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AJNR Awards, New Junior Editors, and more. Read the latest AJNR updates

Review ArticleBrain Tumor Imaging

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 April 2025, 46 (4) 659-665; DOI: https://doi.org/10.3174/ajnr.A8390
Pranjal Rai
aFrom the Department of Radiology (P.R.), Tata Memorial Hospital, Mumbai, India
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Neetu Soni
bRadiology(N.S., G.B., A.D., V.G., A.A.), Mayo Clinic, Jacksonville, Florida
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Girish Bathla
bRadiology(N.S., G.B., A.D., V.G., A.A.), Mayo Clinic, Jacksonville, Florida
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Karuna Raj
cDivision of Neuroradiology (K.R.), University of Texas Southwestern Medical Center, Dallas, Texas
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Amit Desai
bRadiology(N.S., G.B., A.D., V.G., A.A.), Mayo Clinic, Jacksonville, Florida
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Vivek Gupta
bRadiology(N.S., G.B., A.D., V.G., A.A.), Mayo Clinic, Jacksonville, Florida
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Amit Agarwal
bRadiology(N.S., G.B., A.D., V.G., A.A.), Mayo Clinic, Jacksonville, Florida
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SUMMARY:

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.

ABBREVIATIONS:

ALD
amyloidoma
Ig
immunoglobulin
LC
light chain
LCDD
light-chain deposition disease
MIDD
monoclonal immunoglobulin deposition disease
  • © 2025 by American Journal of Neuroradiology
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American Journal of Neuroradiology: 46 (4)
American Journal of Neuroradiology
Vol. 46, Issue 4
1 Apr 2025
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Cite this article
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 Apr 2025, 46 (4) 659-665; DOI: 10.3174/ajnr.A8390

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CNS Light-Chain Deposition Disease: Imaging Review
Pranjal Rai, Neetu Soni, Girish Bathla, Karuna Raj, Amit Desai, Vivek Gupta, Amit Agarwal
American Journal of Neuroradiology Apr 2025, 46 (4) 659-665; DOI: 10.3174/ajnr.A8390
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