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<meta name="DC.Title" content="Light-Chain Deposition Diseases of the CNS: Review of Pathogenesis, Imaging Features, and Radiographic Mimics" />
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<meta name="DC.Date" content="2024-10-31" />
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<meta name="DC.Description" content="Light-chain deposition disease (LCDD) is a rare CNS disorder characterized by the extracellular accumulation of monoclonal immunoglobulin light chains in various organs. LCDD typically arises secondary to an underlying plasma cell dyscrasia, such as monoclonal gammopathy of undetermined significance or multiple myeloma. However, rare cases can occur in the absence of a demonstrable plasma cell disorder. The kidneys, liver, lungs, and heart are the most affected organs. Intracerebral LCDD, particularly without an underlying plasma cell neoplasm, represents an exceedingly uncommon entity with limited documented cases in the literature. This review article explores the pathogenesis, histopathologic features, and characteristic neuroimaging findings of intracerebral LCDD. We emphasize the diverse imaging presentations of this disease, which can closely resemble other neurologic pathologies. Recognizing these potential mimics is crucial for avoiding misdiagnosis, especially in the absence of a known underlying plasma cell disorder. This article aims to provide a comprehensive overview from a neuroradiologic perspective, facilitating the recognition and differentiation of this challenging entity.

ALD
:   amyloidoma
Ig
:   immunoglobulin
LC
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LCDD
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MIDD
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<meta name="DC.Contributor" content="Pranjal Rai" />
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<meta name="DC.Contributor" content="Karuna Raj" />
<meta name="DC.Contributor" content="Amit Desai" />
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&lt;p&gt;Light-chain deposition disease (LCDD) is a rare CNS disorder characterized by the extracellular accumulation of monoclonal immunoglobulin light chains in various organs. LCDD typically arises secondary to an underlying plasma cell dyscrasia, such as monoclonal gammopathy of undetermined significance or multiple myeloma. However, rare cases can occur in the absence of a demonstrable plasma cell disorder. The kidneys, liver, lungs, and heart are the most affected organs. Intracerebral LCDD, particularly without an underlying plasma cell neoplasm, represents an exceedingly uncommon entity with limited documented cases in the literature. This review article explores the pathogenesis, histopathologic features, and characteristic neuroimaging findings of intracerebral LCDD. We emphasize the diverse imaging presentations of this disease, which can closely resemble other neurologic pathologies. Recognizing these potential mimics is crucial for avoiding misdiagnosis, especially in the absence of a known underlying plasma cell disorder. This article aims to provide a comprehensive overview from a neuroradiologic perspective, facilitating the recognition and differentiation of this challenging entity.&lt;/p&gt;" />
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<meta name="twitter:title" content="Light-Chain Deposition Diseases of the CNS: Review of Pathogenesis, Imaging Features, and Radiographic Mimics" />
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<meta name="twitter:description" content="Light-chain deposition disease (LCDD) is a rare CNS disorder characterized by the extracellular accumulation of monoclonal immunoglobulin light chains in various organs. LCDD typically arises secondary to an underlying plasma cell dyscrasia, such as monoclonal gammopathy of undetermined significance or multiple myeloma. However, rare cases can occur in the absence of a demonstrable plasma cell disorder. The kidneys, liver, lungs, and heart are the most affected organs. Intracerebral LCDD, particularly without an underlying plasma cell neoplasm, represents an exceedingly uncommon entity with limited documented cases in the literature. This review article explores the pathogenesis, histopathologic features, and characteristic neuroimaging findings of intracerebral LCDD. We emphasize the diverse imaging presentations of this disease, which can closely resemble other neurologic pathologies. Recognizing these potential mimics is crucial for avoiding misdiagnosis, especially in the absence of a known underlying plasma cell disorder. This article aims to provide a comprehensive overview from a neuroradiologic perspective, facilitating the recognition and differentiation of this challenging entity.

ALD
:   amyloidoma
Ig
:   immunoglobulin
LC
:   light chain
LCDD
:   light-chain deposition disease
MIDD
:   monoclonal immunoglobulin deposition disease" />
<meta name="og-title" property="og:title" content="Light-Chain Deposition Diseases of the CNS: Review of Pathogenesis, Imaging Features, and Radiographic Mimics" />
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<meta name="og-description" property="og:description" content="Light-chain deposition disease (LCDD) is a rare CNS disorder characterized by the extracellular accumulation of monoclonal immunoglobulin light chains in various organs. LCDD typically arises secondary to an underlying plasma cell dyscrasia, such as monoclonal gammopathy of undetermined significance or multiple myeloma. However, rare cases can occur in the absence of a demonstrable plasma cell disorder. The kidneys, liver, lungs, and heart are the most affected organs. Intracerebral LCDD, particularly without an underlying plasma cell neoplasm, represents an exceedingly uncommon entity with limited documented cases in the literature. This review article explores the pathogenesis, histopathologic features, and characteristic neuroimaging findings of intracerebral LCDD. We emphasize the diverse imaging presentations of this disease, which can closely resemble other neurologic pathologies. Recognizing these potential mimics is crucial for avoiding misdiagnosis, especially in the absence of a known underlying plasma cell disorder. This article aims to provide a comprehensive overview from a neuroradiologic perspective, facilitating the recognition and differentiation of this challenging entity.

ALD
:   amyloidoma
Ig
:   immunoglobulin
LC
:   light chain
LCDD
:   light-chain deposition disease
MIDD
:   monoclonal immunoglobulin deposition disease" />
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    <div class="highwire-article-citation highwire-citation-type-highwire-article node111665--5" data-node-nid="111665" id="top-node-111665--61058074719" data-pisa="ajnr;ajnr.A8390v2" data-pisa-master="ajnr;ajnr.A8390" data-apath="/ajnr/early/2024/10/31/ajnr.A8390.atom" data-hw-author-tooltip-instance="highwire_author_tooltip"><div class="highwire-cite highwire-cite-highwire-article highwire-citation-jcore-article-title-complete clearfix has-author-tooltip highwire-citation-highwire-article-top-a">
  
  
      <div class="highwire-cite-overline"><span  class="highwire-cite-metadata-article-type highwire-cite-metadata">Review Article</span><span class="separator-pipe"></span><span  class="highwire-cite-metadata-art-cat highwire-cite-metadata"><span class="wrapper">REVIEW ARTICLE</span></span></div>
  
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      <h1 class="highwire-cite-title" id="page-title">Light-Chain Deposition Diseases of the CNS: Review of Pathogenesis, Imaging Features, and Radiographic Mimics</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">Neetu Soni</span>, <span class="highwire-citation-author" data-delta="2">Girish Bathla</span>, <span class="highwire-citation-author" data-delta="3">Karuna Raj</span>, <span class="highwire-citation-author" data-delta="4">Amit Desai</span>, <span class="highwire-citation-author" data-delta="5">Vivek Gupta</span> and <span class="highwire-citation-author" data-delta="6">Amit Agarwal</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">October 2024,  </span><span  class="highwire-cite-metadata-doi highwire-cite-metadata">DOI: https://doi.org/10.3174/ajnr.A8390 </span></div>
  
  
  
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<div id="hw-article-author-popups-top-node-111665--61058074719" 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'><span class='nlm-sup'>a</span>From the Department of Radiology (P.R.), Tata Memorial Hospital, Mumbai, India</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">Neetu Soni </div><div class="author-tooltip-affiliation"><span class="author-tooltip-text"><div class='author-affiliation'><span class='nlm-sup'>b</span>Radiology(N.S., G.B., A.D., V.G., A.A.), Mayo Clinic, Jacksonville, Florida</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"><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><li class="author-orcid-link last"><a href="https://orcid.org/0000-0003-2082-1634" target="_blank" class="" data-icon-position="" data-hide-link-title="0">ORCID record for Neetu Soni</a></li></ul></div><div class="author-tooltip-2"><div class="author-tooltip-name">Girish Bathla </div><div class="author-tooltip-affiliation"><span class="author-tooltip-text"><div class='author-affiliation'><span class='nlm-sup'>b</span>Radiology(N.S., G.B., A.D., V.G., A.A.), Mayo Clinic, Jacksonville, Florida</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=Girish%2BBathla%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=Bathla%20G&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"><a href="/search/author1%3AGirish%2BBathla%2B" rel="nofollow" class="" data-icon-position="" data-hide-link-title="0">Search for this author on this site</a></li><li class="author-orcid-link last"><a href="https://orcid.org/0000-0003-3170-242X" target="_blank" class="" data-icon-position="" data-hide-link-title="0">ORCID record for Girish Bathla</a></li></ul></div><div class="author-tooltip-3"><div class="author-tooltip-name">Karuna Raj </div><div class="author-tooltip-affiliation"><span class="author-tooltip-text"><div class='author-affiliation'><span class='nlm-sup'>c</span>Division of Neuroradiology (K.R.), University of Texas Southwestern Medical Center, Dallas, Texas</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=Karuna%2BRaj%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=Raj%20K&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%3AKaruna%2BRaj%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 Desai </div><div class="author-tooltip-affiliation"><span class="author-tooltip-text"><div class='author-affiliation'><span class='nlm-sup'>b</span>Radiology(N.S., G.B., A.D., V.G., A.A.), Mayo Clinic, Jacksonville, Florida</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%2BDesai%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=Desai%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"><a href="/search/author1%3AAmit%2BDesai%2B" rel="nofollow" class="" data-icon-position="" data-hide-link-title="0">Search for this author on this site</a></li><li class="author-orcid-link last"><a href="https://orcid.org/0000-0002-3073-6823" target="_blank" class="" data-icon-position="" data-hide-link-title="0">ORCID record for Amit Desai</a></li></ul></div><div class="author-tooltip-5"><div class="author-tooltip-name">Vivek Gupta </div><div class="author-tooltip-affiliation"><span class="author-tooltip-text"><div class='author-affiliation'><span class='nlm-sup'>b</span>Radiology(N.S., G.B., A.D., V.G., A.A.), Mayo Clinic, Jacksonville, Florida</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=Vivek%2BGupta%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=Gupta%20V&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"><a href="/search/author1%3AVivek%2BGupta%2B" rel="nofollow" class="" data-icon-position="" data-hide-link-title="0">Search for 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    <div class="highwire-markup"><div xmlns="http://www.w3.org/1999/xhtml" xmlns:xhtml="http://www.w3.org/1999/xhtml" class="section ref-list" id="ref-list-1"><h2 class="">References</h2><ol class="cit-list ref-use-labels"><li><span class="ref-label">1.</span><a class="rev-xref-ref" href="#xref-ref-1-1" title="View reference 1. in text" id="ref-1">↵</a><div class="cit ref-cit ref-journal" id="cit-ajnr.A8390v2.1" data-doi="10.1016/0002-9343(76)90440-X"><div class="cit-metadata"><ol class="cit-auth-list"><li><span class="cit-auth"><span class="cit-name-surname">Randall</span>  <span class="cit-name-given-names">RE</span></span>, </li><li><span class="cit-auth"><span class="cit-name-surname">Williamson</span>  <span class="cit-name-given-names">WC</span>   <span class="cit-name-suffix">Jr</span>.</span>, </li><li><span class="cit-auth"><span class="cit-name-surname">Mullinax</span>  <span class="cit-name-given-names">F</span></span>, <span class="cit-etal">et al</span></li></ol><cite>. <span class="cit-article-title">Manifestations of systemic light chain deposition</span>. <abbr class="cit-jnl-abbrev">Am J Med</abbr> <span class="cit-pub-date">1976</span>;<span class="cit-vol">60</span>:<span class="cit-fpage">293</span>–<span class="cit-lpage">99</span><span class="cit-pub-id-sep cit-pub-id-doi-sep"> </span><span class="cit-pub-id cit-pub-id-doi"><span class="cit-pub-id-scheme-doi">doi:</span>10.1016/0002-9343(76)90440-x</span><span class="cit-pub-id-sep cit-pub-id-pmid-sep"> </span><span class="cit-pub-id cit-pub-id-pmid"><span class="cit-pub-id-scheme-pmid">pmid:</span>814812</span></cite></div><div class="cit-extra"><a href="/lookup/external-ref?access_num=10.1016/0002-9343(76)90440-X&amp;link_type=DOI" class="cit-ref-sprinkles cit-ref-sprinkles-doi cit-ref-sprinkles-crossref"><span>CrossRef</span></a><a href="/lookup/external-ref?access_num=814812&amp;link_type=MED&amp;atom=%2Fajnr%2Fearly%2F2024%2F10%2F31%2Fajnr.A8390.atom" class="cit-ref-sprinkles cit-ref-sprinkles-medline"><span>PubMed</span></a><a href="/lookup/external-ref?access_num=A1976BF07800016&amp;link_type=ISI" class="cit-ref-sprinkles cit-ref-sprinkles-newisilink cit-ref-sprinkles-webofscience"><span>Web of Science</span></a></div></div></li><li><span class="ref-label">2.</span><a class="rev-xref-ref" href="#xref-ref-2-1" title="View reference 2. in text" id="ref-2">↵</a><div class="cit ref-cit ref-journal" id="cit-ajnr.A8390v2.2" data-doi="10.1309/3GECPW2402F6V8EK"><div class="cit-metadata"><ol class="cit-auth-list"><li><span class="cit-auth"><span class="cit-name-surname">Khoor</span>  <span class="cit-name-given-names">A</span></span>, </li><li><span class="cit-auth"><span class="cit-name-surname">Myers</span>  <span class="cit-name-given-names">JL</span></span>, </li><li><span class="cit-auth"><span class="cit-name-surname">Tazelaar</span>  <span class="cit-name-given-names">HD</span></span>, <span class="cit-etal">et al</span></li></ol><cite>. <span class="cit-article-title">Amyloid-like pulmonary nodules, including localized light-chain deposition: clinicopathologic analysis of three cases</span>. <abbr class="cit-jnl-abbrev">Am J Clin Pathol</abbr> <span class="cit-pub-date">2004</span>;<span class="cit-vol">121</span>:<span class="cit-fpage">200</span>–<span class="cit-lpage">04</span><span class="cit-pub-id-sep cit-pub-id-doi-sep"> </span><span class="cit-pub-id cit-pub-id-doi"><span class="cit-pub-id-scheme-doi">doi:</span>10.1309/3GEC-PW24-02F6-V8EK</span><span class="cit-pub-id-sep cit-pub-id-pmid-sep"> </span><span class="cit-pub-id cit-pub-id-pmid"><span class="cit-pub-id-scheme-pmid">pmid:</span>14983932</span></cite></div><div class="cit-extra"><a href="/lookup/external-ref?access_num=10.1309/3GECPW2402F6V8EK&amp;link_type=DOI" class="cit-ref-sprinkles cit-ref-sprinkles-doi cit-ref-sprinkles-crossref"><span>CrossRef</span></a><a 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non-amyloidotic monoclonal immunoglobulin light chain deposits (‘aggregoma’): presenting feature of B-cell dyscrasia in three cases with immunohistochemical and biochemical analyses</span>. <abbr class="cit-jnl-abbrev">Br J Haematol</abbr> <span class="cit-pub-date">2002</span>;<span class="cit-vol">119</span>:<span class="cit-fpage">62</span>–<span class="cit-lpage">69</span><span class="cit-pub-id-sep cit-pub-id-doi-sep"> </span><span class="cit-pub-id cit-pub-id-doi"><span class="cit-pub-id-scheme-doi">doi:</span>10.1046/j.1365-2141.2002.03781.x</span><span class="cit-pub-id-sep cit-pub-id-pmid-sep"> </span><span class="cit-pub-id cit-pub-id-pmid"><span class="cit-pub-id-scheme-pmid">pmid:</span>12358904</span></cite></div><div class="cit-extra"><a href="/lookup/external-ref?access_num=10.1046/j.1365-2141.2002.03781.x&amp;link_type=DOI" class="cit-ref-sprinkles cit-ref-sprinkles-doi cit-ref-sprinkles-crossref"><span>CrossRef</span></a><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">Oct 2024, </span><span  class="highwire-cite-metadata-doi highwire-cite-metadata"><span class="label">DOI:</span> 10.3174/ajnr.A8390 </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">Neetu</span> <span class="nlm-surname">Soni</span></span>, <span class="highwire-citation-author" data-delta="2"><span class="nlm-given-names">Girish</span> <span class="nlm-surname">Bathla</span></span>, <span class="highwire-citation-author" data-delta="3"><span class="nlm-given-names">Karuna</span> <span class="nlm-surname">Raj</span></span>, <span class="highwire-citation-author" data-delta="4"><span class="nlm-given-names">Amit</span> <span class="nlm-surname">Desai</span></span>, <span class="highwire-citation-author" data-delta="5"><span class="nlm-given-names">Vivek</span> <span class="nlm-surname">Gupta</span></span>, <span class="highwire-citation-author" data-delta="6"><span class="nlm-given-names">Amit</span> <span class="nlm-surname">Agarwal</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">Oct 2024, </span><span  class="highwire-cite-metadata-doi highwire-cite-metadata"><span class="label">DOI:</span> 10.3174/ajnr.A8390 </span></div>
  
  
  
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