Department of Neurology, Medical Faculty RWTH Aachen University, Pauwelsstraße 30, D-52074, Aachen, Germany.
JARA-BRAIN Institute Molecular Neuroscience and Neuroimaging, Forschungszentrum Jülich GmbH, Medical Faculty RWTH Aachen University, Pauwelsstraße 30, D-52074, Aachen, Germany.
J Neurol. 2022 Feb;269(2):923-932. doi: 10.1007/s00415-021-10675-7. Epub 2021 Jun 25.
Studies have reported autonomic impairment in patients with idiopathic REM sleep behaviour disorder (iRBD), which is considered a prodromal stage of alpha-synucleinopathies. It is still debated whether central or peripheral pathologies are first manifestations of alpha-synucleinopathies. This study aimed to characterize autonomic and somatosensory function in iRBD patients.
This cross-sectional prospective case-control study included 17 iRBD patients (mean age 66.3 ± 9.2 years) and 16 healthy controls (HCs, 66.6 ± 11.3 years). Quantitative sensory testing, neurological and neuropsychological assessments, norepinephrine blood plasma levels, tilt table examination with orthostatic blood pressure, and heart rate variability were carried out. Longitudinal data of 10 iRBD patients, including neurological, neuropsychological, and tilt table examination, were assessed.
iRBD patients more frequently presented with orthostatic dysfunction than HCs (70.6% vs. 6.3%, p < 0.0001). Supine norepinephrine plasma levels were normal, but lower in iRBD (249.59 ± 99.78 pg/ml iRBD, 354.13 ± 116.38 pg/ml HCs, p < 0.05). Quantitative sensory testing revealed impaired cold (CDT) and vibration detection thresholds (VDT) on the foot in iRBD (CDT foot iRBD - 1.24 ± 0.31, HCs - 9.89E-17 ± 0.25, VDT iRBD - 1.11 ± 0.47, HCs - 1.46E-16 ± 0.25, p < 0.05). Cold detection thresholds differed between the foot and hand among iRBD patients (foot - 1.24 ± 0.31, hand - 0.56 ± 0.25, p < 0.05). Longitudinal data revealed an increase in maximum systolic and diastolic orthostatic blood pressure changes and a decrease in the Valsalva ratio in the follow-up group (p < 0.05).
This study revealed autonomic dysfunction with somatosensory impairment, and decreased norepinephrine levels in iRBD, which may serve as a possible prodromal marker for developing alpha-synucleinopathy.
研究报告称，特发性 REM 睡眠行为障碍（iRBD）患者存在自主神经损伤，这被认为是α-突触核蛋白病的前驱阶段。中枢或外周病变是否首先表现为α-突触核蛋白病仍存在争议。本研究旨在描述 iRBD 患者的自主神经和躯体感觉功能。
这是一项横断面前瞻性病例对照研究，纳入了 17 名 iRBD 患者（平均年龄 66.3±9.2 岁）和 16 名健康对照者（HCs，66.6±11.3 岁）。进行了定量感觉测试、神经学和神经心理学评估、去甲肾上腺素血浆水平、倾斜台检查伴直立位血压、心率变异性。对 10 名 iRBD 患者的神经学、神经心理学和倾斜台检查的纵向数据进行了评估。
与 HCs 相比，iRBD 患者更常出现直立功能障碍（70.6% vs. 6.3%，p<0.0001）。卧位时去甲肾上腺素血浆水平正常，但 iRBD 组较低（iRBD 249.59±99.78pg/ml，HCs 354.13±116.38pg/ml，p<0.05）。定量感觉测试显示 iRBD 患者足部冷觉（CDT）和振动觉阈值（VDT）受损（iRBD 足部 CDT-1.24±0.31，HCs-9.89E-17±0.25，iRBD 足部 VDT-1.11±0.47，HCs-1.46E-16±0.25，p<0.05）。iRBD 患者足部和手部的冷觉阈值不同（足部-1.24±0.31，手部-0.56±0.25，p<0.05）。纵向数据显示随访组的最大收缩压和舒张压直立位血压变化增加，瓦尔萨尔瓦比值降低（p<0.05）。
本研究显示 iRBD 患者存在自主神经功能障碍和躯体感觉损伤，以及去甲肾上腺素水平降低，这可能是发生α-突触核蛋白病的潜在前驱标志物。
