Course in Parkinson disease subtypes A 39-year clinicopathologic study

Course in Parkinson disease subtypes A 39-year clinicopathologic study
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DOI:
10.1212/wnl.0b013e3181ae7af1
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发表时间:
2009-07-21
期刊:
影响因子:
9.9
通讯作者:
Rajput, A.
Rajput, A.
中科院分区:
医学1区
文献类型:
--
作者:
Rajput, A. H.;Voll, A.;Rajput, A.

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背景:路易体帕金森病(PD)病程中的个体差异是众所周知的。患者被分为不同的临床亚型,以确定不同亚组之间的病程差异。几项研究表明,震颤显性(TD)患者的结果更有利,但其他研究报告没有差异。大多数进展研究是基于横断面单点数据或短期临床观察。缺乏纵向追踪的尸检证实的帕金森病队列仍然是文献中的一个主要弱点。对大脑的生化研究表明,无运动/僵硬(AR)的异常最明显,TD患者的异常最小。目的:比较TD亚型、混合型(MX)和AR亚型PD患者的病程。方法:对1968-2006年收治的166例PD患者进行纵向临床随访和尸检分析。根据整个临床病程将患者分为TD、AR和MX三组。结果:66%的患者有MX,26%的患者为AR,8%的患者为TD。TD患者的发病年龄较早(p<0.001),进展至Hoehn&Yahr分期4期较慢(p=0.016)。痴呆症在AR中最常见(p=0.039),在TD中最不常见。结论:帕金森病三种亚型的病程不同,与脑生化异常的差异相一致。神经病学(R)2009;73:206-12
Background: Individual variations in the course of Lewy body Parkinson disease (PD) are well known. Patients have been classified into different clinical subtypes to identify differences in the course among the subgroups. Several studies indicate that the outcome is more favorable in tremor dominant (TD) cases but others report no difference. A majority of progression studies are based on cross-sectional single point data or short-term clinical observations. The lack of longitudinally followed autopsy-confirmed PD cohort remains a major weakness in the literature. Biochemical studies of brain indicate most pronounced abnormalities in akinetic/rigid (AR) and the least in TD cases. We postulate that PD course in these subtypes is concordant with the biochemical findings.Objective: To compare the course in TD, mixed (MX), and AR subtypes of PD.Methods: Longitudinal clinical follow-up and autopsy studies were performed on 166 patients with PD over 39 years (1968-2006). Patients were classified into TD, AR, and MX based on the entire clinical course. Only the pathologically confirmed PD cases were included.Results: Sixty-six percent of cases had MX, 26% AR, and 8% TD profile. The age at onset was younger (p < 0.001) and progression to Hoehn & Yahr stage 4 was slower (p = 0.016) in the TD cases. Dementia was most common in AR (p = 0.039) and the least common in TD. In general, the course was most favorable in TD, followed by MX and AR subgroups.Conclusion: The three subtypes of Parkinson disease have different courses which are concordant with the differences in brain biochemical abnormalities. Neurology (R) 2009; 73: 206-12