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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="2024-06-24" />
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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 (MGUS) 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 literature. This review article explores the pathogenesis, histopathological features, and characteristic neuroimaging findings of intracerebral LCDD. We emphasize the diverse imaging presentations of this disease, which can closely resemble other neurological 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 neuroradiological perspective, facilitating the recognition and differentiation of this challenging entity.

ABBREVIATIONS: LCDD, light chain deposition disease; ALD, amyloidoma." />
<meta name="DC.Contributor" content="Pranjal Rai" />
<meta name="DC.Contributor" content="Neetu Soni" />
<meta name="DC.Contributor" content="Girish Bathla" />
<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 (MGUS) 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 literature. This review article explores the pathogenesis, histopathological features, and characteristic neuroimaging findings of intracerebral LCDD. We emphasize the diverse imaging presentations of this disease, which can closely resemble other neurological 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 neuroradiological perspective, facilitating the recognition and differentiation of this challenging entity.&lt;/p&gt;&lt;p&gt;ABBREVIATIONS: LCDD, light chain deposition disease; ALD, amyloidoma.&lt;/p&gt;" />
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<meta name="citation_author" content="Pranjal Rai" />
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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 (MGUS) 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 literature. This review article explores the pathogenesis, histopathological features, and characteristic neuroimaging findings of intracerebral LCDD. We emphasize the diverse imaging presentations of this disease, which can closely resemble other neurological 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 neuroradiological perspective, facilitating the recognition and differentiation of this challenging entity.

ABBREVIATIONS: LCDD, light chain deposition disease; ALD, amyloidoma." />
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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 (MGUS) 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 literature. This review article explores the pathogenesis, histopathological features, and characteristic neuroimaging findings of intracerebral LCDD. We emphasize the diverse imaging presentations of this disease, which can closely resemble other neurological 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 neuroradiological perspective, facilitating the recognition and differentiation of this challenging entity.

ABBREVIATIONS: LCDD, light chain deposition disease; ALD, amyloidoma." />
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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">June 2024,  </span><span  class="highwire-cite-metadata-pages highwire-cite-metadata">ajnr.A8390; </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-108503--6417399373" 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) Tata Memorial Hospital, Parel, Mumbai 400012 (P.R) (2) Department of Radiology, Mayo Clinic, 4500 San Pablo Road, Jacksonville, FL 55902, USA (N.S, A.D, V.G, A.A), (3) Department of Radiology, Mayo Clinic, 200 1st Street SW, Rochester, MN 55902, USA (G.B), (4) University of Texas Southwestern Medical School, Dallas TX 75390 (K.R)</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'>From the (1) Tata Memorial Hospital, Parel, Mumbai 400012 (P.R) (2) Department of Radiology, Mayo Clinic, 4500 San Pablo Road, Jacksonville, FL 55902, USA (N.S, A.D, V.G, A.A), (3) Department of Radiology, Mayo Clinic, 200 1st Street SW, Rochester, MN 55902, USA (G.B), (4) University of Texas Southwestern Medical School, Dallas TX 75390 (K.R)</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-2"><div class="author-tooltip-name">Girish Bathla </div><div class="author-tooltip-affiliation"><span class="author-tooltip-text"><div class='author-affiliation'>From the (1) Tata Memorial Hospital, Parel, Mumbai 400012 (P.R) (2) Department of Radiology, Mayo Clinic, 4500 San Pablo Road, Jacksonville, FL 55902, USA (N.S, A.D, V.G, A.A), (3) Department of Radiology, Mayo Clinic, 200 1st Street SW, Rochester, MN 55902, USA (G.B), (4) University of Texas Southwestern Medical School, Dallas TX 75390 (K.R)</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 last"><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></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'>From the (1) Tata Memorial Hospital, Parel, Mumbai 400012 (P.R) (2) Department of Radiology, Mayo Clinic, 4500 San Pablo Road, Jacksonville, FL 55902, USA (N.S, A.D, V.G, A.A), (3) Department of Radiology, Mayo Clinic, 200 1st Street SW, Rochester, MN 55902, USA (G.B), (4) University of Texas Southwestern Medical School, Dallas TX 75390 (K.R)</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="" 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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 last"><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></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'>From the (1) Tata Memorial Hospital, Parel, Mumbai 400012 (P.R) (2) Department of Radiology, Mayo Clinic, 4500 San Pablo Road, Jacksonville, FL 55902, USA (N.S, A.D, V.G, A.A), (3) Department of Radiology, Mayo Clinic, 200 1st Street SW, Rochester, MN 55902, USA (G.B), (4) University of Texas Southwestern Medical School, Dallas TX 75390 (K.R)</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 last"><a href="/search/author1%3AVivek%2BGupta%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-6"><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) Tata Memorial Hospital, Parel, Mumbai 400012 (P.R) (2) Department of Radiology, Mayo Clinic, 4500 San Pablo Road, Jacksonville, FL 55902, USA (N.S, A.D, V.G, A.A), (3) Department of Radiology, Mayo Clinic, 200 1st Street SW, Rochester, MN 55902, USA (G.B), (4) University of Texas Southwestern Medical School, Dallas TX 75390 (K.R)</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="" 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