Singh Divya, Singhal Somya, Kanaujiya Vikas, Ranjan Ankita, Mani Vinita Elizabeth, Paliwal Vimal Kumar, Jain Vaibhav, Aishwarya Ankita, Agarwal Rachna
Department of Ophthalmology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, India.
Department of Neurology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, India.
Rom J Ophthalmol. 2025 Apr-Jun;69(2):200-207. doi: 10.22336/rjo.2025.32.
This study aims to evaluate various optical coherence tomography (OCT) parameters in patients diagnosed with amyotrophic lateral sclerosis (ALS).
Assessment of BCVA was done using Snellen charts, and subjective refraction was done to achieve a BCVA for distance and near. Measurement of intraocular pressure (IOP) was done with Goldman applanation tonometry. Stereoscopic fundus examination was performed using a 90D lens to assess the status of the optic nerve and retina, ruling out any ocular pathology. The patients were then subjected to OCT scanning to measure optic nerve head and macular parameters. Optical coherence tomography was performed using CIRRUS™ HD OCT (500-21822) (version 8.0.0.518) (Carl Zeiss Meditec, Dublin, CA, USA). The analyzed area was centered manually, and the absence of segmentation errors was confirmed for each scan.
RE Avg RNFL and LE Avg RNFL showed weak correlations with ALSFRS, indicated by Pearson Correlation coefficients of 0.073 and -0.026, respectively. The p-values (0.637 and 0.86) suggested that these correlations were not statistically significant. RE Avg GCL and LE Avg GCL, on the other hand, exhibited moderate positive correlations with ALSFRS scores, with correlation coefficients of 0.337 (RE) and 0.389 (LE). These correlations were statistically significant, as indicated by p-values of 0.021 and 0.006, respectively, suggesting a substantial association between GCL thickness and ALS functional outcomes.
All patients in our study were clinically diagnosed cases of ALS, as per the El Escorial criteria. Age group-wise analysis showed statistically significant thinning overall as well as quadrant-wise RNFL parameters in patients less than 50 years compared to age-matched controls, indicating that the pathological process occurring in larger motor neurons in ALS might also be happening in smaller sensory neurons of the retina, causing thinning, which was not due to age-related process. Although GCIPL thinning was occurring in our cases, though statistically not significant compared to control, the significant positive correlation observed between GCIPL and ALS functional outcome and between RNFL and GCIPL measurements highlighted the fact that though the axonal degeneration in retinal neurons might not be translating to the same extent in ganglion cells in ALS, the subtle thinning of GCIPL correlated strongly with functional disability in patients with ALS, implying better functional scores with higher values of GCIPL parameters.
In summary, GCL measurements in both eyes showed a notable relationship with ALSFRS, whereas RNFL did not appear to correlate significantly.
本研究旨在评估诊断为肌萎缩侧索硬化症（ALS）患者的各种光学相干断层扫描（OCT）参数。
使用斯内伦视力表评估最佳矫正视力（BCVA），并进行主观验光以获得远视力和近视力的BCVA。采用戈德曼压平眼压计测量眼压（IOP）。使用90D透镜进行立体眼底检查以评估视神经和视网膜的状态，排除任何眼部病变。然后对患者进行OCT扫描以测量视神经乳头和黄斑参数。使用CIRRUS™ HD OCT（500 - 21822）（版本8.0.0.518）（卡尔蔡司医疗技术公司，美国加利福尼亚州都柏林）进行光学相干断层扫描。手动将分析区域居中，并确认每次扫描均无分割错误。
右眼平均视网膜神经纤维层（RNFL）和左眼平均RNFL与肌萎缩侧索硬化功能评分量表（ALSFRS）呈弱相关性，皮尔逊相关系数分别为0.073和 - 0.026。p值（0.637和0.86）表明这些相关性无统计学意义。另一方面，右眼平均神经节细胞层（GCL）和左眼平均GCL与ALSFRS评分呈中度正相关，相关系数分别为0.337（右眼）和0.389（左眼）。这些相关性具有统计学意义，p值分别为0.021和0.006，表明GCL厚度与ALS功能结果之间存在显著关联。
根据埃尔埃斯科里亚尔标准，我们研究中的所有患者均为临床诊断的ALS病例。按年龄组分析显示，与年龄匹配的对照组相比，小于50岁的患者总体上以及象限性RNFL参数在统计学上有显著变薄，这表明ALS中较大运动神经元发生的病理过程可能也发生在视网膜较小的感觉神经元中，导致变薄，这并非与年龄相关的过程。尽管在我们的病例中发生了神经节细胞内层（GCIPL）变薄，与对照组相比虽无统计学意义，但在GCIPL与ALS功能结果之间以及RNFL与GCIPL测量之间观察到的显著正相关突出了这样一个事实，即尽管视网膜神经元中的轴突退化在ALS的神经节细胞中可能没有同等程度的表现，但GCIPL的细微变薄与ALS患者的功能残疾密切相关，这意味着GCIPL参数值越高，功能评分越好。
总之，双眼的GCL测量与ALSFRS显示出显著关系，而RNFL似乎没有显著相关性。
