Rosenblum Jared S, Tunacao Jessa M, Nazari Matthew A, Ronk Halle, Dang Danielle D, Downing Chad, Zhuang Zhengping, Heiss John D, Smirniotopoulos James G, Bluestone Avraham, Badia James, White Joseph
1Neuro-Oncology Branch, National Cancer Institute, National Institutes of Health, Bethesda, Maryland.
2Department of Radiology, Stony Brook University Medical Center, Stony Brook, New York.
J Neurosurg Case Lessons. 2022 Dec 26;4(26). doi: 10.3171/CASE22413.
Reports of cerebrovascular ischemia and stroke occurring as predominant neurological sequelae of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection, which causes coronavirus disease 2019 (COVID-19), are increasingly evident within the literature. While various pathophysiological mechanisms have been postulated, including hypercoagulability, endothelial invasion, and systemic inflammation, discrete mechanisms for viral neurotropism remain unclear and controversial.
The authors present a unique case study of a 64-year-old male with acute COVID-19 infection and acute worsening of previously stable cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL), a rare heritable arteriopathy due to mutation in the Notch3 gene, which is critical for vascular development and tone. Delayed cranial neuropathies, brainstem fluid-attenuated inversion recovery signal, and enhancement of olfactory and vagus nerves on magnetic resonance neurography in this patient further support viral neurotropism via cranial nerves in addition to cerebral vasculature.
To the authors' knowledge, this is the first case in the literature that not only demonstrates the consequences of COVID-19 infection in a patient with altered cerebrovascular autoregulation such as CADASIL but also highlights the tropism of SARS-CoV-2 for (1) cranial nerves as a mode of entry to the central nervous system and (2) vessels as a cause of cerebrovascular ischemia.
严重急性呼吸综合征冠状病毒2（SARS-CoV-2）感染可导致2019冠状病毒病（COVID-19），文献中越来越多地出现关于脑血管缺血和中风作为该感染主要神经后遗症的报道。虽然已经提出了各种病理生理机制，包括高凝状态、内皮侵袭和全身炎症，但病毒嗜神经性的具体机制仍不明确且存在争议。
作者介绍了一个独特的病例研究，一名64岁男性患有急性COVID-19感染，且先前稳定的伴有皮质下梗死和白质脑病的大脑常染色体显性动脉病（CADASIL）急性加重，CADASIL是一种由于Notch3基因突变导致的罕见遗传性动脉病，该基因对血管发育和张力至关重要。该患者出现延迟性颅神经病变、脑干液体衰减反转恢复信号以及磁共振神经成像显示嗅神经和迷走神经强化，这进一步支持了除脑血管系统外，SARS-CoV-2通过颅神经的嗜神经性。
据作者所知，这是文献中首例不仅展示了COVID-19感染对脑血管自动调节功能改变的患者（如CADASIL患者）的影响，还突出了SARS-CoV-2对（1）作为进入中枢神经系统途径的颅神经以及（2）作为脑血管缺血病因的血管的嗜神经性的病例。
