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帕金森综合征

Parkinsonian syndromes.

作者信息

Williams David R, Litvan Irene

出版信息

Continuum (Minneap Minn). 2013 Oct;19(5 Movement Disorders):1189-212. doi: 10.1212/01.CON.0000436152.24038.e0.

Abstract

PURPOSE OF REVIEW

The different parkinsonian conditions can be challenging to separate clinically. This review highlights the important clinical features that guide the diagnosis of Parkinson disease (PD), progressive supranuclear palsy (PSP), multiple system atrophy (MSA), and corticobasal degeneration (CBD). Strategies for treatment and disease management are also discussed.

RECENT FINDINGS

Over the past decade there has been an increasing recognition of the broad clinical presentations of the neurodegenerative forms of parkinsonism. Nonmotor symptoms in these diseases, including psychiatric, cognitive, autonomic, and gastrointestinal dysfunction, appear to have a major impact on quality of life and disability. PSP and CBD are now considered pathologic diagnoses, with several different and varied clinical phenotypes, that overlap and share features with PD and frontotemporal dementia syndromes. PD is distinguished by its excellent response to dopaminergic medications that is maintained over many years, in contrast to the response seen in patients with MSA and PSP. New diagnostic criteria have been proposed for CBD. No new therapeutic interventions have emerged for PSP, MSA, or CBD. Infusional therapies and deep brain stimulation surgery are established therapies for advanced PD.

SUMMARY

The "parkinsonian syndromes" encompass a number of nosologic entities that are grouped together on the basis of their shared clinical features but are separated on the basis of their different pathologies. Overall, the consideration of clinical signs, mode of disease onset, and nature of disease progression are all important to make a timely and definitive diagnosis.

摘要

综述目的

不同的帕金森综合征在临床上可能难以区分。本综述重点介绍了指导帕金森病(PD)、进行性核上性麻痹(PSP)、多系统萎缩(MSA)和皮质基底节变性(CBD)诊断的重要临床特征。还讨论了治疗和疾病管理策略。

最新发现

在过去十年中,人们越来越认识到帕金森综合征神经退行性形式的广泛临床表现。这些疾病中的非运动症状,包括精神、认知、自主神经和胃肠功能障碍,似乎对生活质量和残疾有重大影响。PSP和CBD现在被认为是病理诊断,有几种不同且多样的临床表型,与PD和额颞叶痴呆综合征有重叠和共同特征。与MSA和PSP患者的反应相比,PD的特点是对多巴胺能药物有良好反应,且多年来一直保持。已提出CBD的新诊断标准。PSP、MSA或CBD尚未出现新的治疗干预措施。输注疗法和脑深部刺激手术是晚期PD的既定治疗方法。

总结

“帕金森综合征”包括一些疾病实体,它们基于共同的临床特征被归为一组,但根据不同的病理情况而区分。总体而言,考虑临床体征、疾病起病方式和疾病进展性质对于及时做出明确诊断都很重要。

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本文引用的文献

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Criteria for the diagnosis of corticobasal degeneration.皮质基底节变性的诊断标准。
Neurology. 2013 Jan 29;80(5):496-503. doi: 10.1212/WNL.0b013e31827f0fd1.
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