtpKahoLDlDnQ1kZtV5-88Y7_9Sm9E6my68oyPFwS_Bpe9QqGKhQF2v07EOR4oIQpQ3_BfvvrR5</recordid><startdate>20110215</startdate><enddate>20110215</enddate><creator>Perren, F</creator><creator>Fankhauser, L</creator><creator>Thiévent, B</creator><creator>Pache, J.-C</creator><creator>Delavelle, J</creator><creator>Rochat, T</creator><creator>Landis, T</creator><creator>Chizzolini, C</creator><general>Elsevier B.V</general><general>Elsevier</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><scope>7TK</scope></search><sort><creationdate>20110215</creationdate><title>Late adult onset of Langerhans cell histiocytosis mimicking glioblastoma multiforme</title><author>Perren, F ; Fankhauser, L ; Thiévent, B ; Pache, J.-C ; Delavelle, J ; Rochat, T ; Landis, T ; Chizzolini, C</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c469t-833e9c7c41bd3e4856c5cb0281a4cd0bc47833a68fb7f670b18dfc85e0618b103</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2011</creationdate><topic>Age of Onset</topic><topic>Alien hand</topic><topic>Alien Hand Syndrome - etiology</topic><topic>Biological and medical sciences</topic><topic>Biopsy</topic><topic>Bone and Bones - pathology</topic><topic>Brain - pathology</topic><topic>Brain Diseases - complications</topic><topic>Brain Diseases - diagnosis</topic><topic>Brain Diseases - drug therapy</topic><topic>Brain Neoplasms - diagnosis</topic><topic>Cerebellar Ataxia - etiology</topic><topic>CNS involvement</topic><topic>Development. Senescence. Regeneration. Transplantation</topic><topic>Dexamethasone - administration & dosage</topic><topic>Dexamethasone - therapeutic use</topic><topic>Diagnosis, Differential</topic><topic>Drug Therapy, Combination</topic><topic>Female</topic><topic>Fundamental and applied biological sciences. Psychology</topic><topic>Glioblastoma - diagnosis</topic><topic>Histiocytosis, Langerhans-Cell - complications</topic><topic>Histiocytosis, Langerhans-Cell - diagnosis</topic><topic>Histiocytosis, Langerhans-Cell - drug therapy</topic><topic>Histiocytosis, Langerhans-Cell - epidemiology</topic><topic>Humans</topic><topic>Langerhans cell histiocytosis</topic><topic>Lung - pathology</topic><topic>Magnetic Resonance Imaging</topic><topic>Medical sciences</topic><topic>Middle Aged</topic><topic>Multiple Sclerosis - diagnosis</topic><topic>Mycophenolate mofetil</topic><topic>Mycophenolic Acid - administration & dosage</topic><topic>Mycophenolic Acid - analogs & derivatives</topic><topic>Mycophenolic Acid - therapeutic use</topic><topic>Neurology</topic><topic>Paresis - etiology</topic><topic>Vertebrates: nervous system and sense organs</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Perren, F</creatorcontrib><creatorcontrib>Fankhauser, L</creatorcontrib><creatorcontrib>Thiévent, B</creatorcontrib><creatorcontrib>Pache, J.-C</creatorcontrib><creatorcontrib>Delavelle, J</creatorcontrib><creatorcontrib>Rochat, T</creatorcontrib><creatorcontrib>Landis, T</creatorcontrib><creatorcontrib>Chizzolini, C</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><collection>Neurosciences Abstracts</collection><jtitle>Journal of the neurological sciences</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Perren, F</au><au>Fankhauser, L</au><au>Thiévent, B</au><au>Pache, J.-C</au><au>Delavelle, J</au><au>Rochat, T</au><au>Landis, T</au><au>Chizzolini, C</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Late adult onset of Langerhans cell histiocytosis mimicking glioblastoma multiforme</atitle><jtitle>Journal of the neurological sciences</jtitle><addtitle>J Neurol Sci</addtitle><date>2011-02-15</date><risdate>2011</risdate><volume>301</volume><issue>1</issue><spage>96</spage><epage>99</epage><pages>96-99</pages><issn>0022-510X</issn><eissn>1878-5883</eissn><coden>JNSCAG</coden><abstract>Abstract Langerhans cell histiocytosis (LCH) with multiple organ involvement is a rare disorder in adults. Extrapituitary involvement of the central nervous system (CNS) is uncommon. We report the unusual case of a 55-year-old woman presenting with a left-sided hemiataxia-hemiparesis, left hemisensory loss and short-lasting episodes of an alien left hand due to lesions of the internal capsule and the right thalamus, extending into the mesencephalon associated with extensive surrounding edema, without pituitary involvement. The neuroradiological image suggested glioblastoma multiforme. Brain biopsy revealed inflammatory tissue and “pseudotumoral” multiple sclerosis was suspected. Biopsy of concomitant lung and bone lesions disclosed Langerhans cell histiocytosis. The treatment with pulsed steroids in association with mycophenolate mofetil led to a sustained, clinical neurological remission.</abstract><cop>Amsterdam</cop><pub>Elsevier B.V</pub><pmid>21131007</pmid><doi>10.1016/j.jns.2010.11.006</doi><tpages>4</tpages></addata></record> |
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subjects | Age of Onset Alien hand Alien Hand Syndrome - etiology Biological and medical sciences Biopsy Bone and Bones - pathology Brain - pathology Brain Diseases - complications Brain Diseases - diagnosis Brain Diseases - drug therapy Brain Neoplasms - diagnosis Cerebellar Ataxia - etiology CNS involvement Development. Senescence. Regeneration. Transplantation Dexamethasone - administration & dosage Dexamethasone - therapeutic use Diagnosis, Differential Drug Therapy, Combination Female Fundamental and applied biological sciences. Psychology Glioblastoma - diagnosis Histiocytosis, Langerhans-Cell - complications Histiocytosis, Langerhans-Cell - diagnosis Histiocytosis, Langerhans-Cell - drug therapy Histiocytosis, Langerhans-Cell - epidemiology Humans Langerhans cell histiocytosis Lung - pathology Magnetic Resonance Imaging Medical sciences Middle Aged Multiple Sclerosis - diagnosis Mycophenolate mofetil Mycophenolic Acid - administration & dosage Mycophenolic Acid - analogs & derivatives Mycophenolic Acid - therapeutic use Neurology Paresis - etiology Vertebrates: nervous system and sense organs |
title | Late adult onset of Langerhans cell histiocytosis mimicking glioblastoma multiforme |
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