Co-occurrence of argyrophilic grain disease in sporadic amyotrophic lateral sclerosis
Co-occurrence of argyrophilic grain disease in sporadic amyotrophic lateral sclerosis
复制标题
散发性肌萎缩侧索硬化症并发嗜银颗粒病
DOI：
10.1111/j.1365-2990.2011.01175.x
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发表时间：
2012
期刊：
影响因子：
2.3
通讯作者：
Takahashi H
中科院分区：
文献类型：
--
作者：
Soma K;Fu YJ;Wakabayashi K;Onodera O;Kakita A;Takahashi H
K.索马，Y. -J. Fu，K.若林岛Onodera，A. Kakita和H. Takahashi（2012）Neuropathology and Applied Neurobiology 38，54- 60散发性肌萎缩侧索硬化症中嗜银颗粒病的共同发生目的：磷酸化TDP-43（pTDP-43）是导致肌萎缩侧索硬化症（ALS）的病理蛋白，ALS是一种致命的神经退行性疾病。最近，有报道称，pTDP-43可在嗜银颗粒病（AGD）患者的脑中蓄积，其中磷酸化4重复tau是病理蛋白。为了阐明ALS与AGD的关系，我们检查了37例连续尸检的散发性ALS患者（年龄范围45-84岁，平均71.5 ± 9.0岁）的脑组织。方法：额颞叶切片用Gallyas-Braak方法染色，并用磷酸化tau、4重复tau和pTDP-43抗体进行免疫染色。 在分期方面，这14例中有5例被评为I级，4例为II级，5例为III级。pTDP-43免疫组化显示，在许多病例中（分别为93%和64%），受影响的内侧颞叶中存在阳性神经元和胶质细胞胞质内含物。另一方面，仅在1例病例中观察到对应于嗜银颗粒的pTDP-43-阳性小结构。AGD和Braak神经病理分期（分期范围0-V，平均2.1）之间存在显著相关性。然而，AGD和任何其他临床病理特征，包括demential.Conclusions之间没有显着的相关性：目前的研究结果表明，AGD在ALS中的共同发生并不罕见，事实上与属于tau蛋白病或α-synucleinopathies的一些疾病相媲美。
K. Soma, Y.‐J. Fu, K. Wakabayashi, O. Onodera, A. Kakita and H. Takahashi (2012)Neuropathology and Applied Neurobiology38,54–60Co‐occurrence of argyrophilic grain disease in sporadic amyotrophic lateral sclerosisAims:Phosphorylated TDP‐43 (pTDP‐43) is the pathological protein responsible for amyotrophic lateral sclerosis (ALS), a fatal neurodegenerative disease. Recently, it has been reported that accumulation of pTDP‐43 can occur in the brains of patients with argyrophilic grain disease (AGD), in which phosphorylated 4‐repeat tau is the pathological protein. To elucidate the association of ALS with AGD, we examined the brains from 37 consecutively autopsied patients with sporadic ALS (age range 45–84 years, mean 71.5 ± 9.0 years).Methods:Sections from the frontotemporal lobe were stained with the Gallyas‐Braak method and also immunostained with antibodies against phosphorylated tau, 4‐repeat tau and pTDP‐43.Results:Fourteen (38%) of the 37 ALS patients were found to have AGD. With regard to staging, 5 of these 14 cases were rated as I, 4 as II and 5 as III. pTDP‐43 immunohistochemistry revealed the presence of positive neuronal and glial cytoplasmic inclusions in the affected medial temporal lobe in many cases (93% and 64%, respectively). On the other hand, pTDP‐43‐positive small structures corresponding to argyrophilic grains were observed only in one case. A significant correlation was found between AGD and the Braak stage for neurofibrillary pathology (stage range 0–V, mean 2.1). However, there were no significant correlations between AGD and any other clinicopathological features, including dementia.Conclusions:The present findings suggest that co‐occurrence of AGD in ALS is not uncommon, and in fact comparable with that in a number of diseases belonging to the tauopathies or α‐synucleinopathies.