Department of Neuromedicine, Nil Ratan Sircar Medical College and Hospital, Kolkata, India.
Department of Neurology, Patna Medical College, Patna, India.
Indian J Ophthalmol. 2023 Feb;71(2):369-378. doi: 10.4103/ijo.IJO_1870_22.
The retinal involvement of amyotrophic lateral sclerosis (ALS) is a novel idea about a possible correlation between retinal nerve fiber layer (RNFL) thickness in different spectra of ALS patients. Finding the association of RNFL with disease duration and severity will help identify a novel noninvasive biomarker.
The study was designed as a cross-sectional study and was conducted with a suitable proforma. We included the ALS cases based on the revised El Escorial criteria. Healthy controls were age and gender matched. We used the revised ALS functional rating scale (ALSFRS-R) to assess the operational status of the patients. We measured RNFL thickness in the four quadrants with spectral-domain optical coherence tomography (OCT) and analyzed it.
We included 30 cases (60 eyes) and 10 healthy controls (20 eyes) having a mean (standard deviation [SD]) age of 49.5 (11.1) years with a median of 50 years, and a majority of them (65%) were middle aged (between 41 and 60 years). We found statistically significant differences in RNFL thicknesses between ALS patients and healthy controls. On segmental analysis, the right eye superior and nasal quadrants and the left eye superior, inferior, and nasal quadrants were significantly affected, along with a gross asymmetry found between the left and right eyes among ALS patients. There was a significant decrease in average RNFL thickness in definite ALS patients than probable ALS patients, with significantly reduced average RNFL thickness in moderate to severe ALS patients. On correlation analysis, disease duration showed a good negative correlation with bilateral average RNFL thickness, and the ALSFRS-R score demonstrated a good positive correlation with bilateral average RNFL thickness, which was statistically significant. Thus, a reduced bilateral RNFL thickness is associated with a decreased ALSFRS-R score.
The retinal changes can serve as a marker for diagnosing and monitoring patients with ALS.
肌萎缩侧索硬化症（ALS）的视网膜受累是一种关于不同光谱 ALS 患者的视网膜神经纤维层（RNFL）厚度之间可能存在相关性的新观点。发现 RNFL 与疾病持续时间和严重程度的关联将有助于确定一种新的无创生物标志物。
该研究设计为横断面研究，并使用合适的表格进行。我们根据修订后的埃尔埃斯克里尔标准纳入 ALS 病例。健康对照组按年龄和性别匹配。我们使用修订后的 ALS 功能评定量表（ALSFRS-R）评估患者的操作状态。我们使用频域光相干断层扫描（OCT）测量四个象限的 RNFL 厚度并进行分析。
我们纳入了 30 例（60 只眼）和 10 例健康对照者（20 只眼），平均（标准差[SD]）年龄为 49.5（11.1）岁，中位数为 50 岁，其中大多数（65%）为中年（41 至 60 岁之间）。我们发现 ALS 患者和健康对照组之间的 RNFL 厚度存在统计学显著差异。在节段性分析中，右眼上象限和鼻象限以及左眼上、下和鼻象限均受到显著影响，并且 ALS 患者的左眼和右眼之间存在明显的不对称。明确的 ALS 患者的平均 RNFL 厚度明显低于可能的 ALS 患者，中度至重度 ALS 患者的平均 RNFL 厚度明显降低。在相关分析中，疾病持续时间与双侧平均 RNFL 厚度呈良好的负相关，ALSFRS-R 评分与双侧平均 RNFL 厚度呈良好的正相关，具有统计学意义。因此，双侧 RNFL 厚度减少与 ALSFRS-R 评分降低相关。
视网膜变化可以作为诊断和监测 ALS 患者的标志物。
