IRCCS Istituto delle Scienze Neurologiche di Bologna, UOC Clinica Neurologica, Bologna, Italy.
Dipartimento di Scienze Biomediche e Neuromotorie, Università di Bologna, Bologna, Italy.
Mov Disord. 2022 Jan;37(1):205-210. doi: 10.1002/mds.28809. Epub 2021 Oct 7.
Melanopsin retinal ganglion cell (mRGC)-mediated pupillary light reflex (PLR) abnormalities have been documented in several neurodegenerative disorders including Parkinson's disease. Overall, isolated rapid eye movement (REM) sleep behavior disorder (iRBD) represents the strongest prodromal risk factor for impending α-synucleinopathies.
To quantitatively compare PLR and mRGC-mediated contribution to PLR in 16 iRBD patients and 16 healthy controls.
iRBD and controls underwent extensive neuro-ophthalmological evaluation and chromatic pupillometry. In iRBD, PLR metrics were correlated with clinical variables and with additional biomarkers including REM atonia index (RAI), DaTscan, and presence of phosphorylated-α-synuclein (p-α-syn) deposition in skin biopsy.
We documented higher baseline pupil diameter and decreased rod-transient PLR amplitude in iRBD patients compared to controls. PLR rod-contribution correlated with RAI. Moreover, only iRBD patients with evidence of p-α-syn deposition at skin biopsy showed reduced PLR amplitude compared to controls.
The observed PLR abnormalities in iRBD might be considered as potential biomarkers for the risk stratification of phenoconversion of the disease. © 2021 The Authors. Movement Disorders published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society.
多项神经退行性疾病的研究已证实,包括帕金森病在内,视网膜中的黑视素能神经节细胞(mRGC)介导的瞳孔光反射(PLR)异常。总的来说,孤立性快速眼动睡眠行为障碍(iRBD)是即将发生α-突触核蛋白病的最强前驱风险因素。
定量比较 16 例 iRBD 患者和 16 例健康对照者的瞳孔光反射和 mRGC 介导的瞳孔光反射的差异。
iRBD 组和对照组均接受了全面的神经眼科评估和色觉瞳孔测量。在 iRBD 患者中,PLR 指标与临床变量以及其他生物标志物(包括 REM 弛缓指数、DaTscan 和皮肤活检中磷酸化-α-突触核蛋白(p-α-syn)沉积的存在)进行了相关性分析。
与对照组相比,iRBD 患者的基础瞳孔直径更大,视杆-瞬态 PLR 振幅降低。PLR 视杆贡献与 RAI 相关。此外,只有在皮肤活检中存在 p-α-syn 沉积的 iRBD 患者与对照组相比,PLR 振幅降低。
iRBD 中观察到的瞳孔光反射异常可被视为疾病表型转化风险分层的潜在生物标志物。© 2021 作者。运动障碍协会代表国际帕金森病和运动障碍协会由 Wiley 期刊出版公司出版。