Hu Wen-Zheng, Cao Ling-Xiao, Yin Jin-Hui, Zhao Xue-Song, Piao Ying-Shan, Gu Wei-Hong, Ma Jing-Hong, Wan Zhi-Rong, Huang Yue
China National Clinical Research Center for Neurological Diseases, Beijing Tiantan Hospital, Capital Medical University, Beijing, China.
Department of Neurology, Beijing Tiantan Hospital, Capital Medical University, Beijing, China.
Front Neurol. 2023 Jan 23;13:1081219. doi: 10.3389/fneur.2022.1081219. eCollection 2022.
Non-motor symptoms (NMS) are compulsory clinical features for the clinical diagnosis of multiple system atrophy (MSA), some of which precede motor symptoms onset. To date, few studies have systematically investigated NMS in MSA and the timing of presenting NMS as the disease progresses. Clinically, MSA is difficult to be differentiated from Parkinson's disease (PD) and progressive supranuclear palsy (PSP), and the differences in NMS between MSA and PD/PSP remain unclear. The aim of this study was to compare the burden of NMS between MSA and PD/PSP and to delineate the timing of NMS presentation relative to the onset of motor symptoms in MSA.
A total of 61, 87, and 30 patients with MSA, PD, and PSP, respectively, were enrolled in this study. NMS was systematically assessed in all patients using the NMS scale (NMSS), and the onset of NMS relative to the onset of motor symptoms in MSA was investigated.
MSA group had higher total NMSS scores (82.15 ± 46.10) than the PD (36.14 ± 30.78) and PSP (50.30 ± 55.05) groups ( < 0.001 overall). The number distribution pattern of the NMS was significantly different among the three parkinsonian disorders ( < 0.001 overall). In total, 85.2% of patients with MSA had more than 10 NMS, which was significantly higher than PD (28.7%) and PSP (33.3%). The frequency and scores of many NMSS subdomains and symptoms were higher in MSA than in PD and PSP (all < 0.05). Multivariate logistic regression analysis revealed that patients with fainting, lack of motivation, swallowing, and loss of sexual interest could be attributed to MSA rather than PD or PSP, while patients with loss of concentration and forgetfulness were characteristic features of PD or PSP rather than MSA. REM-sleep behavior disorder (RBD), constipation, problems having sex, and loss of sexual interest preceded the motor symptoms onset of MSA by 2.81 ± 4.51, 1.54 ± 6.32, 1.35 ± 4.70, and 0.45 ± 3.61 years, respectively.
The NMS spectrum in MSA differs from that of PD and PSP. Patients with MSA have a higher NMS burden than patients with PD or PSP. RBD, constipation, problems having sex, and loss of sexual interest may become early diagnostic clinical markers of MSA.
非运动症状（NMS）是多系统萎缩（MSA）临床诊断的必备临床特征，其中一些症状先于运动症状出现。迄今为止，很少有研究系统地调查MSA中的NMS以及随着疾病进展NMS出现的时间。临床上，MSA难以与帕金森病（PD）和进行性核上性麻痹（PSP）相鉴别，MSA与PD/PSP之间NMS的差异仍不清楚。本研究的目的是比较MSA与PD/PSP之间NMS的负担，并确定MSA中NMS相对于运动症状发作的出现时间。
本研究共纳入61例MSA患者、87例PD患者和30例PSP患者。使用非运动症状量表（NMSS）对所有患者进行系统的NMS评估，并调查MSA中NMS相对于运动症状发作的情况。
MSA组的NMSS总分（82.15±46.10）高于PD组（36.14±30.78）和PSP组（50.30±55.05）（总体P<0.001）。三种帕金森病之间NMS 的数量分布模式有显著差异（总体P<0.001）。总的来说，85.2%的MSA患者有超过10种NMS，这显著高于PD患者（28.7%）和PSP患者（33.3%）。MSA中许多NMSS子领域和症状的频率和得分高于PD和PSP（均P<0.05）。多因素逻辑回归分析显示，晕厥、缺乏动力、吞咽困难和性兴趣丧失的患者可归因于MSA而非PD或PSP，而注意力不集中和健忘是PD或PSP而非MSA的特征性表现。快速眼动睡眠行为障碍（RBD）、便秘、性生活问题和性兴趣丧失分别比MSA运动症状发作提前2.81±4.51年、1.54±6.32年、1.35±4.70年和0.45±3.61年。
MSA中的NMS谱与PD和PSP不同。MSA患者的NMS负担高于PD或PSP患者。RBD、便秘、性生活问题和性兴趣丧失可能成为MSA的早期诊断临床标志物。
