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我的多系统萎缩治疗方法。

My Treatment Approach to Multiple System Atrophy.

机构信息

Department of Neurology, Mayo Clinic, Rochester, MN.

Department of Neurology, Mayo Clinic, Rochester, MN.

出版信息

Mayo Clin Proc. 2021 Mar;96(3):708-719. doi: 10.1016/j.mayocp.2020.10.005.

Abstract

Multiple system atrophy (MSA) is a neurodegenerative disorder primarily characterized by autonomic failure plus parkinsonism or cerebellar ataxia. The diagnosis may be challenging and is usually made at a tertiary care center. The long-term management issues are equally challenging and frequently require collaboration with the patient's local care providers. Whereas there is currently no cure for MSA, treatment focuses on the most problematic symptoms experienced by the patient. Autonomic symptoms may include severe orthostatic hypotension with syncope, urinary symptoms culminating in incontinence, constipation, anhidrosis, and erectile dysfunction. Motor symptoms include parkinsonism, cerebellar ataxia, and falls. Although certain motor symptoms may respond partially to medications, some of these medications may exacerbate autonomic problems. In this manuscript, we seek to bridge the gap between tertiary care providers and the patient's local care providers to provide multidisciplinary care to the MSA patient. Patients are often best served by management of their chronic and evolving complex problems with a team approach involving their primary care providers and subspecialists. Treatment guidelines typically list myriad therapeutic options without clarifying the most efficacious and simplest treatment strategies. Herein, we provide a guideline based on what has worked in our MSA clinic, a clinic designed to provide care throughout the disease course with subspecialty integration with the goal of empowering a partnership with the patient's home primary care providers.

摘要

多系统萎缩(MSA)是一种主要以自主神经衰竭加帕金森病或小脑共济失调为特征的神经退行性疾病。诊断可能具有挑战性,通常在三级护理中心进行。长期管理问题同样具有挑战性,经常需要与患者当地的护理提供者合作。虽然目前尚无治疗 MSA 的方法,但治疗重点是针对患者最棘手的症状。自主症状可能包括严重的直立性低血压伴晕厥、导致失禁的泌尿系统症状、便秘、无汗和勃起功能障碍。运动症状包括帕金森病、小脑共济失调和跌倒。虽然某些运动症状可能会部分对药物治疗有反应,但其中一些药物可能会加重自主问题。在本文中,我们旨在弥合三级护理提供者和患者当地护理提供者之间的差距,为 MSA 患者提供多学科护理。患者通常通过团队方法来管理其慢性和不断发展的复杂问题,包括他们的初级保健提供者和专家,得到最佳服务。治疗指南通常列出了无数的治疗选择,但没有澄清最有效和最简单的治疗策略。在此,我们根据我们的 MSA 诊所的经验提供了一份指南,该诊所旨在通过与专家的整合,为整个疾病过程提供护理,目标是与患者的家庭初级保健提供者建立合作关系。

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