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暴发性路易体病

Fulminant Lewy body disease.

作者信息

Momjian-Mayor Isabelle, Pizzolato Gian Paolo, Burkhardt Karim, Landis Theodor, Coeytaux Alessandra, Burkhard Pierre R

机构信息

Department of Neurology, Geneva University Hospitals and Medical School, Geneva, Switzerland.

出版信息

Mov Disord. 2006 Oct;21(10):1748-51. doi: 10.1002/mds.21034.

Abstract

The clinical distinction between Parkinson's disease (PD) with dementia (PDD) and dementia with Lewy bodies (DLB) is challenged by most neuropathological studies showing nearly identical changes in both conditions. We report an unusual case of PD evolving into a rapidly progressive dementia leading to death within 3 months that showed nearly all clinical features of DLB. At autopsy, numerous Lewy bodies and Lewy neurites were found in several areas of the brainstem, the limbic system, and the neocortex, consistent with pure DLB. This case demonstrates that Lewy body disease may exhibit a dramatic course without any coexisting pathology and exemplifies that PD, PDD, and DLB may sometimes represent sequential, yet overlapping, phenotypes of a same clinicopathological entity.

摘要

帕金森病(PD)伴发痴呆(PDD)与路易体痴呆(DLB)之间的临床鉴别受到了大多数神经病理学研究的挑战,这些研究表明这两种病症存在几乎相同的变化。我们报告了一例不寻常的病例,一名帕金森病患者演变成快速进展性痴呆,并在3个月内死亡,该病例几乎呈现出路易体痴呆的所有临床特征。尸检发现,在脑干、边缘系统和新皮质的多个区域存在大量路易小体和路易神经突,符合单纯路易体痴呆的表现。该病例表明,路易体病可能呈现出急剧的病程且无任何并存的病理学改变,例证了帕金森病、帕金森病伴发痴呆和路易体痴呆有时可能代表同一临床病理实体的连续但重叠的表型。

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