Posterior Reversible Encephalopathy Syndrome: An Expanding Phenotype

Area of Science:

• Neurology
• Critical Care Medicine

Background:

• Posterior Reversible Encephalopathy Syndrome (PRES) is a clinicoradiological entity characterized by neurological symptoms and characteristic white matter changes on neuroimaging.
• Traditionally associated with hypertension and certain medical conditions, PRES is increasingly recognized to have a broader spectrum of presentations.

Discussion:

• The typical PRES phenotype includes headache, seizures, visual disturbances, and altered mental status.
• However, atypical presentations such as focal neurological deficits, encephalopathy, and even stroke-like symptoms are being reported.
• Neuroimaging findings, while often showing posterior white matter edema, can also manifest differently, necessitating careful interpretation.

Key Insights:

• PRES exhibits an expanding phenotype, encompassing a wider range of neurological symptoms and imaging findings than previously understood.
• Early recognition of atypical PRES is crucial for timely intervention and management to prevent irreversible neurological damage.
• The differential diagnosis for PRES should be broadened to include conditions presenting with similar neurological and radiological features.

Outlook:

• Further research is needed to elucidate the underlying pathophysiology of atypical PRES presentations.
• Developing standardized diagnostic criteria for the expanding phenotype of PRES will aid in clinical practice.
• Investigating novel therapeutic strategies for PRES, particularly for refractory cases, remains an important future direction.