Pay attention to the value of autonomic dysfunction in diagnosing and prognosis prediction of central α-synucleinopathy

doi:10.3969/j.issn.1672-6731.2024.03.001

Abstract

Abstract:

Central α -synucleinopathy includes Parkinson's disease (PD), dementia with Lewy bodies (DLB) and multiple system atrophy (MSA). The clinical manifestations of these diseases usually overlap, which make the differential diagnosis difficult in clinical practice. Autonomic dysfunction is a common feature of these diseases. Recent studies indicated the autonomic dysfunction would contribute to our further understanding of central α-synucleinopathy. This article reviews the value of autonomic dysfunction in the predictive diagnosis, differential diagnosis and prognosis of the above diseases, to help clinical practice.

Key words: Synucleinopathies, Autonomic nervous system, Review

摘要：

中枢性α-突触核蛋白病包括帕金森病、路易体痴呆和多系统萎缩，上述疾病的临床表现部分重叠，鉴别诊断较为困难。自主神经功能障碍是这些疾病的共同常见特征，对自主神经功能障碍的研究有助于进一步理解中枢性α-突触核蛋白病。本文对上述3种疾病自主神经功能障碍在疾病早期诊断、鉴别诊断和预后预测方面的应用价值进行梳理，以指导临床诊断与治疗。

关键词: 突触核蛋白病, 自主神经系统, 综述

Han WANG, Yi-chun WANG, Zhe ZHANG. Pay attention to the value of autonomic dysfunction in diagnosing and prognosis prediction of central α-synucleinopathy[J]. Chinese Journal of Contemporary Neurology and Neurosurgery, 2024, 24(3): 113-119.

王含, 王一淳, 张哲. 应重视自主神经功能障碍在中枢性α-突触核蛋白病诊断与预后预测中的作用[J]. 中国现代神经疾病杂志, 2024, 24(3): 113-119.

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URL: http://journal11.magtechjournal.com/cjcnn/EN/10.3969/j.issn.1672-6731.2024.03.001

http://journal11.magtechjournal.com/cjcnn/EN/Y2024/V24/I3/113

Figures/Tables 1

References 56

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累及系统	临床症状	量表	辅助检查
心血管系统	直立性低血压，餐后低血压，卧位高血压	直立性评分量表（OGS），直立性低血压问卷（OHQ）	直立倾斜试验，TCD，Valsalva动作，24小时动态血压监测，进食试验，冷压力试验，运动试验，血浆去甲肾上腺素和其他儿茶酚胺，心率变异性，皮肤血管舒缩反射，¹²³I-MIBG/¹³¹I-MIBG
消化系统	便秘，吞咽障碍和流涎，其他胃肠道运动障碍如恶心、呕吐、早饱、纳差、胃扩张、胃轻瘫	吞咽障碍：吞咽障碍问卷（SDQ），吞咽生活质量量表（SWAL-QoL）流涎：流涎严重程度和频率量表（DSFS），流涎评价量表（DRS），帕金森病流涎临床量表（SCS-PD）胃排空障碍：胃瘫主要症状指数（GCSI）便秘：功能性消化不良罗马Ⅱ和Ⅲ诊断标准	静脉造影剂吞咽造影检查（VFSS），测压法检查，软式喉内镜吞咽功能检查（FEES），改良吞钡试验（MBSS），手持咳嗽试验（HCT），¹³C辛酸或 ¹³C乙酸呼吸试验，EGG，结肠通过时间测定和肛肠压力测定
泌尿系统	尿频，尿急，夜尿症，尿失禁，尿潴留	膀胱过度活动症评分（OABSS），丹麦前列腺症状评分（DAN-PSS）；国际尿失禁咨询委员会尿失禁问卷简表（ICIQ-SF）：男性下尿路症状	残余尿超声，尿动力学检查，肛门外括约肌肌电图（EAS-EMG）
生殖系统	男性：勃起功能障碍，射精障碍，难以达到性高潮女性：阴道干燥，性欲降低，难以达到性高潮	性生活质量问卷（QoSL-Q）；亚利桑那性体验量表；性功能障碍量表	球海绵体反射
体温调节系统	冷热不耐受；发作性泌汗，泌汗过多，泌汗过少，无汗症	无	体温调节汗液测验（TST），定量催汗轴突反射试验（QSART），硅胶印迹，交感皮肤反应（SSR），动态泌汗试验（DST），乙酰胆碱汗斑试验，定量直接和间接轴突反射测验（QDIRT），皮肤肌电图
瞳孔对光反射通路	光和疼痛刺激下瞳孔反应延迟	无	瞳孔对光反射和瞳孔测量；拟交感神经药物和拟副交感神经药物的瞳孔敏感性试验

累及系统	临床症状	量表	辅助检查
心血管系统	直立性低血压，餐后低血压，卧位高血压	直立性评分量表（OGS），直立性低血压问卷（OHQ）	直立倾斜试验，TCD，Valsalva动作，24小时动态血压监测，进食试验，冷压力试验，运动试验，血浆去甲肾上腺素和其他儿茶酚胺，心率变异性，皮肤血管舒缩反射，¹²³I-MIBG/¹³¹I-MIBG
消化系统	便秘，吞咽障碍和流涎，其他胃肠道运动障碍如恶心、呕吐、早饱、纳差、胃扩张、胃轻瘫	吞咽障碍：吞咽障碍问卷（SDQ），吞咽生活质量量表（SWAL-QoL）流涎：流涎严重程度和频率量表（DSFS），流涎评价量表（DRS），帕金森病流涎临床量表（SCS-PD）胃排空障碍：胃瘫主要症状指数（GCSI）便秘：功能性消化不良罗马Ⅱ和Ⅲ诊断标准	静脉造影剂吞咽造影检查（VFSS），测压法检查，软式喉内镜吞咽功能检查（FEES），改良吞钡试验（MBSS），手持咳嗽试验（HCT），¹³C辛酸或 ¹³C乙酸呼吸试验，EGG，结肠通过时间测定和肛肠压力测定
泌尿系统	尿频，尿急，夜尿症，尿失禁，尿潴留	膀胱过度活动症评分（OABSS），丹麦前列腺症状评分（DAN-PSS）；国际尿失禁咨询委员会尿失禁问卷简表（ICIQ-SF）：男性下尿路症状	残余尿超声，尿动力学检查，肛门外括约肌肌电图（EAS-EMG）
生殖系统	男性：勃起功能障碍，射精障碍，难以达到性高潮女性：阴道干燥，性欲降低，难以达到性高潮	性生活质量问卷（QoSL-Q）；亚利桑那性体验量表；性功能障碍量表	球海绵体反射
体温调节系统	冷热不耐受；发作性泌汗，泌汗过多，泌汗过少，无汗症	无	体温调节汗液测验（TST），定量催汗轴突反射试验（QSART），硅胶印迹，交感皮肤反应（SSR），动态泌汗试验（DST），乙酰胆碱汗斑试验，定量直接和间接轴突反射测验（QDIRT），皮肤肌电图
瞳孔对光反射通路	光和疼痛刺激下瞳孔反应延迟	无	瞳孔对光反射和瞳孔测量；拟交感神经药物和拟副交感神经药物的瞳孔敏感性试验

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