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引用次数: 0
摘要
杜兴氏肌肉萎缩症(DMD)是一种主要影响男性的 X 连锁隐性神经肌肉疾病,由肌营养不良蛋白基因突变引起。缺乏肌营养不良蛋白会导致骨骼肌进行性退化。治疗领域的最新进展突出表明,有必要在临床和研究环境中确定适当的结果测量方法,以评估行动自如和非行动自如的 DMD 患者的治疗效果。这对于准确评估新疗法和治疗效果至关重要。这项任务具有挑战性,因为功能测试表现可能受到不同患者特征的影响,包括神经发育的生理演变和疾病进展。我们介绍并讨论了现有 DMD 结果测量的特点(相关性、可量化性、有效性、客观性、可靠性、敏感性、特异性、精确性),包括数字工具和人工智能可能提供的新的潜在测量。
"If you cannot measure it, you cannot improve it". Outcome measures in Duchenne Muscular Dystrophy: current and future perspectives.
Duchenne Muscular Dystrophy (DMD) is an X-linked recessive neuromuscular disorder primarily affecting males, caused by mutations in the dystrophin gene. The absence of dystrophin protein leads to progressive skeletal muscle degeneration. Recent advances in the therapeutic landscape underscore the need to identify appropriate outcome measures to assess treatment efficacy in ambulant and non-ambulant DMD patients, across clinical and research settings. This is essential for accurately evaluating new treatments and attributing therapeutic benefits.It is crucial to establish a robust correlation between outcome scores and disease progression patterns. This task is challenging since functional test performance may be influenced by different patient's characteristics, including the physiological evolution of the neurodevelopment together with the disease progression. While widely used DMD outcomes such as the North Star Ambulatory Assessment, the 6-Minute Walking Test, the 4 stairs climbed, and the Performance of the Upper Limb exhibit reliability and validity, their clinical significance is influenced by the wide phenotype and progression variability of the disease.We present and discuss the features (relevance, quantifiability, validity, objectivity, reliability, sensitivity, specificity, precision) of available DMD outcome measures, including new potential measures that may be provided by digital tools and artificial intelligence.
期刊介绍:
Peer-reviewed and published quarterly, Acta Neurologica Belgicapresents original articles in the clinical and basic neurosciences, and also reports the proceedings and the abstracts of the scientific meetings of the different partner societies. The contents include commentaries, editorials, review articles, case reports, neuro-images of interest, book reviews and letters to the editor.
Acta Neurologica Belgica is the official journal of the following national societies:
Belgian Neurological Society
Belgian Society for Neuroscience
Belgian Society of Clinical Neurophysiology
Belgian Pediatric Neurology Society
Belgian Study Group of Multiple Sclerosis
Belgian Stroke Council
Belgian Headache Society
Belgian Study Group of Neuropathology