Department of Neurology, University of Utah, Salt Lake City, UT, United States.
Department of Neurology, Mayo Clinic, Rochester, MN, United States.
Handb Clin Neurol. 2023;196:175-202. doi: 10.1016/B978-0-323-98817-9.00032-6.
The α-synucleinopathies include pure autonomic failure, multiple system atrophy, dementia with Lewy bodies, and Parkinson disease. The past two decades have witnessed significant advances in the diagnostic strategies and symptomatic treatment of motor and nonmotor symptoms of the synucleinopathies. This chapter provides an in-depth review of the pathophysiology, pathology, genetic, epidemiology, and clinical and laboratory autonomic features that distinguish the different synucleinopathies with an emphasis on autonomic failure as a common feature. The treatment of the different synucleinopathies is discussed along with the proposal for multidisciplinary, individualized care models that optimally address the various symptoms. There is an urgent need for clinical scientific studies addressing patients at risk of developing synucleinopathies and the investigation of disease mechanisms, biomarkers, potential disease-modifying therapies, and further advancement of symptomatic treatments for motor and nonmotor symptoms.
α-突触核蛋白病包括单纯自主神经衰竭、多系统萎缩、路易体痴呆和帕金森病。过去二十年见证了在突触核蛋白病的运动和非运动症状的诊断策略和对症治疗方面的重大进展。本章深入探讨了不同突触核蛋白病的病理生理学、病理学、遗传学、流行病学以及临床和实验室自主神经特征，重点介绍了自主神经衰竭作为共同特征。讨论了不同突触核蛋白病的治疗方法，以及提出了多学科、个体化的护理模式，以最佳地解决各种症状。迫切需要进行临床科学研究，以确定有患突触核蛋白病风险的患者，并研究疾病机制、生物标志物、潜在的疾病修饰治疗方法，以及进一步推进运动和非运动症状的对症治疗。
