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

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.