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
Hauptverfasser: Perren, F, Fankhauser, L, Thiévent, B, Pache, J.-C, Delavelle, J, Rochat, T, Landis, T, Chizzolini, C
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container_end_page 99
container_issue 1
container_start_page 96
container_title Journal of the neurological sciences
container_volume 301
creator Perren, F
Fankhauser, L
Thiévent, B
Pache, J.-C
Delavelle, J
Rochat, T
Landis, T
Chizzolini, C
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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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 &amp; 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 &amp; dosage</subject><subject>Mycophenolic Acid - analogs &amp; 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 &amp; 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 &amp; dosage</topic><topic>Mycophenolic Acid - analogs &amp; 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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