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<meta name="DC.Title" content="Neuroimaging Spectrum of Erdheim-Chester Disease: An Image-based Review" />
<meta name="DC.Identifier" content="10.3174/ajnr.A8599" />
<meta name="DC.Date" content="2024-11-22" />
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<meta name="DC.Description" content="Erdheim-Chester Disease (ECD) is a rare, multisystem histiocytic disorder characterized by its variable clinical presentations. Central Nervous System (CNS) involvement is observed in approximately half of ECD patients (up to 76% in some series), and often carries a poorer prognosis. While CNS involvement may remain asymptomatic, others may experience a range of neurological symptoms, including cognitive decline, neuropsychiatric disturbances, motor deficits, cranial or peripheral neuropathies, and endocrine abnormalities.

Neuroimaging findings in CNS-ECD are diverse, including neurodegeneration manifesting as cerebral or cerebellar volume loss, solitary or multifocal variably enhancing intraparenchymal lesions along the neuroaxis, meningeal infiltration, involvement of the Hypothalamo-pituitary axis, perivascular sheathing or basal ganglia lesions. Other well documented sites of involvement include the craniofacial region, orbits and spine. Awareness of these findings is relevant, not only because of the non-specific nature of these findings, but also given the high proportion of CNS involvement in ECD as well as the higher mortality associated with CNS involvement.

This review provides an in-depth overview of the various manifestations of CNS involvement in ECD and their imaging features, along with a brief overview of the differential considerations which include other histiocytic and non-histiocytic processes.

ABBREVIATIONS: ECD＝Erdheim-Chester Disease; RDD＝Rosai-Dorfman Disease; LCH= Langerhans cell histiocytosis;" />
<meta name="DC.Contributor" content="Pranjal Rai" />
<meta name="DC.Contributor" content="Haydern J. Swartz" />
<meta name="DC.Contributor" content="Neetu Soni" />
<meta name="DC.Contributor" content="John C Benson" />
<meta name="DC.Contributor" content="Amit Agarwal" />
<meta name="DC.Contributor" content="Steven A Messina" />
<meta name="DC.Contributor" content="Paul J Farnsworth" />
<meta name="DC.Contributor" content="Carrie M Carr" />
<meta name="DC.Contributor" content="Girish Bathla" />
<meta name="article:published_time" content="2024-11-22" />
<meta name="article:section" content="REVIEW ARTICLE" />
<meta name="citation_title" content="Neuroimaging Spectrum of Erdheim-Chester Disease: An Image-based Review" />
<meta name="citation_abstract" lang="en" content="&lt;h3&gt;ABSTRACT&lt;/h3&gt;
&lt;p&gt;Erdheim-Chester Disease (ECD) is a rare, multisystem histiocytic disorder characterized by its variable clinical presentations. Central Nervous System (CNS) involvement is observed in approximately half of ECD patients (up to 76% in some series), and often carries a poorer prognosis. While CNS involvement may remain asymptomatic, others may experience a range of neurological symptoms, including cognitive decline, neuropsychiatric disturbances, motor deficits, cranial or peripheral neuropathies, and endocrine abnormalities.&lt;/p&gt;&lt;p&gt;Neuroimaging findings in CNS-ECD are diverse, including neurodegeneration manifesting as cerebral or cerebellar volume loss, solitary or multifocal variably enhancing intraparenchymal lesions along the neuroaxis, meningeal infiltration, involvement of the Hypothalamo-pituitary axis, perivascular sheathing or basal ganglia lesions. Other well documented sites of involvement include the craniofacial region, orbits and spine. Awareness of these findings is relevant, not only because of the non-specific nature of these findings, but also given the high proportion of CNS involvement in ECD as well as the higher mortality associated with CNS involvement.&lt;/p&gt;&lt;p&gt;This review provides an in-depth overview of the various manifestations of CNS involvement in ECD and their imaging features, along with a brief overview of the differential considerations which include other histiocytic and non-histiocytic processes.&lt;/p&gt;&lt;p&gt;ABBREVIATIONS: ECD＝Erdheim-Chester Disease; RDD＝Rosai-Dorfman Disease; LCH= Langerhans cell histiocytosis;&lt;/p&gt;" />
<meta name="citation_journal_title" content="American Journal of Neuroradiology" />
<meta name="citation_publisher" content="American Journal of Neuroradiology" />
<meta name="citation_publication_date" content="2024/11/22" />
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<meta name="citation_author" content="Pranjal Rai" />
<meta name="citation_author_institution" content="From the (1) Department of Radiology, Tata Memorial Hospital, Parel, Mumbai 400012 (P.R.), (2) Department of Radiology, Mayo Clinic, Rochester, MN (H.J.S, J.C.B, S.A.M, P.J.F, C.M.C, G.B.), and (3) Department of Radiology, Mayo Clinic, 4500 San Pablo Road, Jacksonville, FL 55902 (N.S., A.A), and The authors have no relevant disclosures of interest." />
<meta name="citation_author" content="Haydern J. Swartz" />
<meta name="citation_author_institution" content="From the (1) Department of Radiology, Tata Memorial Hospital, Parel, Mumbai 400012 (P.R.), (2) Department of Radiology, Mayo Clinic, Rochester, MN (H.J.S, J.C.B, S.A.M, P.J.F, C.M.C, G.B.), and (3) Department of Radiology, Mayo Clinic, 4500 San Pablo Road, Jacksonville, FL 55902 (N.S., A.A), and The authors have no relevant disclosures of interest." />
<meta name="citation_author" content="Neetu Soni" />
<meta name="citation_author_institution" content="From the (1) Department of Radiology, Tata Memorial Hospital, Parel, Mumbai 400012 (P.R.), (2) Department of Radiology, Mayo Clinic, Rochester, MN (H.J.S, J.C.B, S.A.M, P.J.F, C.M.C, G.B.), and (3) Department of Radiology, Mayo Clinic, 4500 San Pablo Road, Jacksonville, FL 55902 (N.S., A.A), and The authors have no relevant disclosures of interest." />
<meta name="citation_author" content="John C Benson" />
<meta name="citation_author_institution" content="From the (1) Department of Radiology, Tata Memorial Hospital, Parel, Mumbai 400012 (P.R.), (2) Department of Radiology, Mayo Clinic, Rochester, MN (H.J.S, J.C.B, S.A.M, P.J.F, C.M.C, G.B.), and (3) Department of Radiology, Mayo Clinic, 4500 San Pablo Road, Jacksonville, FL 55902 (N.S., A.A), and The authors have no relevant disclosures of interest." />
<meta name="citation_author" content="Amit Agarwal" />
<meta name="citation_author_institution" content="From the (1) Department of Radiology, Tata Memorial Hospital, Parel, Mumbai 400012 (P.R.), (2) Department of Radiology, Mayo Clinic, Rochester, MN (H.J.S, J.C.B, S.A.M, P.J.F, C.M.C, G.B.), and (3) Department of Radiology, Mayo Clinic, 4500 San Pablo Road, Jacksonville, FL 55902 (N.S., A.A), and The authors have no relevant disclosures of interest." />
<meta name="citation_author" content="Steven A Messina" />
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<meta name="citation_author" content="Paul J Farnsworth" />
<meta name="citation_author_institution" content="From the (1) Department of Radiology, Tata Memorial Hospital, Parel, Mumbai 400012 (P.R.), (2) Department of Radiology, Mayo Clinic, Rochester, MN (H.J.S, J.C.B, S.A.M, P.J.F, C.M.C, G.B.), and (3) Department of Radiology, Mayo Clinic, 4500 San Pablo Road, Jacksonville, FL 55902 (N.S., A.A), and The authors have no relevant disclosures of interest." />
<meta name="citation_author" content="Carrie M Carr" />
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<meta name="citation_author" content="Girish Bathla" />
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<meta name="twitter:title" content="Neuroimaging Spectrum of Erdheim-Chester Disease: An Image-based Review" />
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<meta name="twitter:description" content="Erdheim-Chester Disease (ECD) is a rare, multisystem histiocytic disorder characterized by its variable clinical presentations. Central Nervous System (CNS) involvement is observed in approximately half of ECD patients (up to 76% in some series), and often carries a poorer prognosis. While CNS involvement may remain asymptomatic, others may experience a range of neurological symptoms, including cognitive decline, neuropsychiatric disturbances, motor deficits, cranial or peripheral neuropathies, and endocrine abnormalities.

Neuroimaging findings in CNS-ECD are diverse, including neurodegeneration manifesting as cerebral or cerebellar volume loss, solitary or multifocal variably enhancing intraparenchymal lesions along the neuroaxis, meningeal infiltration, involvement of the Hypothalamo-pituitary axis, perivascular sheathing or basal ganglia lesions. Other well documented sites of involvement include the craniofacial region, orbits and spine. Awareness of these findings is relevant, not only because of the non-specific nature of these findings, but also given the high proportion of CNS involvement in ECD as well as the higher mortality associated with CNS involvement.

This review provides an in-depth overview of the various manifestations of CNS involvement in ECD and their imaging features, along with a brief overview of the differential considerations which include other histiocytic and non-histiocytic processes.

ABBREVIATIONS: ECD＝Erdheim-Chester Disease; RDD＝Rosai-Dorfman Disease; LCH= Langerhans cell histiocytosis;" />
<meta name="og-title" property="og:title" content="Neuroimaging Spectrum of Erdheim-Chester Disease: An Image-based Review" />
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<meta name="og-description" property="og:description" content="Erdheim-Chester Disease (ECD) is a rare, multisystem histiocytic disorder characterized by its variable clinical presentations. Central Nervous System (CNS) involvement is observed in approximately half of ECD patients (up to 76% in some series), and often carries a poorer prognosis. While CNS involvement may remain asymptomatic, others may experience a range of neurological symptoms, including cognitive decline, neuropsychiatric disturbances, motor deficits, cranial or peripheral neuropathies, and endocrine abnormalities.

Neuroimaging findings in CNS-ECD are diverse, including neurodegeneration manifesting as cerebral or cerebellar volume loss, solitary or multifocal variably enhancing intraparenchymal lesions along the neuroaxis, meningeal infiltration, involvement of the Hypothalamo-pituitary axis, perivascular sheathing or basal ganglia lesions. Other well documented sites of involvement include the craniofacial region, orbits and spine. Awareness of these findings is relevant, not only because of the non-specific nature of these findings, but also given the high proportion of CNS involvement in ECD as well as the higher mortality associated with CNS involvement.

This review provides an in-depth overview of the various manifestations of CNS involvement in ECD and their imaging features, along with a brief overview of the differential considerations which include other histiocytic and non-histiocytic processes.

ABBREVIATIONS: ECD＝Erdheim-Chester Disease; RDD＝Rosai-Dorfman Disease; LCH= Langerhans cell histiocytosis;" />
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      <h1 class="highwire-cite-title" id="page-title">Neuroimaging Spectrum of Erdheim-Chester Disease: An Image-based Review</h1>  
    	<div class="highwire-cite-authors"><span  class="highwire-citation-authors"><span class="highwire-citation-author first" data-delta="0">Pranjal Rai</span>, <span class="highwire-citation-author" data-delta="1">Haydern J. Swartz</span>, <span class="highwire-citation-author" data-delta="2">Neetu Soni</span>, <span class="highwire-citation-author" data-delta="3">John C Benson</span>, <span class="highwire-citation-author" data-delta="4">Amit Agarwal</span>, <span class="highwire-citation-author" data-delta="5">Steven A Messina</span>, <span class="highwire-citation-author" data-delta="6">Paul J Farnsworth</span>, <span class="highwire-citation-author" data-delta="7">Carrie M Carr</span> and <span class="highwire-citation-author" data-delta="8">Girish Bathla</span></span></div>
  
    	<div class="highwire-cite-metadata"><span  class="highwire-cite-metadata-journal highwire-cite-metadata">American Journal of Neuroradiology </span><span  class="highwire-cite-metadata-date highwire-cite-metadata">November 2024,  </span><span  class="highwire-cite-metadata-pages highwire-cite-metadata">ajnr.A8599; </span><span  class="highwire-cite-metadata-doi highwire-cite-metadata">DOI: https://doi.org/10.3174/ajnr.A8599 </span></div>
  
  
  
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<div id="hw-article-author-popups-top-node-111991--61745238351" style="display: none;"><div class="author-tooltip-0"><div class="author-tooltip-name">Pranjal Rai </div><div class="author-tooltip-affiliation"><span class="author-tooltip-text"><div class='author-affiliation'>From the (1) Department of Radiology, Tata Memorial Hospital, Parel, Mumbai 400012 (P.R.), (2) Department of Radiology, Mayo Clinic, Rochester, MN (H.J.S, J.C.B, S.A.M, P.J.F, C.M.C, G.B.), and (3) Department of Radiology, Mayo Clinic, 4500 San Pablo Road, Jacksonville, FL 55902 (N.S., A.A), and The authors have no relevant disclosures of interest.</div></span></div><ul class="author-tooltip-find-more"><li class="author-tooltip-gs-link first"><a href="/lookup/google-scholar?link_type=googlescholar&amp;gs_type=author&amp;author%5B0%5D=Pranjal%2BRai%2B" target="_blank" class="" data-icon-position="" data-hide-link-title="0">Find this author on Google Scholar</a></li><li class="author-tooltip-pubmed-link"><a href="/lookup/external-ref?access_num=Rai%20P&amp;link_type=AUTHORSEARCH" target="_blank" class="" data-icon-position="" data-hide-link-title="0">Find this author on PubMed</a></li><li class="author-site-search-link last"><a href="/search/author1%3APranjal%2BRai%2B" rel="nofollow" class="" data-icon-position="" data-hide-link-title="0">Search for this author on this site</a></li></ul></div><div class="author-tooltip-1"><div class="author-tooltip-name">Haydern J. Swartz </div><div class="author-tooltip-affiliation"><span class="author-tooltip-text"><div class='author-affiliation'>From the (1) Department of Radiology, Tata Memorial Hospital, Parel, Mumbai 400012 (P.R.), (2) Department of Radiology, Mayo Clinic, Rochester, MN (H.J.S, J.C.B, S.A.M, P.J.F, C.M.C, G.B.), and (3) Department of Radiology, Mayo Clinic, 4500 San Pablo Road, Jacksonville, FL 55902 (N.S., A.A), and The authors have no relevant disclosures of interest.</div></span></div><ul class="author-tooltip-find-more"><li class="author-tooltip-gs-link first"><a href="/lookup/google-scholar?link_type=googlescholar&amp;gs_type=author&amp;author%5B0%5D=Haydern%2BJ.%2BSwartz%2B" target="_blank" class="" data-icon-position="" data-hide-link-title="0">Find this author on Google Scholar</a></li><li class="author-tooltip-pubmed-link"><a href="/lookup/external-ref?access_num=Swartz%20HJ&amp;link_type=AUTHORSEARCH" target="_blank" class="" data-icon-position="" data-hide-link-title="0">Find this author on PubMed</a></li><li class="author-site-search-link last"><a href="/search/author1%3AHaydern%2BJ.%2BSwartz%2B" rel="nofollow" class="" data-icon-position="" data-hide-link-title="0">Search for this author on this site</a></li></ul></div><div class="author-tooltip-2"><div class="author-tooltip-name">Neetu Soni </div><div class="author-tooltip-affiliation"><span class="author-tooltip-text"><div class='author-affiliation'>From the (1) Department of Radiology, Tata Memorial Hospital, Parel, Mumbai 400012 (P.R.), (2) Department of Radiology, Mayo Clinic, Rochester, MN (H.J.S, J.C.B, S.A.M, P.J.F, C.M.C, G.B.), and (3) Department of Radiology, Mayo Clinic, 4500 San Pablo Road, Jacksonville, FL 55902 (N.S., A.A), and The authors have no relevant disclosures of interest.</div></span></div><ul class="author-tooltip-find-more"><li class="author-tooltip-gs-link first"><a href="/lookup/google-scholar?link_type=googlescholar&amp;gs_type=author&amp;author%5B0%5D=Neetu%2BSoni%2B" target="_blank" class="" data-icon-position="" data-hide-link-title="0">Find this author on Google Scholar</a></li><li class="author-tooltip-pubmed-link"><a href="/lookup/external-ref?access_num=Soni%20N&amp;link_type=AUTHORSEARCH" target="_blank" class="" data-icon-position="" data-hide-link-title="0">Find this author on PubMed</a></li><li class="author-site-search-link last"><a href="/search/author1%3ANeetu%2BSoni%2B" rel="nofollow" class="" data-icon-position="" data-hide-link-title="0">Search for this author on this site</a></li></ul></div><div class="author-tooltip-3"><div class="author-tooltip-name">John C Benson </div><div class="author-tooltip-affiliation"><span class="author-tooltip-text"><div class='author-affiliation'>From the (1) Department of Radiology, Tata Memorial Hospital, Parel, Mumbai 400012 (P.R.), (2) Department of Radiology, Mayo Clinic, Rochester, MN (H.J.S, J.C.B, S.A.M, P.J.F, C.M.C, G.B.), and (3) Department of Radiology, Mayo Clinic, 4500 San Pablo Road, Jacksonville, FL 55902 (N.S., A.A), and The authors have no relevant disclosures of interest.</div></span></div><ul class="author-tooltip-find-more"><li class="author-tooltip-gs-link first"><a href="/lookup/google-scholar?link_type=googlescholar&amp;gs_type=author&amp;author%5B0%5D=John%2BC%2BBenson%2B" target="_blank" class="" data-icon-position="" data-hide-link-title="0">Find this author on Google Scholar</a></li><li class="author-tooltip-pubmed-link"><a href="/lookup/external-ref?access_num=Benson%20JC&amp;link_type=AUTHORSEARCH" target="_blank" class="" data-icon-position="" data-hide-link-title="0">Find this author on PubMed</a></li><li class="author-site-search-link last"><a href="/search/author1%3AJohn%2BC%2BBenson%2B" rel="nofollow" class="" data-icon-position="" data-hide-link-title="0">Search for this author on this site</a></li></ul></div><div class="author-tooltip-4"><div class="author-tooltip-name">Amit Agarwal </div><div class="author-tooltip-affiliation"><span class="author-tooltip-text"><div class='author-affiliation'>From the (1) Department of Radiology, Tata Memorial Hospital, Parel, Mumbai 400012 (P.R.), (2) Department of Radiology, Mayo Clinic, Rochester, MN (H.J.S, J.C.B, S.A.M, P.J.F, C.M.C, G.B.), and (3) Department of Radiology, Mayo Clinic, 4500 San Pablo Road, Jacksonville, FL 55902 (N.S., A.A), and The authors have no relevant disclosures of interest.</div></span></div><ul class="author-tooltip-find-more"><li class="author-tooltip-gs-link first"><a href="/lookup/google-scholar?link_type=googlescholar&amp;gs_type=author&amp;author%5B0%5D=Amit%2BAgarwal%2B" target="_blank" class="" data-icon-position="" data-hide-link-title="0">Find this author on Google Scholar</a></li><li class="author-tooltip-pubmed-link"><a href="/lookup/external-ref?access_num=Agarwal%20A&amp;link_type=AUTHORSEARCH" target="_blank" class="" data-icon-position="" data-hide-link-title="0">Find this author on PubMed</a></li><li class="author-site-search-link 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and The authors have no relevant disclosures of interest.</div></span></div><ul class="author-tooltip-find-more"><li class="author-tooltip-gs-link first"><a href="/lookup/google-scholar?link_type=googlescholar&amp;gs_type=author&amp;author%5B0%5D=Paul%2BJ%2BFarnsworth%2B" target="_blank" class="" data-icon-position="" data-hide-link-title="0">Find this author on Google Scholar</a></li><li class="author-tooltip-pubmed-link"><a href="/lookup/external-ref?access_num=Farnsworth%20PJ&amp;link_type=AUTHORSEARCH" target="_blank" class="" data-icon-position="" data-hide-link-title="0">Find this author on PubMed</a></li><li class="author-site-search-link last"><a href="/search/author1%3APaul%2BJ%2BFarnsworth%2B" rel="nofollow" class="" data-icon-position="" data-hide-link-title="0">Search for this author on this site</a></li></ul></div><div class="author-tooltip-7"><div class="author-tooltip-name">Carrie M Carr </div><div class="author-tooltip-affiliation"><span class="author-tooltip-text"><div class='author-affiliation'>From the (1) Department of Radiology, Tata Memorial Hospital, Parel, Mumbai 400012 (P.R.), (2) Department of Radiology, Mayo Clinic, Rochester, MN (H.J.S, J.C.B, S.A.M, P.J.F, C.M.C, G.B.), and (3) Department of Radiology, Mayo Clinic, 4500 San Pablo Road, Jacksonville, FL 55902 (N.S., A.A), and The authors have no relevant disclosures of interest.</div></span></div><ul class="author-tooltip-find-more"><li class="author-tooltip-gs-link first"><a href="/lookup/google-scholar?link_type=googlescholar&amp;gs_type=author&amp;author%5B0%5D=Carrie%2BM%2BCarr%2B" target="_blank" class="" data-icon-position="" data-hide-link-title="0">Find this author on Google Scholar</a></li><li class="author-tooltip-pubmed-link"><a href="/lookup/external-ref?access_num=Carr%20CM&amp;link_type=AUTHORSEARCH" target="_blank" class="" data-icon-position="" data-hide-link-title="0">Find this author on PubMed</a></li><li class="author-site-search-link last"><a 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    	<div class="highwire-cite-metadata"><span  class="highwire-cite-metadata-journal highwire-cite-metadata">American Journal of Neuroradiology </span><span  class="highwire-cite-metadata-date highwire-cite-metadata">Nov 2024, </span><span  class="highwire-cite-metadata-pages highwire-cite-metadata">ajnr.A8599; </span><span  class="highwire-cite-metadata-doi highwire-cite-metadata"><span class="label">DOI:</span> 10.3174/ajnr.A8599 </span></div>
  
  
  
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    	<div class="highwire-cite-authors"><span  class="highwire-citation-authors"><span class="highwire-citation-author first" data-delta="0"><span class="nlm-given-names">Pranjal</span> <span class="nlm-surname">Rai</span></span>, <span class="highwire-citation-author" data-delta="1"><span class="nlm-given-names">Haydern J.</span> <span class="nlm-surname">Swartz</span></span>, <span class="highwire-citation-author" data-delta="2"><span class="nlm-given-names">Neetu</span> <span class="nlm-surname">Soni</span></span>, <span class="highwire-citation-author" data-delta="3"><span class="nlm-given-names">John C</span> <span class="nlm-surname">Benson</span></span>, <span class="highwire-citation-author" data-delta="4"><span class="nlm-given-names">Amit</span> <span class="nlm-surname">Agarwal</span></span>, <span class="highwire-citation-author" data-delta="5"><span class="nlm-given-names">Steven A</span> <span class="nlm-surname">Messina</span></span>, <span class="highwire-citation-author" data-delta="6"><span class="nlm-given-names">Paul J</span> <span class="nlm-surname">Farnsworth</span></span>, <span class="highwire-citation-author" data-delta="7"><span class="nlm-given-names">Carrie M</span> <span class="nlm-surname">Carr</span></span>, <span class="highwire-citation-author" data-delta="8"><span class="nlm-given-names">Girish</span> <span class="nlm-surname">Bathla</span></span></span></div>
  
    	<div class="highwire-cite-metadata"><span  class="highwire-cite-metadata-journal highwire-cite-metadata">American Journal of Neuroradiology </span><span  class="highwire-cite-metadata-date highwire-cite-metadata">Nov 2024, </span><span  class="highwire-cite-metadata-pages highwire-cite-metadata">ajnr.A8599; </span><span  class="highwire-cite-metadata-doi highwire-cite-metadata"><span class="label">DOI:</span> 10.3174/ajnr.A8599 </span></div>
  
  
  
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