Murphy G M, Eng L F, Ellis W G, Perry G, Meissner L C, Tinklenberg J R
Department of Psychiatry and Behavioral Sciences, Stanford University School of Medicine, Palo Alto, CA 94304.
Brain Res. 1990 Dec 24;537(1-2):102-8. doi: 10.1016/0006-8993(90)90345-c.
Most patients with Down's syndrome (DS) undergo a premature cognitive decline with aging, and eventually develop the neuropathologic changes of Alzheimer's disease (AD), including amyloid-containing neuritic plaques, and the formation of neurofibrillary tangles. The amygdala is a focus of marked neuropathologic change in older patients with DS and in AD. We examined the amygdala with immunocytochemical and histochemical methods in 6 cases with DS, ages 19, 20, 27, 29, 56 and 64 years and compared them to 4 cases with AD, ages 54, 76, 77 and 80 years. An antiserum to the A4 amyloid peptide demonstrated amyloid deposition in plaques in all 10 cases. Plaques were also revealed in all cases by the Alcian blue stain for glycosaminoglycans and by the Bielschowsky and Bodian silver stains. An antiserum to alpha-1-antichymotrypsin (ACT) showed plaques in the AD cases and in the 19, 56 and 64 year old DS cases. Neurofibrillary tangles were observed with silver stains only in the older DS and in the AD cases, and not in the 19, 20, 27 and 29 year old DS cases. Likewise, antisera to paired helical filament, to microtubule associated proteins tau and microtubule associated protein-2 (MAP-2), and to ubiquitin, all of which are components of neurofibrillary tangles, reacted with tangles and abnormal neurites only in the older DS and the AD cases. An antiserum to neurofilament epitopes labeled NFTs in the older DS cases and the AD cases, but not in the younger DS cases, except for two intraneuronal NFTs in the 27 year old case.(ABSTRACT TRUNCATED AT 250 WORDS)
大多数唐氏综合征(DS)患者随着年龄增长会出现认知功能过早衰退,并最终发展为阿尔茨海默病(AD)的神经病理变化,包括含淀粉样蛋白的神经炎斑块以及神经原纤维缠结的形成。杏仁核是老年DS患者和AD患者明显神经病理变化的焦点。我们用免疫细胞化学和组织化学方法检查了6例DS患者(年龄分别为19、20、27、29、56和64岁)的杏仁核,并将其与4例AD患者(年龄分别为54、76、77和80岁)进行比较。一种针对A4淀粉样肽的抗血清在所有10例病例中均显示出斑块中的淀粉样蛋白沉积。通过用于糖胺聚糖的阿尔辛蓝染色以及 Bielschowsky 和 Bodian 银染色,在所有病例中也都发现了斑块。一种针对α-1-抗糜蛋白酶(ACT)的抗血清在AD病例以及19、5岁和64岁的DS病例中显示出斑块。仅在老年DS患者和AD患者中,用银染色观察到了神经原纤维缠结,而在19、20、27和29岁的DS病例中未观察到。同样,针对成对螺旋丝、微管相关蛋白tau和微管相关蛋白-2(MAP-2)以及泛素的抗血清,这些都是神经原纤维缠结的组成部分,仅在老年DS患者和AD患者中与缠结和异常神经突发生反应。一种针对神经丝表位的抗血清在老年DS患者和AD患者中标记了神经原纤维缠结,但在年轻DS患者中未标记,除了27岁病例中有两个神经元内神经原纤维缠结。(摘要截断于250字)