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MRI demonstrated nodular masses in the pons, peduncles, and cerebellar hemispheres, initially suggestive of metastatic disease. After craniectomy, dense histiocyte proliferation, Touton giant cells, and lymphocytic infiltrates supported Erdheim-Chester disease. Cytogenetics confirmed a BRAFV600 mutation, and vemurafenib was started. Six months later, follow-up MRI showed sequelae with reduced volume and persistent T2/FLAIR hyperintensity, with resolution of expansile posterior fossa lesions and regression of enhancement.",{"@graph":69,"@context":122},[70,84,105],{"@type":71,"itemListElement":72},"BreadcrumbList",[73,77,79,82],{"item":74,"name":75,"@type":76,"position":8},"https://docshare.wps.com","Home","ListItem",{"item":78,"name":9,"@type":76,"position":14},"https://docshare.wps.com/document/",{"item":80,"name":35,"@type":76,"position":81},"https://docshare.wps.com/document/healthcare/",3,{"item":83,"name":65,"@type":76,"position":19},"https://docshare.wps.com/document/erdheim-chester-manifesting-purely-as-a-neurological-disease-short-case-report-management-and-imaging/435860/",{"url":83,"name":65,"@type":85,"image":86,"author":91,"headline":65,"publisher":94,"fileFormat":97,"inLanguage":63,"description":67,"dateModified":98,"datePublished":99,"encodingFormat":97,"isAccessibleForFree":100,"interactionStatistic":101},"DigitalDocument",{"url":87,"@type":88,"width":89,"height":90},"https://docshare.wps.com/thumbnails/erdheim-chester-manifesting-purely-as-a-neurological-disease-short-case-report-management-and-imaging/435860.png","ImageObject",300,407,{"name":92,"@type":93},"Logic","Person",{"url":74,"name":95,"@type":96},"DocShare","Organization","application/pdf","2026-09-30","2026-09-29",true,{"@type":102,"interactionType":103,"userInteractionCount":14},"InteractionCounter",{"@type":104},"ViewAction",{"@type":106,"mainEntity":107},"FAQPage",[108,114,118],{"name":109,"@type":110,"acceptedAnswer":111},"What symptoms did the 60-year-old patient present with, and how long did they evolve?","Question",{"text":112,"@type":113},"He presented with vertigo, paraparesis, and dysarthria, which evolved over three months.","Answer",{"name":115,"@type":110,"acceptedAnswer":116},"How was the diagnosis of Erdheim-Chester disease confirmed?",{"text":117,"@type":113},"MRI showed nodular lesions, and craniectomy demonstrated dense histiocyte proliferation with Touton giant cells and lymphocytic infiltrate; cytogenetics confirmed a BRAFV600 mutation.",{"name":119,"@type":110,"acceptedAnswer":120},"What was the treatment and what did follow-up MRI show after six months?",{"text":121,"@type":113},"Vemurafenib was initiated. Six months later, MRI showed sequelae in the left cerebellar hemisphere with volumetric reduction and persistent T2/FLAIR hyperintensity, while expansile lesions resolved and enhancement regressed.","https://schema.org",{"og:url":83,"og:type":124,"og:title":65,"og:site_name":95,"og:description":67},"article",{"robots":126,"canonical":83},"index,follow",{"doc_id":128,"site_id":62},435860,1790741910,{"code":4,"msg":5,"data":131},{"doc_id":128,"user_id":132,"nickname":92,"user_avatar":133,"doc_module":4,"category_id":34,"category_name":35,"doc_title":65,"doc_description":67,"doc_content":134,"file_id":135,"file_url":136,"file_type":137,"file_size":138,"view_count":14,"is_deleted":4,"is_public":8,"is_downloadable":8,"audit_status":8,"page_count":14,"language":139,"language_code":63,"site_id":62,"html_lang":63,"table_of_contents":140,"faqs":141,"seo_title":142,"seo_description":67,"update_tm":143,"read_time":24},1099513958762,"https://ap-avatar.wpscdn.com/avatar/1000023916a998db790?x-image-process=image/resize,m_fixed,w_180,h_180&k=1784791008015729253","Article published online: 2025-06-21  \nNeuroimaging 1  \nErdheim-Chester manifesting purely as a neurological disease  \nLídia Laura Salvador Ramos 1 Iago Resende Carvalho2 Diogo Fernandes dos Santos3 Andrea de Martino Luppi2 Bruno de Carvalho Dornelas4  \n1 Universidade Federal de Uberlândia, Hospital de Clínicas, Departamento de Diagnóstico por Imagem, Uberlândia MG, Brazil.  \n2 Universidade Federal de Uberlândia, Faculdade de Medicina, Uberlândia MG, Brazil.  \n3 Universidade Federal de Uberlândia, Hospital de Clínicas, Departamento de Neurologia, Uberlândia MG, Brazil.  \n4 Universidade Federal de Uberlândia, Hospital de Clínicas, Departamento de Anatomia Patológica, Uberlândia MG, Brazil.  \nAddress for correspondence Bruno de Carvalho Dornelas (email: [dornelasbruno@gmail.com](dornelasbruno@gmail.com))  \nArq. Neuro-Psiquiatr. 2025;83(9):s00451809403 .  \nA 60-year-old man presented with vertigo, paraparesis, and dysarthria that evolved over 3 months. A magnetic resonance imaging (MRI) scan revealed nodular masses in the pons, peduncles, and cerebellar hemispheres, indicating a metastatic neoplasm ( ►Figure 1) . A craniectomy was performed, showing dense histiocyte proliferation, Touton giant cells, and lymphocytic infiltrate consistent with Erdheim-Chester disease ( ►Figure 2). The cytogenetic analysis confirmed a BRAFV600 mutation. Vemurafenib therapy was initiated. Six months later, the patient still exhibited dyslalia and left  \nhemiparesis, and a follow-up MRI scan was performed, revealing an area of sequelae ( ►Figure 3). Erdheim-Chester disease is a rare non-Langerhans histiocytosis, with neurological symptoms occurring in 41% of the cases. 1,2  \nAuthors’ Contributions  \nConceptualization: AML, BCD; Data curation: AML, BCD, LLR, ICR; Formal analysis: DS; Investigation: LLR; Supervision: BCD; Writing – original draft: LLR, ICR; Writing – review & editing: AML, DS.  \nFigure 1 Magnetic resonance imaging scan showing expansive intraparenchymal nodular lesions located in the posterior fossa, involving the pons, peduncles, and cerebellar hemispheres, presenting mild hypointense signal in the T2-weighted sequence (A) and vasogenic edema characterized by marginal fluid-attenuated inversion recovery (FLAIR) hypersignal (B) and intense contrast enhancement (C,D) .  \nreceived  \nMarch 7, 2025  \nreceived in its final form April 8, 2025  \naccepted  \nApril 17, 2025  \nDOI [https://doi.org/](https://doi.org/)[ ](https://doi.org/)10.1055/s-0045-1809403 . ISSN 0004-282X.  \nEditor-in-Chief: Hélio A. G. Teive.  \nAssociate Editor: Leandro Tavares Lucato.  \n© 2025 . The Author(s) .  \nThis is an open access article published by Thieme under the terms of the Creative Commons Attribution 4.0 International License, permitting copying and reproduction so long as the original work is given appropriate credit ([https://creativecommons.org/licenses/by/4.0/](https://creativecommons.org/licenses/by/4.0/))  \nThieme Revinter Publicações Ltda. , Rua Rego Freitas, 175, loja 1, República, São Paulo, SP, CEP 01220-010, Brazil  \n2  \nFigure 2 Erdheim-Chester disease in the cerebellum. (A) An infiltrate of histiocytes with a bland appearance and giant cells associated with a sparse lymphoplasmacytic infiltrate (hematoxylin and eosin staining; magnification: 4x) . (B) Histiocytes characterized by abundant foamy (xanthomatous) (arrows) cytoplasm with surrounding fibrosis (hematoxylin and eosin staining; magnification: 100x) . (C) Touton giant cells are frequently present(􀀁 ) (hematoxylin and eosin staining; magnification: 100x) .  \nFigure 3 Images after the treatment with vemurafenib showing an area of sequelae in the left cerebellar hemisphere, characterized by volumetric reduction and T2/FLAIR hyperintensity (A–C), with resolution of expansile lesions in the posterior fossa and regression of enhancement (D) .  \nConflict of Interest  \nThe authors have no conflict of interest to declare.  \nData Availability Statement  \nData is available from the corresponding author upon reaso","cbCaidHgFdn3MCNj","https://ap.wps.com/l/cbCaidHgFdn3MCNj","pdf",1667049,"English","# Case presentation\n## Imaging and diagnostic workup\n## Treatment and follow-up\n# Authors’ contributions\n# Figures\n## Figure 1\n## Figure 2\n## Figure 3\n# Declarations\n## Conflict of interest\n## Data availability\n## References","[{\"question\":\"What symptoms did the 60-year-old patient present with, and how long did they evolve?\",\"answer\":\"He presented with vertigo, paraparesis, and dysarthria, which evolved over three months.\"},{\"question\":\"How was the diagnosis of Erdheim-Chester disease confirmed?\",\"answer\":\"MRI showed nodular lesions, and craniectomy demonstrated dense histiocyte proliferation with Touton giant cells and lymphocytic infiltrate; cytogenetics confirmed a BRAFV600 mutation.\"},{\"question\":\"What was the treatment and what did follow-up MRI show after six months?\",\"answer\":\"Vemurafenib was initiated. Six months later, MRI showed sequelae in the left cerebellar hemisphere with volumetric reduction and persistent T2/FLAIR hyperintensity, while expansile lesions resolved and enhancement regressed.\"}]","Erdheim-Chester manifesting purely as a neurological disease - short case report - management and imaging | PDF",1790675590]