Kitamoto T, Tateishi J, Sato Y
Department of Neuropathology, Faculty of Medicine, Kyushu University, Japan.
Ann Neurol. 1988 Oct;24(4):537-42. doi: 10.1002/ana.410240410.
We investigated paraffin-embedded brain sections from 41 patients with Creutzfeldt-Jakob disease (CJD) and from 9 with Gerstmann-Sträussler syndrome (GSS) using anti-human prion protein (PrP) antisera (anti-GSS kuru plaque cores and anti-PrP synthetic peptide) and anti-beta protein antiserum. The anti-human PrP antiserum reacted with the plaques in CJD and GSS, with or without degenerative neurites (neuritic components). In addition, the anti-beta protein antiserum immunolabeled kuru plaque-like compact plaques in some cases of CJD. Therefore, previous morphological classifications of the plaques may not always be valid. Senile plaques labeled with anti-beta protein antiserum were evident in 65% of the CJD brains and 50% of GSS brains from patients in their 60s, and in 73% of brains from CJD patients in their 70s, but not in brains from patients under 60 years of age. The incidence of the senile plaques was compatible with the normal aging process and was apparently not accelerated by the disease process of CJD or GSS. These immunostaining approaches using anti-human PrP and anti-beta protein antisera allow classification of plaque types and increase the reliability of the pathological diagnosis in persons with dementia.
我们使用抗人朊蛋白（PrP）抗血清（抗GSS库鲁病斑块核心和抗PrP合成肽）以及抗β蛋白抗血清，对41例克雅氏病（CJD）患者和9例格斯特曼-施特劳斯勒综合征（GSS）患者的石蜡包埋脑切片进行了研究。抗人PrP抗血清与CJD和GSS中的斑块发生反应，无论有无变性神经突（神经炎性成分）。此外，抗β蛋白抗血清在某些CJD病例中对库鲁病斑块样致密斑块进行了免疫标记。因此，先前对斑块的形态学分类可能并非总是有效。用抗β蛋白抗血清标记的老年斑在60多岁的CJD患者脑中有65%可见，在60多岁的GSS患者脑中有50%可见，在70多岁的CJD患者脑中有73%可见，但在60岁以下患者的脑中未见。老年斑的发生率与正常衰老过程相符，显然未因CJD或GSS的疾病过程而加速。这些使用抗人PrP和抗β蛋白抗血清的免疫染色方法有助于对斑块类型进行分类，并提高痴呆患者病理诊断的可靠性。
