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帕金森病亚型病程:一项39年的临床病理研究。

Course in Parkinson disease subtypes: A 39-year clinicopathologic study.

作者信息

Rajput A H, Voll A, Rajput M L, Robinson C A, Rajput A

机构信息

Division of Neurology, Department of Pathology, Saskatoon Health Region/University of Saskatchewan, Canada.

出版信息

Neurology. 2009 Jul 21;73(3):206-12. doi: 10.1212/WNL.0b013e3181ae7af1.

DOI:10.1212/WNL.0b013e3181ae7af1
PMID:19620608
Abstract

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.

摘要

背景

路易体帕金森病(PD)病程中的个体差异众所周知。患者已被分为不同的临床亚型,以识别各亚组病程中的差异。多项研究表明,震颤为主型(TD)病例的预后更佳,但其他研究报告并无差异。大多数进展研究基于横断面单点数据或短期临床观察。缺乏经纵向随访且尸检确诊的PD队列仍是文献中的一个主要不足。脑生化研究表明,运动不能/强直型(AR)的异常最为明显,而TD型病例的异常最少。我们推测这些亚型的PD病程与生化研究结果一致。

目的

比较PD的TD、混合型(MX)和AR亚型的病程。

方法

对166例PD患者进行了长达39年(1968 - 2006年)的纵向临床随访和尸检研究。根据整个临床病程将患者分为TD、AR和MX型。仅纳入经病理确诊的PD病例。

结果

66%的病例为MX型,26%为AR型,8%为TD型。TD型病例的起病年龄较轻(p < 0.001),进展至 Hoehn & Yahr 4期的速度较慢(p = 0.016)。痴呆在AR型中最为常见(p = 0.039),在TD型中最不常见。总体而言,病程在TD型中最为有利,其次是MX型和AR型亚组。

结论

帕金森病的三种亚型有不同的病程,这与脑生化异常的差异一致。

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