Practical Management of Progressive Multifocal Leukoencephalopathy and Immune Reconstitution Inflammatory Syndrome

Area of Science:

• Neuroimmunology
• Viral Encephalopathies
• Clinical Neurology

Background:

• Progressive multifocal leukoencephalopathy (PML) presents a significant clinical challenge.
• Disease presentation is influenced by the host's immune response.
• Accurate disease characterization is crucial for effective management.

Purpose of the Study:

• To provide physicians with practical, evidence-informed guidance on PML diagnosis and management.
• To outline clinical scenarios for suspecting PML.
• To detail patient evaluation at diagnosis and during follow-up.

Main Methods:

• Narrative review of published literature.
• Incorporation of authors' collective clinical experience.
• Emphasis on characterizing disease status, including inflammatory features and IRIS.

Main Results:

• PML presentation varies based on immune response strength.
• Prognostic markers aid in evaluating immune recovery and viral control.
• Experimental immunotherapies may be considered but require careful risk-benefit assessment.
• Not all inflammatory PML presentations necessitate corticosteroid therapy.

Conclusions:

• Standardizing PML assessment protocols can improve data quality and facilitate collaboration.
• Individualized, risk-benefit-based decision-making is essential for treatment.
• Management strategies should adapt to immune reconstitution and viral control status.