Posterior Reversible Encephalopathy Syndrome: A Case Report With Contemporary Literature Review and Neuropathological Evidence From Autopsy

后部可逆性脑病综合征:一例病例报告,并结合当代文献回顾和尸检神经病理学证据

阅读:1

Abstract

Posterior reversible encephalopathy syndrome (PRES) is a neuro-radiological condition characterized by vasogenic edema in the parieto-occipital brain regions. Although typically reversible, some cases result in irreversible damage, particularly with delayed treatment. This study reviews 12 autopsy-confirmed cases and presents a novel case, emphasizing neuropathological findings that challenge the assumption of reversibility. Common pathological features include white matter edema, axonal swelling, and fibrinoid necrosis. Risk factors for PRES include hypertension (HTN), autoimmune diseases, renal failure, immunosuppressive therapies, and organ transplantation. Clinical presentations range from headaches and seizures to coma, while MRI often shows T2 hyperintensities in posterior brain regions, which may resolve in follow-up imaging. Electroencephalogram (EEG) in the presented case demonstrated moderate diffuse slowing and some focal abnormalities, suggestive of cerebral dysfunction related to PRES. This novel case involves a 27-year-old homeless African American woman with a history of autoimmune disease, who presented with pain, weight loss, and weakness. Initial findings included elevated inflammatory markers and antibodies, leading to diagnoses of polymyositis, kidney injury, and pericardial effusion. During hospitalization, she experienced seizures and was suspected of having anti-NMDA (anti-N-methyl-D-aspartate) receptor encephalitis, for which she received IV immunoglobulins. MRI findings aligned with PRES. Despite interventions, including plasma exchange and rituximab therapy, she experienced cardiac arrest and ultimately passed away. Autopsy data provides crucial insights into the progression of PRES, emphasizing the importance of prompt diagnosis and treatment to prevent irreversible damage and fatal complications.

特别声明

1、本页面内容包含部分的内容是基于公开信息的合理引用;引用内容仅为补充信息,不代表本站立场。

2、若认为本页面引用内容涉及侵权,请及时与本站联系,我们将第一时间处理。

3、其他媒体/个人如需使用本页面原创内容,需注明“来源:[生知库]”并获得授权;使用引用内容的,需自行联系原作者获得许可。

4、投稿及合作请联系:info@biocloudy.com。