Ito H, Kusaka H, Matsumoto S, Imai T
Department of Neurology, Kitano Hospital and Neurological Center, Osaka, Japan.
Acta Neuropathol. 1995;89(6):513-8. doi: 10.1007/BF00571505.
This report concerns the topographic immunohistochemical analysis of the putamen, globus pallidus (GP) and substantia nigra (SN) of two patients with adult-onset motor neuron disease with basophilic inclusions (MND/BIs), seven patients with sporadic classic amyotrophic lateral sclerosis (sporadic ALS) and five neurologically normal individuals. The striatal efferent terminals of the GP and SN were visualized immunohistochemically using antibodies to met-enkephalin (MEnk) and substance P (SP). In specimens from patients with sporadic ALS and normal subjects there was intense immunostaining for MEnk and SP throughout the external and internal segments of the GP, respectively. By contrast, a marked reduction of MEnk- and SP-positive striatal efferents was seen in the ventrocaudal portions of both GP segments from the MND/BIs patients. Moreover, while MEnk-positive striosomes was readily detected in the putamen of normals and sporadic ALS patients, there was significant reduction in MEnk immunoreactivity, and no evidence of striosomal organization in the putamen of MND/BIs patients. In addition, whereas the SN of patients with sporadic ALS expressed SP, the ventrolateral SN portion of the MND/BIs patient tested had reduced immunoreactivity. The present findings on patients with MND/BIs may represent a reflection of the topographic striatum degeneration in this disease and appear to provide additional evidence for the heterogeneity of MND.
本报告涉及两名患有成年起病型运动神经元病伴嗜碱性包涵体（MND/BIs）的患者、七名散发性经典肌萎缩侧索硬化症（散发性ALS）患者以及五名神经功能正常个体的壳核、苍白球（GP）和黑质（SN）的组织形态免疫组化分析。使用甲硫氨酸脑啡肽（MEnk）和P物质（SP）抗体对GP和SN的纹状体传出终末进行免疫组化可视化。在散发性ALS患者和正常受试者的标本中，GP的外部和内部节段分别对MEnk和SP进行了强烈免疫染色。相比之下，在MND/BIs患者的GP两个节段的腹尾部分，观察到MEnk和SP阳性的纹状体传出纤维明显减少。此外，虽然在正常人和散发性ALS患者的壳核中很容易检测到MEnk阳性的纹状体小体，但在MND/BIs患者的壳核中，MEnk免疫反应性显著降低，且没有纹状体小体组织的证据。另外，散发性ALS患者的SN表达SP，而测试的MND/BIs患者的SN腹外侧部分免疫反应性降低。关于MND/BIs患者的目前发现可能反映了该疾病中纹状体的局部变性，似乎为MND的异质性提供了额外证据。
