Teaching NeuroImages: Rosai-Dorfman disease presenting with progressive early-onset cerebellar ataxia
A 13-year-old girl presented with a 3-year history of progressive gait abnormality. She recently had a self-limited cervical lymphadenopathy. Neurologic examination showed brisk tendon reflexes and moderate ataxia. Brain MRI disclosed hyperintense lesions in the cerebellum and pons (figure, A). Spin...
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Veröffentlicht in: | Neurology 2013-07, Vol.81 (5), p.e27-e28 |
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creator | Candeias da Silva, Carolina Pedroso, José Luiz Moraes, Fabiano Moulin de Rivero, René Leandro M Callegari, Fabiano Mesquita Araujo, Francisco Toso, Fabio Fieni Stávale, João Norberto Barsottini, Orlando Graziani Povoas |
description | A 13-year-old girl presented with a 3-year history of progressive gait abnormality. She recently had a self-limited cervical lymphadenopathy. Neurologic examination showed brisk tendon reflexes and moderate ataxia. Brain MRI disclosed hyperintense lesions in the cerebellum and pons (figure, A). Spine MRI showed a heterogeneous lesion in the T12 vertebra (figure, B). Histopathology of the cervical lymph node confirmed Rosai-Dorfman disease (RDD) by showing emperipolesis (figure, C and D). The patient will be followed up in order to determine disease progression and therapy. RDD is a rare autoimmune histiocytic proliferative disorder first recognized in 1969. The CNS is involved in 5% of cases and generally mimics meningiomas. Bone erosion can be detected in the spine. Herein, we describe a rare CNS manifestation of RDD resembling a neurodegenerative ataxia. |
doi_str_mv | 10.1212/WNL.0b013e31829d85d2 |
format | Article |
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She recently had a self-limited cervical lymphadenopathy. Neurologic examination showed brisk tendon reflexes and moderate ataxia. Brain MRI disclosed hyperintense lesions in the cerebellum and pons (figure, A). Spine MRI showed a heterogeneous lesion in the T12 vertebra (figure, B). Histopathology of the cervical lymph node confirmed Rosai-Dorfman disease (RDD) by showing emperipolesis (figure, C and D). The patient will be followed up in order to determine disease progression and therapy. RDD is a rare autoimmune histiocytic proliferative disorder first recognized in 1969. The CNS is involved in 5% of cases and generally mimics meningiomas. Bone erosion can be detected in the spine. Herein, we describe a rare CNS manifestation of RDD resembling a neurodegenerative ataxia.</description><identifier>ISSN: 0028-3878</identifier><identifier>EISSN: 1526-632X</identifier><identifier>DOI: 10.1212/WNL.0b013e31829d85d2</identifier><identifier>PMID: 23897881</identifier><language>eng</language><publisher>United States: by AAN Enterprises, Inc</publisher><subject>Adolescent ; Diagnosis, Differential ; Disease Progression ; Female ; Histiocytosis, Sinus - complications ; Histiocytosis, Sinus - diagnosis ; Humans ; Spinocerebellar Degenerations - diagnosis ; Spinocerebellar Degenerations - etiology</subject><ispartof>Neurology, 2013-07, Vol.81 (5), p.e27-e28</ispartof><rights>2013 by AAN Enterprises, Inc.</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>314,780,784,27923,27924</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/23897881$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Candeias da Silva, Carolina</creatorcontrib><creatorcontrib>Pedroso, José Luiz</creatorcontrib><creatorcontrib>Moraes, Fabiano Moulin de</creatorcontrib><creatorcontrib>Rivero, René Leandro M</creatorcontrib><creatorcontrib>Callegari, Fabiano Mesquita</creatorcontrib><creatorcontrib>Araujo, Francisco</creatorcontrib><creatorcontrib>Toso, Fabio Fieni</creatorcontrib><creatorcontrib>Stávale, João Norberto</creatorcontrib><creatorcontrib>Barsottini, Orlando Graziani Povoas</creatorcontrib><title>Teaching NeuroImages: Rosai-Dorfman disease presenting with progressive early-onset cerebellar ataxia</title><title>Neurology</title><addtitle>Neurology</addtitle><description>A 13-year-old girl presented with a 3-year history of progressive gait abnormality. She recently had a self-limited cervical lymphadenopathy. Neurologic examination showed brisk tendon reflexes and moderate ataxia. Brain MRI disclosed hyperintense lesions in the cerebellum and pons (figure, A). Spine MRI showed a heterogeneous lesion in the T12 vertebra (figure, B). Histopathology of the cervical lymph node confirmed Rosai-Dorfman disease (RDD) by showing emperipolesis (figure, C and D). The patient will be followed up in order to determine disease progression and therapy. RDD is a rare autoimmune histiocytic proliferative disorder first recognized in 1969. The CNS is involved in 5% of cases and generally mimics meningiomas. Bone erosion can be detected in the spine. Herein, we describe a rare CNS manifestation of RDD resembling a neurodegenerative ataxia.</description><subject>Adolescent</subject><subject>Diagnosis, Differential</subject><subject>Disease Progression</subject><subject>Female</subject><subject>Histiocytosis, Sinus - complications</subject><subject>Histiocytosis, Sinus - diagnosis</subject><subject>Humans</subject><subject>Spinocerebellar Degenerations - diagnosis</subject><subject>Spinocerebellar Degenerations - etiology</subject><issn>0028-3878</issn><issn>1526-632X</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2013</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNqF0c1OGzEQAGCroioh7RtUaI9cDJ6xd21zQ6GFSBGVEKjcVt54kizsT7B3G3h7jALn-jKamU-j0ZixnyBOAQHP_t4sTkUlQJIEg9ab3OMXNoEcC15IfDhgEyHQcGm0OWRHMT4KkZrafmOHKI3VxsCE0R255abu1tkNjaGft25N8Ty77aOr-WUfVq3rMl9HcpGybaBI3fCud_WwSXm_TqVY_6OMXGheed9FGrIlBaqoaVzI3OBeavedfV25JtKPjzhl979_3c2u-eLP1Xx2seBbNBY4OdBKWqNlYVHrilBqpayX2ucA2ktpsZArTKZyxipv0ShRAOVWVtXKyyk72c9Nmz2PFIeyrePyfZOO-jGWkBcCBFqt_08VFEIlLxI9_qBj1ZIvt6FuXXgtP6-YgNqDXd8MFOJTM-4olBtyzbApRXoFgOKYPktoKQRPFQT5Bo4JhMk</recordid><startdate>20130730</startdate><enddate>20130730</enddate><creator>Candeias da Silva, Carolina</creator><creator>Pedroso, José Luiz</creator><creator>Moraes, Fabiano Moulin de</creator><creator>Rivero, René Leandro M</creator><creator>Callegari, Fabiano Mesquita</creator><creator>Araujo, Francisco</creator><creator>Toso, Fabio Fieni</creator><creator>Stávale, João Norberto</creator><creator>Barsottini, Orlando Graziani Povoas</creator><general>by AAN Enterprises, Inc</general><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>7X8</scope><scope>7TK</scope></search><sort><creationdate>20130730</creationdate><title>Teaching NeuroImages: Rosai-Dorfman disease presenting with progressive early-onset cerebellar ataxia</title><author>Candeias da Silva, Carolina ; Pedroso, José Luiz ; Moraes, Fabiano Moulin de ; Rivero, René Leandro M ; Callegari, Fabiano Mesquita ; Araujo, Francisco ; Toso, Fabio Fieni ; Stávale, João Norberto ; Barsottini, Orlando Graziani Povoas</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-p2891-ea1743987369277be237449d37d5117d339263f2439ba894d9284061e593bbfd3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2013</creationdate><topic>Adolescent</topic><topic>Diagnosis, Differential</topic><topic>Disease Progression</topic><topic>Female</topic><topic>Histiocytosis, Sinus - complications</topic><topic>Histiocytosis, Sinus - diagnosis</topic><topic>Humans</topic><topic>Spinocerebellar Degenerations - diagnosis</topic><topic>Spinocerebellar Degenerations - etiology</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Candeias da Silva, Carolina</creatorcontrib><creatorcontrib>Pedroso, José Luiz</creatorcontrib><creatorcontrib>Moraes, Fabiano Moulin de</creatorcontrib><creatorcontrib>Rivero, René Leandro M</creatorcontrib><creatorcontrib>Callegari, Fabiano Mesquita</creatorcontrib><creatorcontrib>Araujo, Francisco</creatorcontrib><creatorcontrib>Toso, Fabio Fieni</creatorcontrib><creatorcontrib>Stávale, João Norberto</creatorcontrib><creatorcontrib>Barsottini, Orlando Graziani Povoas</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>MEDLINE - Academic</collection><collection>Neurosciences Abstracts</collection><jtitle>Neurology</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Candeias da Silva, Carolina</au><au>Pedroso, José Luiz</au><au>Moraes, Fabiano Moulin de</au><au>Rivero, René Leandro M</au><au>Callegari, Fabiano Mesquita</au><au>Araujo, Francisco</au><au>Toso, Fabio Fieni</au><au>Stávale, João Norberto</au><au>Barsottini, Orlando Graziani Povoas</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Teaching NeuroImages: Rosai-Dorfman disease presenting with progressive early-onset cerebellar ataxia</atitle><jtitle>Neurology</jtitle><addtitle>Neurology</addtitle><date>2013-07-30</date><risdate>2013</risdate><volume>81</volume><issue>5</issue><spage>e27</spage><epage>e28</epage><pages>e27-e28</pages><issn>0028-3878</issn><eissn>1526-632X</eissn><abstract>A 13-year-old girl presented with a 3-year history of progressive gait abnormality. She recently had a self-limited cervical lymphadenopathy. Neurologic examination showed brisk tendon reflexes and moderate ataxia. Brain MRI disclosed hyperintense lesions in the cerebellum and pons (figure, A). Spine MRI showed a heterogeneous lesion in the T12 vertebra (figure, B). Histopathology of the cervical lymph node confirmed Rosai-Dorfman disease (RDD) by showing emperipolesis (figure, C and D). The patient will be followed up in order to determine disease progression and therapy. RDD is a rare autoimmune histiocytic proliferative disorder first recognized in 1969. The CNS is involved in 5% of cases and generally mimics meningiomas. Bone erosion can be detected in the spine. Herein, we describe a rare CNS manifestation of RDD resembling a neurodegenerative ataxia.</abstract><cop>United States</cop><pub>by AAN Enterprises, Inc</pub><pmid>23897881</pmid><doi>10.1212/WNL.0b013e31829d85d2</doi></addata></record> |
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subjects | Adolescent Diagnosis, Differential Disease Progression Female Histiocytosis, Sinus - complications Histiocytosis, Sinus - diagnosis Humans Spinocerebellar Degenerations - diagnosis Spinocerebellar Degenerations - etiology |
title | Teaching NeuroImages: Rosai-Dorfman disease presenting with progressive early-onset cerebellar ataxia |
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