Quantitative MRI distinguishes different leukodystrophies and correlates with clinical measures.

IF 4.7 2区 医学 Q1 RADIOLOGY, NUCLEAR MEDICINE & MEDICAL IMAGING
European Radiology Pub Date : 2025-04-01 Epub Date: 2024-09-25 DOI:10.1007/s00330-024-11089-5
Menno D Stellingwerff, Murtadha L Al-Saady, Kwok-Shing Chan, Adam Dvorak, José P Marques, Shannon Kolind, Daphne H Schoenmakers, Romy van Voorst, Stefan D Roosendaal, Frederik Barkhof, Nicole I Wolf, Johannes Berkhof, Petra J W Pouwels, Marjo S van der Knaap
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引用次数: 0

Abstract

Objectives: The leukodystrophy "vanishing white matter" (VWM) and "metachromatic leukodystrophy" (MLD) affect the brain's white matter, but have very different underlying pathology. We aim to determine whether quantitative MRI reflects known neuropathological differences and correlates with clinical scores in these leukodystrophies.

Methods: VWM and MLD patients and controls were prospectively included between 2020 and 2023. Clinical scores were recorded. MRI at 3 T included multi-compartment relaxometry diffusion-informed myelin water imaging (MCR-DIMWI) and multi-echo T2-relaxation imaging with compressed sensing (METRICS) to determine myelin water fractions (MWF). Multi-shell diffusion-weighted data were used for diffusion tensor imaging measures and neurite orientation dispersion and density imaging (NODDI) analysis, which estimates neurite density index, orientation dispersion index, and free water fraction. As quantitative MRI measures are age-dependent, ratios between actual and age-expected MRI measures were calculated. We performed the multilevel analysis with subsequent post-hoc and correlation tests to assess differences between groups and clinico-radiological correlations.

Results: Sixteen control (age range: 2.3-61.3 years, 8 male), 37 VWM (2.4-56.5 years, 20 male), and 14 MLD (2.2-41.7 years, 6 male) subjects were included. Neurite density index and MWF were lower in patients than in controls (p < 0.001). Free water fraction was highest in VWM (p = 0.01), but similar to controls in MLD (p = 0.99). Changes in diffusion tensor imaging measures relative to controls were generally more pronounced in VWM than in MLD. In both patient groups, MCR-DIMWI MWF correlated strongest with clinical measures.

Conclusion: Quantitative MRI correlates to clinical measures and yields differential profiles in VWM and MLD, in line with differences in neuropathology.

Key points: Question Can quantitative MRI reflect known neuropathological differences and correlate with clinical scores for these leukodystrophies? Finding Quantitative MRI measures, e.g., MWF, neurite density index, and free water fraction differ between leukodystrophies and controls, in correspondence to known histological differences. Clinical relevance MRI techniques producing quantitative, biologically-specific, measures regarding the health of myelin and axons deliver more comprehensive information regarding pathological changes in leukodystrophies than current approaches, and are thus viable tools for monitoring patients and providing clinical trial outcome measures.

定量磁共振成像可区分不同的白质营养不良症,并与临床测量结果相关。
目的:白质营养不良症 "消失的白质"(VWM)和 "变色性白质营养不良症"(MLD)会影响大脑白质,但其潜在病理却截然不同。我们的目的是确定核磁共振成像定量分析是否反映了这些白营养不良症已知的神经病理学差异并与临床评分相关:方法:2020 年至 2023 年间,我们对 VWM 和 MLD 患者及对照组进行了前瞻性研究。记录临床评分。3T核磁共振成像包括多室弛豫测量弥散信息髓鞘水成像(MCR-DIMWI)和多回波T2松弛成像与压缩传感(METRICS),以确定髓鞘水分数(MWF)。多壳弥散加权数据用于弥散张量成像测量和神经元取向弥散和密度成像(NODDI)分析,从而估算神经元密度指数、取向弥散指数和游离水分数。由于核磁共振成像定量指标与年龄有关,我们计算了核磁共振成像实际指标与年龄预期指标之间的比率。我们进行了多层次分析,并随后进行了事后检验和相关检验,以评估组间差异和临床放射学相关性:结果:共纳入 16 名对照组受试者(年龄范围:2.3-61.3 岁,8 名男性)、37 名 VWM 受试者(2.4-56.5 岁,20 名男性)和 14 名 MLD 受试者(2.2-41.7 岁,6 名男性)。与对照组相比,患者的神经元密度指数和MWF较低(P 结论:MRI的定量分析结果显示,患者的神经元密度指数和MWF均低于对照组:磁共振成像定量分析与临床测量结果相关,并根据神经病理学的差异得出了VWM和MLD的不同特征:问题 MRI 定量成像能否反映已知的神经病理学差异,并与这些白质营养不良症的临床评分相关?研究结果 MRI 定量指标,如 MWF、神经元密度指数和游离水分数,在白质营养不良症和对照组之间存在差异,这与已知的组织学差异相符。与目前的方法相比,具有临床意义的核磁共振成像技术能对髓鞘和轴突的健康状况进行生物特异性定量测量,提供有关白质营养不良症病理变化的更全面信息,因此是监测患者和提供临床试验结果测量的可行工具。
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来源期刊
European Radiology
European Radiology 医学-核医学
CiteScore
11.60
自引率
8.50%
发文量
874
审稿时长
2-4 weeks
期刊介绍: European Radiology (ER) continuously updates scientific knowledge in radiology by publication of strong original articles and state-of-the-art reviews written by leading radiologists. A well balanced combination of review articles, original papers, short communications from European radiological congresses and information on society matters makes ER an indispensable source for current information in this field. This is the Journal of the European Society of Radiology, and the official journal of a number of societies. From 2004-2008 supplements to European Radiology were published under its companion, European Radiology Supplements, ISSN 1613-3749.
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