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
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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 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.

中枢神经系统轻链沉积病:中枢神经系统轻链沉积疾病:发病机制、影像学特征和放射学模拟综述》。
轻链沉积病(LCDD)是一种罕见的中枢神经系统疾病,其特征是单克隆免疫球蛋白轻链在不同器官的细胞外堆积。LCDD 通常继发于潜在的浆细胞异常,如意义未定的单克隆丙种球蛋白病(MGUS)或多发性骨髓瘤。不过,也有极少数病例可在无明显浆细胞障碍的情况下发生。肾脏、肝脏、肺和心脏是受影响最严重的器官。脑内LCDD,尤其是没有潜在浆细胞肿瘤的脑内LCDD,是一种极为罕见的疾病,文献中记载的病例非常有限。这篇综述文章探讨了脑内 LCDD 的发病机制、组织病理学特征和特征性神经影像学发现。我们强调这种疾病的影像学表现多种多样,可能与其他神经系统病变非常相似。识别这些潜在的模仿者对于避免误诊至关重要,尤其是在没有已知潜在浆细胞疾病的情况下。本文旨在从神经放射学的角度提供一个全面的概述,以便于识别和区分这一具有挑战性的实体:LCDD,轻链沉积症;ALD,淀粉样变性。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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