The natural history of Parkinson's disease
The natural history of Parkinson's disease
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DOI：
10.1007/s00415-006-7002-7
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发表时间：
2006-12-01
影响因子：
6
通讯作者：
Poewe, Werner
中科院分区：
文献类型：
--
作者：
Poewe, Werner
虽然帕金森氏病（PD）是唯一的慢性神经退行性疾病，有有效的对症治疗，但尚未确定任何治疗方法可以显着减缓其自然进展。帕金森病自然史的研究因诊断准确性、疾病不同形式的异质性以及年龄相关合并症的混杂效应等问题而变得复杂。只有最近安慰剂对照临床试验的结果才有助于确定早期PD中运动功能障碍进展的速度，如通过评估早期PD中的运动功能障碍。治疗患者的研究表明，在疾病的不同阶段可能有不同的进展速度，早期比晚期下降更快，然而，单独测量运动症状的进展不足以描述PD的自然史，也可能不足以在干预试验中定义有临床意义的疾病改善。Hoehn和Yahr量表中记录的整体残疾进展是重要的考虑因素，进展至III期并开始出现姿势障碍定义了该疾病自然史中的一个重要里程碑。此外，非运动症状对PD的自然病程有重要影响。在最近的一项15年随访研究中，50-80%的患者存在痴呆、睡眠-觉醒周期失调和自主神经功能衰竭。痴呆和精神病也是帕金森病疗养院安置的主要风险因素。改变帕金森病疾病进展的有效干预措施的先决条件是识别疾病的最早临床前阶段。随着嗅觉功能的筛选测试和随后的功能成像，这已经成为一种现实的可能。有帕金森病风险的受试者应该成为神经保护试验未来的重点。
Although Parkinson's disease (PD) is the only chronic neurodegenerative disorder for which there are effective symptomatic therapies no treatment has yet been identified that would significantly slow its natural progression. Studies into the natural history of Parkinson's disease have been complicated by issues of diagnostic accuracy, heterogeneity of different forms of the disease as well as confounding effects of age related comorbidities.Only recently results from placebo-controlled clinical trials have helped to define the rates of progression of motor dysfunction as assessed by the UPDRS in early PD. Studies in treated patients suggest that there maybe different rates of progression in different phases of the disease with faster decline in earlier versus later stages.Measuring progression of motor symptoms, however, alone is insufficient to describe the natural history of PD and may also be inadequate to define clinically meaningful disease modification in intervention trials. Progression of global disability as captured in the Hoehn and Yahr scale is important to consider and progression to stage III with beginning postural impairment defines a major milestone in the natural history of this illness. In addition, non-motor symptoms have major impact on the natural history of PD. Dementia, sleep-wake cycle dysregulation and autonomic failure were present in 50-80% of patients in one recent 15 year-follow up study. Dementia and psychosis are also major risk factors for nursing home placement in PD.A prerequisite for effective interventions that would modify disease progression in Parkinson's disease is the identification of the earliest preclinical stages of illness. This is now become a realistic possibility with screening tests of olfactory function and subsequent functional imaging. Subjects at risk for Parkinson's disease should be the future focus of neuroprotective trials.