Numerous and widespread α-synuclein-negative Lewy bodies in an asymptomatic patient
Numerous and widespread α-synuclein-negative Lewy bodies in an asymptomatic patient
复制标题
无症状患者体内存在大量广泛的 α-突触核蛋白阴性路易体
作者：
S. Duinen;G. Lammers;M. Maat‐Schieman;R. Roos
摘要路易小体(Lb)和苍白小体(PB)是帕金森病和/或痴呆疾病的前驱物质。此外，在其他一些神经退行性疾病和正常衰老中偶尔也能观察到Lb。经典的LB通常见于脑干，尤其是黑质，这些包涵体与神经元丢失和特发性帕金森病(PD)的临床症状有关。大脑皮层LB位于大脑皮层、杏仁核和屏蔽区，几乎没有或几乎没有神经元丢失，临床上与LB痴呆(DLB)有关。我们描述了一位没有明显帕金森氏症和/或痴呆症临床症状的患者，他的脑中有许多经典的LB，苍白的内含物，具有PB的特征，以及两者之间的过渡。这些包涵体的免疫组织学特征(泛素阳性；神经丝阳性；tau阴性)和超微结构特征与PD和DLB中的LB相似，只是对α-突触核蛋白缺乏免疫反应。脑桥和大脑皮层的LB值最高，达165个/1.76mm2。这些数字与没有明显的神经元丢失或胶质增生形成对比。在这例无症状患者的脑脊液中，α-突触核蛋白活性的缺失证实了α-突触核蛋白在脑脊液中的积聚是帕金森病和酒精性脑病神经退行性变的重要步骤的假说，但总体上淡化了α-突触核蛋白在脑脊液形成中的作用。这位患者似乎代表了一种与肝病相关的疾病谱中的新变种。
Abstract Lewy bodies (LB) and pale bodies (PB), their putative precursors, can be found in a spectrum of diseases characterized by parkinsonism and/or dementia. Furthermore, LB are occasionally observed in some other neurodegenerative diseases and in normal aging. Classical LB are typically found in the brain stem, especially in the substantia nigra, where these inclusions are associated with neuronal loss and clinical signs of idiopathic Parkinson’s disease (PD). The so-called cortical LB occur in the cerebral cortex, amygdala and claustrum with little or no neuronal loss and are clinically associated with dementia in dementia with LB (DLB). We describe a patient without apparent clinical signs of parkinsonism and/or dementia, whose brain contained numerous classical-like LB, pale inclusions with features of PB and transitions between these two. These inclusions had similar immunohistological (ubiquitin positive; neurofilament positive; tau negative) and ultrastructural features as the LB in PD and DLB except for the lack of immunoreactivity for α-synuclein. The pons and cerebral cortex showed the highest number of LB, up to 165/1.76 mm2. These numbers were contrasted by the lack of obvious neuronal loss or gliosis. The absence of α-synuclein reactivity in the LB in this symptomless patient corroborates the hypothesis that α-synuclein accumulation in LB is an important step in neurodegeneration in PD and DLB, but tones down the role of α-synuclein in LB formation in general. This patient seems to represent a new variant in the spectrum of diseases associated with LB.
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DOI：
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发表时间：
1998-04
期刊：
The American journal of pathology
影响因子：
--
作者：
M. Baba;S. Nakajo;Ping-Hui Tu;T. Tomita;K. Nakaya;V. Lee;J. Trojanowski;T. Iwatsubo
通讯作者：
M. Baba;S. Nakajo;Ping-Hui Tu;T. Tomita;K. Nakaya;V. Lee;J. Trojanowski;T. Iwatsubo
影响因子：
56.9
作者：
GOLDMAN, JE;YEN, SH;PERESS, NS
通讯作者：
PERESS, NS
DOI：
--
发表时间：
1997-04
期刊：
Laboratory investigation; a journal of technical methods and pathology
影响因子：
--
作者：
Pang-Hsien Tu;M. Gurney;Jean-Pierre Julien;V. Lee;J. Trojanowski
通讯作者：
Pang-Hsien Tu;M. Gurney;Jean-Pierre Julien;V. Lee;J. Trojanowski
路易体的细丝含有不溶性细胞骨架成分。
DOI：
--
发表时间：
1992
期刊：
The American journal of pathology
影响因子：
--
作者：
Galloway,PG;Mulvihill,P;Perry,G
通讯作者：
Perry,G