Wakabayashi K, Piao Y S, Hayashi S, Kakita A, Yamada M, Takahashi H
Brain Disease Research Center, Niigata University, Japan.
Clin Neuropathol. 2001 Mar-Apr;20(2):47-52.
Neuronal degeneration in amyotrophic lateral sclerosis (ALS) is associated with ubiquitinated cytoskeletal alterations in the motor neuron system. Ubiquitin-positive inclusions are also seen in the limbic system in ALS with dementia (ALS-D). Recently, similar inclusions were reported to occur in the neostriatum in a case of ALS-D. We, therefore, immunohistochemically examined the neostriatum from 60 patients with ALS and 60 control subjects.
Two forms of ubiquitin-positive inclusions were found in the ALS neostriatum: rod-like inclusions in the large neurons and crescent-shaped inclusions in the small neurons. The former were found in 14 ALS and 18 control subjects, whereas the latter were specific to ALS; the crescent-shaped inclusions in small neurons were found in 27 ALS patients, and the immunohistochemical and ultrastructural features were identical to those of the extra-motor inclusions of ALS. Moreover, characteristic temporal lesions consistent with those seen in ALS-D were found in 8 patients, of whom 2 had shown dementia.
The present findings strongly suggest that neostriatal small neurons are also involved in the disease process in ALS.
肌萎缩侧索硬化症（ALS）中的神经元变性与运动神经元系统中泛素化的细胞骨架改变有关。在伴有痴呆的ALS（ALS-D）患者的边缘系统中也可见泛素阳性包涵体。最近，在1例ALS-D患者的新纹状体中也报道了类似的包涵体。因此，我们对60例ALS患者和60例对照者的新纹状体进行了免疫组化检查。
在ALS患者的新纹状体中发现了两种形式的泛素阳性包涵体：大神经元中的棒状包涵体和小神经元中的新月形包涵体。前者在14例ALS患者和18例对照者中发现，而后者是ALS所特有的；在27例ALS患者中发现了小神经元中的新月形包涵体，其免疫组化和超微结构特征与ALS的运动外包涵体相同。此外，在8例患者中发现了与ALS-D中所见一致的特征性颞叶病变，其中2例有痴呆表现。
目前的研究结果强烈提示，新纹状体中的小神经元也参与了ALS的疾病进程。
