Steiner Oliver L, Klostermann Fabian
Department of Neurology, Motor and Cognition Group, Charité-Universitätsmedizin Berlin, Freie Universität Berlin and Humboldt-Universität zu Berlin, Campus Benjamin Franklin (CBF), 12203 Berlin, Germany.
Berlin School of Mind and Brain, Humboldt-Universität zu Berlin, 10099 Berlin, Germany.
Brain Commun. 2024 Jun 26;6(4):fcae206. doi: 10.1093/braincomms/fcae206. eCollection 2024.
Chronic inflammatory demyelinating polyneuropathy (CIDP) compromises functions of the peripheral nervous system (PNS). Recently, however, symptoms such as cognitive deficits, visual dysfunction and circadian disorders were reported, compatible with additional involvement of the central nervous system (CNS) in CIDP. Against this background, we were interested in the functional state of melanopsin-expressing retinal ganglion cells (mRGCs) as a potential biomarker for sleep-wake abnormalities and CNS involvement in CIDP. Based on a chromatic pupillometry protocol, we examined the integrity of the melanopsin system in a prospective case-control study in 20 persons with CIDP compared to 20 controls without CIDP. The results were referred to clinical measures of disease severity and sleep behaviour. Patients with CIDP had a significantly reduced melanopsin-mediated post-illumination pupil response (PIPR) compared to healthy controls (25% versus 36%; < 0.01). This reduction correlated with disease severity ( = 0.478, < 0.05). Further, patients with CIDP reported diminished sleep quality ( < 0.05); however, there was no significant correlation with the melanopsin-mediated PIPR. The results demonstrate an impairment of mRGC function related to CIDP. Since the PIPR reduction correlated with disease severity, it could be an easily available biomarker for CNS affection in CIDP, a condition defined as PNS disorder.
慢性炎症性脱髓鞘性多发性神经病（CIDP）会损害周围神经系统（PNS）的功能。然而，最近有报道称出现了认知缺陷、视觉功能障碍和昼夜节律紊乱等症状，这与CIDP中中枢神经系统（CNS）的额外受累情况相符。在此背景下，我们对表达黑视蛋白的视网膜神经节细胞（mRGCs）的功能状态感兴趣，其作为睡眠 - 觉醒异常和CIDP中CNS受累情况的潜在生物标志物。基于色觉瞳孔测量方案，我们在一项前瞻性病例对照研究中，对20名CIDP患者和20名无CIDP的对照者的黑视蛋白系统完整性进行了检查。结果与疾病严重程度和睡眠行为的临床测量结果相关。与健康对照相比，CIDP患者的黑视蛋白介导的光照后瞳孔反应（PIPR）显著降低（25%对36%；<0.01）。这种降低与疾病严重程度相关（=0.478，<0.05）。此外，CIDP患者报告睡眠质量下降（<0.05）；然而，与黑视蛋白介导的PIPR无显著相关性。结果表明与CIDP相关的mRGC功能受损。由于PIPR降低与疾病严重程度相关，它可能是CIDP中CNS受累情况的一种易于获得的生物标志物，CIDP是一种定义为PNS疾病的病症。
