Ophthalmology Department, Centro Hospitalar Universitário do Porto, Porto, Portugal.
Nephrology Department, Centro Hospitalar Universitário do Porto, Porto, Portugal.
Eur J Ophthalmol. 2022 Jan;32(1):637-642. doi: 10.1177/1120672121997294. Epub 2021 Feb 18.
To automatically study the pupillary light reflex in patients with hereditary transthyretin-associated amyloidosis (hATTR).
Prospective cross-sectional observational study in patients with hATTR with unilateral scalloped iris. Pupillary light reflex of scalloped iris eyes (21 eyes) were compared with non-scalloped iris eyes (21 eyes, paired eyes of the same patients) and also with a control group of 20 healthy eyes, using static and dynamic pupillometry with the Metrovision MonPack One.
No patient presented evident neurological involvment of the cranial nerves. No significant differences were found in the pupillary diameters under standardized lighting conditions (static pupillometry) among groups. In dynamic pupillometry, the amplitude of contraction, the velocity of contraction and the velocity of dilation were statistically significantly lower in eyes with scalloped iris, comparing both with the contralateral non-scalloped iris eyes ( < 0.001 for all) and with eyes from healthy subjects ( < 0.05 for all).
A scalloped iris reflects a more advanced endocular hATTR and it is associated with an altered pupillary light reflex. Pupillometry may be a quick, simple, and portable test to objectively evaluate ocular amyloid deposition in hATTR eyes. Pupillary light reflex may not be reliable to evaluate neurological dysfunction in these patients.
自动研究遗传性转甲状腺素蛋白相关性淀粉样变性（hATTR）患者的瞳孔光反射。
对单侧扇形虹膜的 hATTR 患者进行前瞻性横断面观察性研究。扇形虹膜眼（21 只眼）的瞳孔光反射与非扇形虹膜眼（同一患者的配对眼，21 只眼）和 20 只健康眼对照组进行比较，使用 Metrovision MonPack One 进行静态和动态瞳孔测量。
没有患者出现明显的颅神经神经功能障碍。在标准化照明条件下（静态瞳孔测量），各组瞳孔直径无显著差异。在动态瞳孔测量中，扇形虹膜眼的收缩幅度、收缩速度和扩张速度均显著低于对侧非扇形虹膜眼（所有比较均为 < 0.001）和健康受试者眼（所有比较均为 < 0.05）。
扇形虹膜反映了更晚期的眼内 hATTR，并且与瞳孔光反射改变有关。瞳孔测量可能是一种快速、简单、便携的测试方法，可客观评估 hATTR 眼的眼部淀粉样沉积。瞳孔光反射可能不可靠，无法评估这些患者的神经功能障碍。
