Schänzer Anne, Dittmayer Carsten, Weis Joachim, Stenzel Werner, Goebel Hans-Hilmar
Institut für Neuropathologie, Justus-Liebig-Universität Gießen, Arndtstr. 16, 35392, Gießen, Deutschland.
Institut für Neuropathologie, Charité - Universitätsmedizin Berlin, Corporate Member der Freien Universität Berlin und Humboldt-Universität zu Berlin, Berlin, Deutschland.
Pathologie (Heidelb). 2023 Mar;44(2):113-120. doi: 10.1007/s00292-022-01178-x. Epub 2023 Jan 30.
In the diagnosis of diseases of the central and peripheral nervous systems, the use of electron microscopic analyses has become rare these days. However, there are questions in which the method is helpful in confirming the etiopathogenesis of the disease. Hereditary neurodegenerative and metabolic diseases, such as the lysosomal storage disease neuronal ceroid lipofuscinosis, are associated with pathognomonic storage products not only in the central nervous system (CNS) but also in extracerebral tissues such as sweat glands and lymphocytes. These tissues are easily accessible and thus function as "windows to the CNS". In addition, there are new methods that overcome limitations of conventional electron microscopy and may improve ultrastructural diagnostics. This is particularly important for the correct classification of viral particles such as SARS-CoV‑2, leading to a better understanding of COVID19-associated diseases in the CNS and peripheral nervous system.
如今,在中枢神经系统和周围神经系统疾病的诊断中,电子显微镜分析的应用已变得罕见。然而,在某些问题上,该方法有助于确认疾病的病因发病机制。遗传性神经退行性和代谢性疾病,如溶酶体贮积病神经元蜡样脂褐质沉积症,不仅在中枢神经系统(CNS),而且在脑外组织如汗腺和淋巴细胞中都与特征性的贮积产物有关。这些组织易于获取,因此可作为“中枢神经系统的窗口”。此外,还有一些新方法克服了传统电子显微镜的局限性,可能会改善超微结构诊断。这对于正确分类诸如严重急性呼吸综合征冠状病毒2(SARS-CoV-2)等病毒颗粒尤为重要,有助于更好地理解中枢神经系统和周围神经系统中与2019冠状病毒病(COVID-19)相关的疾病。