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German physicist Wilhelm Röntgen (1845–1923) was experimenting with electrical current when he discovered that a mysterious and invisible "ray" would pass through his flesh but leave an outline of his bones on a screen coated with a metal compound. In 1895, Röntgen made the first durable record of the internal parts of a living human: an "X-ray" image (as it came to be called) of his wife’s hand. Scientists worldwide quickly began their own experiments with...
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Magnetic resonance imaging (MRI) is a noninvasive medical imaging technique based on a phenomenon of nuclear physics discovered in the 1930s, in which matter exposed to magnetic fields and radio waves was found to emit radio signals. In 1970, a physician and researcher named Raymond Damadian noticed that malignant (cancerous) tissue gave off different signals than normal body tissue. He applied for a patent for the first MRI scanning device in clinical use by the early 1980s. The early MRI...
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一个自动化分析工具的扩散张力成像为基础的定量MRI在X链接上腺核缩 X-链接上腺核缩.

Eda G Kabak1, Fenna van Dodewaard2, Stefan D Roosendaal3

  • 1Department of Neurology and Pediatric Neurology, Emma Children's Hospital, Amsterdam Leukodystrophy Center, Amsterdam University Medical Center, Amsterdam, AZ, the Netherlands.

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概括

使用BrainQuant的定量MRI (qMRI) 有效地跟踪了X-链接上腺核缩症 (X-ALD) 患者的骨髓病变进展. 虽然没有区分大脑ALD (CALD),但辐射扩散性 (RD) - 全球已成为监测疾病进展的有希望的生物标志物.

关键词:
与X结合的 adrenoleukodystrophy与X结合的 adrenoleukodystrophy有关,而X结合的 adrenoleukodystrophy与X结合的 adrenoleukodystrophy有关,而X结合的 adrenoleukodystrophy与X结合的 adrenoleukodystrophy有关,而X结合的 adrenoleukodystrophy与X结合的 adrenoleukodystrophy有关.大脑 量子大脑大脑ALD是大脑的ALD.扩散张力成像的成像方法骨髓病变是一种骨髓病变.定量的MRI是指MRI的数量.辐射扩散性 辐射扩散性

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科学领域:

  • 神经成像是一种神经成像.
  • 神经学 神经学
  • 生物标志物 生物标志物

背景情况:

  • X链 adrenoleukodystrophy (X-ALD) 是一种由ABCD1变异引起的遗传性疾病,导致非常长链脂肪酸的积累.
  • 现象类型范围从大脑ALD (CALD) 到上腺神经病变 (AMN),影响神经功能.
  • 定量MRI (qMRI) 提供了客观疾病评估的潜力.

研究的目的:

  • 评估 BrainQuant 的自动化 qMRI 参数是否可以区分 CALD 与非 CALD 现象型.
  • 为了确定qMRI指标是否反映X-ALD患者骨髓病变的严重程度.
  • 评估qMRI参数的纵向变化及其与临床结果的相关性.

主要方法:

  • 患有X-ALD的成年男性每年接受神经学检查和脑部MRI (2015-2024年).
  • BrainQuant处理了扩散张力成像 (DTI) 来提取全球指标:分数异构 (FA),平均扩散率 (MD),轴向扩散率 (AD) 和辐射扩散率 (RD).
  • 患者根据洛斯得分和扩展残疾状况尺度 (EDSS) 分层;与临床得分 (SSPROM,6-MWT) 的相关性被分析.

主要成果:

  • 全球DTI指标没有显著区分CALD与非CALD.
  • 患有较高EDSS (>2) 的患者表现出明显较差的全球DTI值,与临床严重程度相关.
  • 在纵向上,全球辐射扩散率 (RD) 随着时间的推移显著增加,不管EDSS组.

结论:

  • BrainQuant qMRI有效地跟踪X-ALD中的骨髓病变的进展,但不能区分CALD和非CALD.
  • 全球辐射扩散率 (RD) 是监测X-ALD疾病进展的敏感生物标志物.
  • 自动化QMRI对X-ALD患者的客观评估和管理具有前景.