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首页> 外文期刊>Neuropathology: official journal of the Japanese Society of Neuropathology >Sporadic amyotrophic lateral sclerosis with pallido-nigro-luysian degeneration: a TDP-43 immunohistochemical study.
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Sporadic amyotrophic lateral sclerosis with pallido-nigro-luysian degeneration: a TDP-43 immunohistochemical study.

机译：散发性肌萎缩性侧索硬化伴帕利多-黑人-路易氏变性：TDP-43免疫组织化学研究。

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摘要

Recently, Nishihira et al. demonstrated the presence of two types of TDP-43 pathology in sporadic amyotrophic lateral sclerosis (ALS) (Acta Neuropathol 2008; 116: 169-182). Type 1 represents the TDP-43 distribution pattern observed in classic ALS, whereas type 2 shows the presence of TDP-43 inclusions in the frontotemporal cortex, hippocampal formation, neostriatum and substantia nigra and is significantly associated with dementia. However, ALS with pallido-nigro-luysian degeneration (PNLD) is very rare. We recently encountered a case of ALS with PNLD of 9 years duration, in which the patient received artificial respiratory support for 6 years. In our case, neuronal loss and TDP-43-positive neuronal cytoplasmic inclusions were found in the globus pallidus, substantia nigra and subthalamic nucleus. Neither neuronal loss nor TDP-43-immunoreactive inclusions were found in the frontotemporal cortex and hippocampus. These findings suggest that the pallido-nigro-luysian system is also involved in the disease process of ALS and that ALS with PNLD is different from ALS with dementia based on the distribution pattern of neuronal loss and TDP-43 accumulation.
机译：最近，Nishihira等。证明了在散发性肌萎缩性侧索硬化症（ALS）中存在两种类型的TDP-43病理学（Acta Neuropathol 2008; 116：169-182）。类型1代表在经典ALS中观察到的TDP-43分布模式，而类型2显示在额颞叶皮质，海马结构，新纹状体和黑质中存在TDP-43内含物，并且与痴呆症显着相关。但是，伴有pallido-nigro-luysian变性（PNLD）的ALS非常罕见。我们最近遇到一例PNLD为9年的ALS患者，其中该患者接受了6年的人工呼吸支持。在我们的案例中，在苍白球，黑质和丘脑底核中发现了神经元丢失和TDP-43阳性神经元胞浆内含物。在额颞叶皮质和海马体中均未发现神经元丢失或TDP-43-免疫反应性包涵体。这些发现表明，神经元丢失和TDP-43积累的分布模式表明，帕利多-黑质-卢西斯系统也参与了ALS的发病过程，并且PNLD的ALS与痴呆的ALS不同。

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