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Improved Turnaround Times | Median time to first decision: 12 days

Review ArticleReview Articles

Light-chain Deposition Diseases of the CNS: Review of Pathogenesis, Imaging Features and Radiographic Mimics

Pranjal Rai, Neetu Soni, Girish Bathla, Karuna Raj, Amit Desai, Vivek Gupta and Amit Agarwal
American Journal of Neuroradiology June 2024, ajnr.A8390; DOI: https://doi.org/10.3174/ajnr.A8390
Pranjal Rai
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)
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Neetu Soni
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)
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Girish Bathla
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)
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Karuna Raj
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)
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Amit Desai
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)
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Vivek Gupta
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)
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Amit Agarwal
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)
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ABSTRACT

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.

Footnotes

  • No potential conflicts of interest for all authors.

  • © 2024 by American Journal of Neuroradiology
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Accepted Manuscript
Light-chain Deposition Diseases of the CNS: Review of Pathogenesis, Imaging Features and Radiographic Mimics
Pranjal Rai, Neetu Soni, Girish Bathla, Karuna Raj, Amit Desai, Vivek Gupta, Amit Agarwal
American Journal of Neuroradiology Jun 2024, ajnr.A8390; DOI: 10.3174/ajnr.A8390
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Cite this article
Accepted Manuscript
Pranjal Rai, Neetu Soni, Girish Bathla, Karuna Raj, Amit Desai, Vivek Gupta, Amit Agarwal
Light-chain Deposition Diseases of the CNS: Review of Pathogenesis, Imaging Features and Radiographic Mimics
American Journal of Neuroradiology Jun 2024, ajnr.A8390; DOI: 10.3174/ajnr.A8390

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