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血管性帕金森综合征的临床病理研究,包括诊断的临床标准。

Clinicopathological investigation of vascular parkinsonism, including clinical criteria for diagnosis.

作者信息

Zijlmans Jan C M, Daniel Susan E, Hughes Andrew J, Révész Tamas, Lees Andrew J

机构信息

Queen Square Brain Bank for Neurological Disorders, Institute of Neurology Queen Square, London, United Kingdom.

出版信息

Mov Disord. 2004 Jun;19(6):630-40. doi: 10.1002/mds.20083.

Abstract

Vascular parkinsonism (VP) is difficult to diagnose with any degree of clinical certainty. We investigated the importance of macroscopic cerebral infarcts and pathological findings associated with microscopic "small vessel disease" (SVD) in the aetiology of VP. The severity of microscopic SVD pathology (perivascular pallor, gliosis, hyaline thickening, and enlargement of perivascular spaces) and the presence of macroscopically visible infarcts were assessed in 17 patients with parkinsonism and no pathological evidence of either Parkinson's disease or any histopathological condition known to be associated with a parkinsonian syndrome, and compared with age-matched controls. Microscopic SVD pathology was significantly more severe in the parkinsonian brains. Most patients presented with bilateral bradykinesia and rigidity together with a gait disorder characterised predominantly by a shuffling gait. Four patients presented acutely with hemiparesis and then progressed to develop a parkinsonian syndrome. They could be distinguished from the remaining VP patients by the presence at autopsy of macroscopically visible lacunar infarcts in regions where contralateral thalamocortical drive might be reduced. The clinical features at presentation varied according to the speed of onset and the underlying vascular pathological state. New clinical criteria for a diagnosis of VP are proposed based on the clinicopathological findings of this study.

摘要

血管性帕金森综合征(VP)很难在临床上确诊。我们研究了宏观脑梗死以及与微观“小血管疾病”(SVD)相关的病理发现在VP病因学中的重要性。在17例帕金森综合征患者中评估了微观SVD病理的严重程度(血管周围苍白、胶质增生、透明样增厚和血管周围间隙扩大)以及宏观可见梗死灶的存在情况,这些患者没有帕金森病或任何已知与帕金森综合征相关的组织病理学状况的病理证据,并与年龄匹配的对照组进行比较。帕金森病患者脑部微观SVD病理明显更严重。大多数患者表现为双侧运动迟缓、强直以及以拖步为主的步态障碍。4例患者急性起病时出现偏瘫,随后进展为帕金森综合征。尸检发现,在对侧丘脑皮质驱动可能减弱的区域存在宏观可见的腔隙性梗死灶,这可以将他们与其余VP患者区分开来。临床表现根据起病速度和潜在的血管病理状态而有所不同。基于本研究的临床病理结果,提出了诊断VP的新临床标准。

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