PDF(Pubmed)
Abstract：
Background: The postmortem diagnostic of individuals having suffered presumptive neurodegenerative disease comprises exclusion of a prion disease, extensive brain sampling and histopathological evaluation, which are resource-intensive and time consuming. To exclude prion disease and to achieve prompt accurate preliminary diagnosis, we developed a fast-track procedure for the histopathological assessment of brains from patients with suspected neurodegenerative disease. Methods: Based on the screening of two brain regions (frontal cortex and cerebellum) with H&E and six immunohistochemical stainings in 133 brain donors, a main histopathological diagnosis was established and compared to the final diagnosis made after a full histopathological work-up according to our brain bank standard procedure. Results: In over 96 % of cases there was a concordance between the fast-track and the final main neuropathological diagnosis. A prion disease was identified in four cases without prior clinical suspicion of a prion infection. Conclusion: The fast-track screening approach relying on two defined, easily accessible brain regions is sufficient to obtain a reliable tentative main diagnosis in individuals with neurodegenerative disease and thus allows for a prompt feedback to the physicians. However, a more thorough histological work-up taking into account the clinical history and the working diagnosis from fast-track screening is necessary for accurate staging and for assessment of co-pathologies.
摘要：
背景：患有推定神经退行性疾病的个体的死后诊断包括排除朊病毒疾病，广泛的脑部取样和组织病理学评估，这是资源密集型和耗时的。为了排除朊病毒病，达到及时准确的初步诊断，我们开发了一种快速程序,用于对疑似神经退行性疾病患者的大脑进行组织病理学评估.方法:在对133名脑供者进行H&E和6次免疫组织化学染色的基础上,对2个脑区(额叶皮质和小脑)进行筛查,根据我们的脑库标准程序,建立了主要的组织病理学诊断,并与经过全面组织病理学检查后的最终诊断进行了比较.结果：在超过96％的病例中，快速通道与最终的主要神经病理学诊断之间存在一致性。在四例病例中发现了pr病毒疾病，但先前没有临床怀疑pr病毒感染。结论：快速筛查方法依赖于两个定义的，容易接近的大脑区域足以在患有神经退行性疾病的个体中获得可靠的初步主要诊断，因此可以向医生提供及时的反馈。然而,考虑到临床病史和快速筛查的有效诊断,进行更彻底的组织学检查对于准确分期和评估共病是必要的.
