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

## TL;DR

This paper presents a case of a fatal PRES condition and reviews autopsy findings to highlight the importance of timely diagnosis and treatment.

## Contribution

The paper provides novel neuropathological evidence from autopsy data in a case of PRES, challenging the assumption of reversibility.

## Key findings

- Autopsy findings revealed irreversible damage in PRES cases, including white matter edema and axonal swelling.
- The case highlights the association of PRES with autoimmune disease and immunosuppressive therapy.
- MRI findings aligned with PRES diagnosis, but the patient's condition progressed to fatal complications despite treatment.

## 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.