运动神经元疾病和中枢神经系统束病变:临床-放射学相关性和诊断方法。

IF 5.2 1区 医学 Q1 RADIOLOGY, NUCLEAR MEDICINE & MEDICAL IMAGING
Radiographics Pub Date : 2025-01-01 DOI:10.1148/rg.240067
Amit B Desai, Amit Agarwal, Alaa S Mohamed, Khalid H Mohamed, Erik H Middlebrooks, Alok A Bhatt, Vivek Gupta, Neeraj Kumar, Elia Sechi, Eoin P Flanagan, Sebastian López Chiriboga
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引用次数: 0

摘要

中枢神经系统中的白质束被组织成上行和下行通路,分别传递感觉输入和运动输出。束病,或这些束的损伤,可损害感觉或运动功能。运动神经元疾病是影响上或下运动神经元的病理过程。肌萎缩性侧索硬化症(ALS)是获得性运动神经元疾病最常见的形式。传统上,ALS会影响四肢、躯干、头颈部的上、下运动神经元。ALS有几种变体,其中一些仅影响上部运动神经元(如原发性侧索硬化),下部运动神经元(如进行性肌肉萎缩)或头颈部运动神经元(如进行性球性麻痹)。ALS的特征性影像学表现包括沿皮质脊髓束的脑内异常T2高信号,以及沿中央前回的皮质易感信号强度,称为“运动带”征象。脊髓性肌萎缩是一种不太常见的原发性运动神经元疾病,在图像上表现为脊髓前角萎缩,以及近端肌肉萎缩。除了纯粹的运动神经元疾病外,还有许多毒性和代谢性疾病、遗传性疾病、传染性疾病和免疫介导的疾病可继发影响皮质脊髓束(皮质脊髓束病变),产生上运动神经元损伤的症状。这些气管病变在MRI上表现为沿皮质脊髓束不同节段的t2高信号病变。综合临床症状、放射学和实验室检查结果的综合诊断方法对于区分这些不同的病症至关重要。©RSNA, 2024本文可获得补充材料。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Motor Neuron Diseases and Central Nervous System Tractopathies: Clinical-Radiologic Correlation and Diagnostic Approach.

White matter tracts within the central nervous system are organized into ascending and descending pathways that transmit sensory input and motor output, respectively. Tractopathy, or damage to these tracts, can impair sensory or motor functions. Motor neuron diseases are pathologic processes affecting the upper or lower motor neurons. Amyotrophic lateral sclerosis (ALS) is the most common form of acquired motor neuron disease. Traditionally, ALS has affected upper and lower motor neurons of the extremities, torso, and head and neck. There are several ALS variants, some of which affect only the upper motor neurons (eg, primary lateral sclerosis), lower motor neurons (eg, progressive muscular atrophy), or motor neurons of the head and neck (eg, progressive bulbar palsy). Characteristic imaging features of ALS include abnormal T2 hyperintensity within the brain along the corticospinal tract, as well as cortical susceptibility signal intensity along the precentral gyrus, termed the "motor band" sign. Spinal muscular atrophy is a less common primary motor neuron disease and appears on images as atrophy of the anterior horn of the spinal cord, as well as proximal muscle atrophy. In addition to pure motor neuron diseases, there are numerous toxic and metabolic conditions, genetic disorders, infectious diseases, and immune-mediated disorders that can secondarily affect the corticospinal tracts (corticospinal tractopathies), producing symptoms of upper motor neuron injury. These tractopathies are visible at MRI as T2-hyperintense lesions along varying segments of the corticospinal tract. A comprehensive diagnostic approach that integrates clinical symptoms with radiologic and laboratory findings is crucial to distinguish among these varied conditions. ©RSNA, 2024 Supplemental material is available for this article.

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来源期刊
Radiographics
Radiographics 医学-核医学
CiteScore
8.20
自引率
5.50%
发文量
224
审稿时长
4-8 weeks
期刊介绍: Launched by the Radiological Society of North America (RSNA) in 1981, RadioGraphics is one of the premier education journals in diagnostic radiology. Each bimonthly issue features 15–20 practice-focused articles spanning the full spectrum of radiologic subspecialties and addressing topics such as diagnostic imaging techniques, imaging features of a disease or group of diseases, radiologic-pathologic correlation, practice policy and quality initiatives, imaging physics, informatics, and lifelong learning. A special issue, a monograph focused on a single subspecialty or on a crossover topic of interest to multiple subspecialties, is published each October. Each issue offers more than a dozen opportunities to earn continuing medical education credits that qualify for AMA PRA Category 1 CreditTM and all online activities can be applied toward the ABR MOC Self-Assessment Requirement.
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