M Khamaysa, M El Mendili, V Marchand, G Querin, P-F Pradat
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引用次数: 0
摘要
肌萎缩侧索硬化症(ALS)是一种致命的神经退行性疾病,其特征是大脑皮层、脑干和脊髓中的运动神经元逐渐退化。这种退化导致肌肉无力,逐渐损害运动功能,最终导致呼吸衰竭。渐冻人症在临床、遗传和病理上的异质性,再加上缺乏可靠的生物标志物,给治疗试验的疗效带来了巨大挑战。尽管存在这些障碍,神经成像,尤其是脊髓成像,已成为一种很有前途的工具。它可以深入了解上下运动神经元的参与情况。脊髓定量成像具有促进早期诊断、准确监测疾病进展和完善临床试验设计的潜力。在本综述中,我们将在开发 ALS 脊柱成像生物标记物的大背景下探讨脊髓成像的效用。我们将重点放在 ALS 的诊断和预后生物标志物上,强调其在阐明该疾病潜在病理方面的关键作用。我们还讨论了现有的局限性和未来的研究途径,旨在弥合学术研究与临床实践和治疗试验应用之间的转化差距。
Quantitative spinal cord imaging: Early ALS diagnosis and monitoring of disease progression.
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disorder characterized by the progressive degeneration of motor neurons in the cortex, brainstem, and spinal cord. This degeneration leads to muscular weakness, progressively impairing motor functions and ultimately resulting in respiratory failure. The clinical, genetic, and pathological heterogeneity of ALS, combined with the absence of reliable biomarkers, significantly challenge the efficacy of therapeutic trials. Despite these hurdles, neuroimaging, and particularly spinal cord imaging, has emerged as a promising tool. It provides insights into the involvement of both upper and lower motor neurons. Quantitative spinal imaging has the potential to facilitate early diagnosis, enable accurate monitoring of disease progression, and refine the design of clinical trials. In this review, we explore the utility of spinal cord imaging within the broader context of developing spinal imaging biomarkers in ALS. We focus on a both diagnostic and prognostic biomarker in ALS, highlighting its pivotal role in elucidating the disease's underlying pathology. We also discuss the existing limitations and future avenues for research, aiming to bridge the translational gap between academic research and its application in clinical practice and therapeutic trials.
期刊介绍:
The first issue of the Revue Neurologique, featuring an original article by Jean-Martin Charcot, was published on February 28th, 1893. Six years later, the French Society of Neurology (SFN) adopted this journal as its official publication in the year of its foundation, 1899.
The Revue Neurologique was published throughout the 20th century without interruption and is indexed in all international databases (including Current Contents, Pubmed, Scopus). Ten annual issues provide original peer-reviewed clinical and research articles, and review articles giving up-to-date insights in all areas of neurology. The Revue Neurologique also publishes guidelines and recommendations.
The Revue Neurologique publishes original articles, brief reports, general reviews, editorials, and letters to the editor as well as correspondence concerning articles previously published in the journal in the correspondence column.