用于评估肌萎缩侧索硬化症疾病严重程度的小纤维神经病：角膜共聚焦显微镜检查结果。
Small fiber neuropathy for assessment of disease severity in amyotrophic lateral sclerosis: corneal confocal microscopy findings.
机构信息
Department of Neurology, Peking University Third Hospital, 49 North Garden Road, Haidian District, Beijing, 100191, China.
Beijing Municipal Key Laboratory of Biomarker and Translational Research in Neurodegenerative Diseases, Beijing, China.
出版信息
Orphanet J Rare Dis. 2022 Jan 6;17(1):7. doi: 10.1186/s13023-021-02157-w.
BACKGROUND
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disorder with progressive motor system impairment, and recent evidence has identified the extra-motor involvement. Small fiber neuropathy reflecting by sensory and autonomic disturbances in ALS has been reported to accompany the motor damage. However, non-invasive assessment of this impairment and its application in disease evaluation of ALS is scarce. We aim to evaluate the use of corneal confocal microscopy (CCM) to non-invasively quantify the corneal small fiber neuropathy in ALS and explore its clinical value in assessing disease severity of ALS.
METHODS
Sixty-six patients with ALS and 64 healthy controls were included in this cross-sectional study. Participants underwent detailed clinical assessments and corneal imaging with in vivo CCM. Using ImageJ, the following parameters were quantified: corneal nerve length (IWL) and dendritic cell density (IWDC) in the inferior whorl region and corneal nerve fiber length (CNFL), nerve fiber density (CNFD), nerve branch density (CNBD), and dendritic cell density (CDC) in the peripheral region. Disease severity was evaluated using recognized scales.
RESULTS
Corneal nerve lengths (IWL and CNFL) were lower while dendritic cell densities (IWDC and CDC) were higher in patients with ALS than controls in peripheral and inferior whorl regions (p < 0.05). Additionally, corneal nerve complexity in the peripheral region was greater in patients than controls with higher CNBD (p = 0.040) and lower CNFD (p = 0.011). IWL was significantly associated with disease severity (p < 0.001) and progression (p = 0.002) in patients with ALS. Patients with bulbar involvement showed significantly lower IWL (p = 0.014) and higher IWDC (p = 0.043) than patients without bulbar involvement.
CONCLUSIONS
CCM quantified significant corneal neuropathy in ALS, and alterations in the inferior whorl region were closely associated with disease severity. CCM could serve as a noninvasive, objective imaging tool to detect corneal small fiber neuropathy for clinical evaluation in ALS.
背景
肌萎缩侧索硬化症（ALS）是一种致命的神经退行性疾病，表现为进行性运动系统损伤，最近的证据表明其存在运动外的累及。已有报道称 ALS 存在小纤维神经病，表现为感觉和自主神经紊乱，伴发运动损伤。然而，目前对于这种损伤的非侵入性评估及其在 ALS 疾病评估中的应用仍十分匮乏。我们旨在评估使用角膜共聚焦显微镜（CCM）无创性定量评估 ALS 中的角膜小纤维神经病，并探讨其在评估 ALS 疾病严重程度中的临床价值。
方法
本横断面研究纳入了 66 例 ALS 患者和 64 名健康对照者。所有参与者均接受详细的临床评估和 CCM 角膜成像。使用 ImageJ 定量分析下象限区域的角膜神经纤维长度（IWL）和树突状细胞密度（IWDC），以及周边区域的角膜神经纤维长度（CNFL）、神经纤维密度（CNFD）、神经分支密度（CNBD）和树突状细胞密度（CDC）。使用公认的量表评估疾病严重程度。
结果
与对照组相比，ALS 患者的角膜神经长度（IWL 和 CNFL）在周边和下象限均较低，而树突状细胞密度（IWDC 和 CDC）较高（p<0.05）。此外，与对照组相比，ALS 患者的角膜神经在周边区域更复杂，表现为更高的 CNBD（p=0.040）和更低的 CNFD（p=0.011）。IWL 与 ALS 患者的疾病严重程度（p<0.001）和进展（p=0.002）显著相关。有延髓受累的 ALS 患者的 IWL 明显低于无延髓受累的患者（p=0.014），而 IWDC 明显高于无延髓受累的患者（p=0.043）。
结论
CCM 定量评估了 ALS 中的显著角膜神经病，下象限的改变与疾病严重程度密切相关。CCM 可作为一种非侵入性、客观的成像工具，用于检测 ALS 中的角膜小纤维神经病，进行临床评估。
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