Abstract：
OBJECTIVE: Amyotrophic lateral sclerosis (ALS) is a severe motor neuron disorder. Diagnosis is challenging due to its clinical heterogeneity and the absence of definitive diagnostic tools, leading to delays averaging between 9.1 and 27 months. In vivo corneal confocal microscopy, assessing the sub-basal nerve plexus of the cornea, has been proposed as a potential biomarker for ALS. We aimed to determine whether the assessment of corneal nerves using in vivo confocal microscopy can serve as an imaging biomarker for ALS.
METHODS: A single-centre prospective case-control study was conducted in France from September 2021 to March 2023 including patients with ALS according to the revised EI Escorial criteria. The corneal sub-basal nerve plexus was analysed using in vivo confocal microscopy. An automated algorithm (ACCMetrics) was used to evaluate corneal parameters: nerve fibre density, nerve branch density, nerve fibre length, nerve fibre area, nerve total branch density, nerve fibre width, and nerve fractal dimension.
RESULTS: Twenty-two patients with ALS and 30 controls were included. No significant differences were found between ALS and control groups for all corneal parameters (p > 0.05). Corneal sensitivity did not differ between groups, and no correlation was identified between corneal nerve parameters and ALS disease duration, severity and rate of progression (p > 0.05).
CONCLUSIONS: The present study does not support the use of in vivo corneal confocal microscopy as an early diagnostic or prognostic tool for ALS. Further research, especially longitudinal investigations, is needed to understand any potential corneal innervation changes as ALS progresses.
METHODS: A single-centre prospective case-control study was conducted in France from September 2021 to March 2023 including patients with ALS according to the revised EI Escorial criteria. The corneal sub-basal nerve plexus was analysed using in vivo confocal microscopy. An automated algorithm (ACCMetrics) was used to evaluate corneal parameters: nerve fibre density, nerve branch density, nerve fibre length, nerve fibre area, nerve total branch density, nerve fibre width, and nerve fractal dimension.
RESULTS: Twenty-two patients with ALS and 30 controls were included. No significant differences were found between ALS and control groups for all corneal parameters (p > 0.05). Corneal sensitivity did not differ between groups, and no correlation was identified between corneal nerve parameters and ALS disease duration, severity and rate of progression (p > 0.05).
CONCLUSIONS: The present study does not support the use of in vivo corneal confocal microscopy as an early diagnostic or prognostic tool for ALS. Further research, especially longitudinal investigations, is needed to understand any potential corneal innervation changes as ALS progresses.
摘要：
目的：肌萎缩侧索硬化症（ALS）是一种严重的运动神经元疾病。由于其临床异质性和缺乏明确的诊断工具，诊断具有挑战性。导致平均在9.1到27个月之间的延误。体内角膜共聚焦显微镜，评估角膜基底下神经丛，已被提议作为ALS的潜在生物标志物。我们旨在确定使用体内共聚焦显微镜评估角膜神经是否可以作为ALS的成像生物标志物。
方法：根据修订的EIEscorial标准，于2021年9月至2023年3月在法国进行了一项单中心前瞻性病例对照研究，包括ALS患者。使用体内共聚焦显微镜分析角膜基底下神经丛。自动算法（ACCMetrics）用于评估角膜参数：神经纤维密度，神经分支密度,神经纤维长度，神经纤维区，神经总分支密度，神经纤维宽度，和神经分形维数。
结果：纳入了22例ALS患者和30例对照。ALS组和对照组的所有角膜参数均无显著差异(p>0.05)。各组之间角膜敏感性没有差异，角膜神经参数和ALS疾病持续时间之间没有相关性，严重程度和进展率（p>0.05）。
结论：本研究不支持使用体内角膜共聚焦显微镜作为ALS的早期诊断或预后工具。进一步研究,尤其是纵向调查，随着ALS的进展，需要了解任何潜在的角膜神经支配变化。
方法：根据修订的EIEscorial标准，于2021年9月至2023年3月在法国进行了一项单中心前瞻性病例对照研究，包括ALS患者。使用体内共聚焦显微镜分析角膜基底下神经丛。自动算法（ACCMetrics）用于评估角膜参数：神经纤维密度，神经分支密度,神经纤维长度，神经纤维区，神经总分支密度，神经纤维宽度，和神经分形维数。
结果：纳入了22例ALS患者和30例对照。ALS组和对照组的所有角膜参数均无显著差异(p>0.05)。各组之间角膜敏感性没有差异，角膜神经参数和ALS疾病持续时间之间没有相关性，严重程度和进展率（p>0.05）。
结论：本研究不支持使用体内角膜共聚焦显微镜作为ALS的早期诊断或预后工具。进一步研究,尤其是纵向调查，随着ALS的进展，需要了解任何潜在的角膜神经支配变化。
