Learning objectives
To know the clinical context and recognize the imaging findings of Progressive multifocal leukoencephalopathy(PML).
To know why and when immune reconstitution inflammatory syndrome occurs (IRIS) and how to recognize it by image.
To emphasize the basic image characteristics to be able to differentiate the two entities.
Background
Progressive multifocal leukoencephalopathy (PML) is a rare but sever viral demyelinating disease of the central nervous system (CNS) caused by the polyomavirus JC (JCV).
It occurs almost exclusively in immunosuppressed individuals but more frequently occurs in AIDS than in other immunosuppressive conditions.
Immune reconstitution inflammatory syndrome (IRIS) is defined as a paradoxical clinical worsening of a known condition or the appearance of a new condition after initiating antiretroviral therapy in HIV-infected patients.
IRIS is the result an exaggerated inflammatory response to an antigen of a...
Findings and procedure details
Clinical context and recognize the imaging findings of PML
Progressive multifocal leukoencephalopathy (PML) is a rare but sever viral subacute progressive demyelinating disease of the central nervous system (CNS) caused by the polyomavirus JC (JCV) infecting oligodendrocytes.
Primary PML developing in an immunocompetent patient is very rare.
It occurs almost exclusively in immunosuppressed individuals.
Is seen in clinical contexts such as severe combined immunodeficiency (SCID),
leukemia,
lymphoma,
systemic lupus erythematosus,
and organ transplantation) but more frequently occurs in AIDS than in other immunosuppressive conditions (typically...
Conclusion
Even though IRIS in the setting of HIV infection is a diagnosis of exclusion the early detection of MRI findings to differentiate the progression of PML from IRIS is crucial clinical relevance for the treatment decision-making and clinical outcome.
References
Berger JR,
Aksamit AJ,
Clifford DB,
et al.
PML diagnostic criteria: Consensus statement from the AAN Neuroinfectious Disease Section.
Neurology.
2013;80(15):1430-1438.
doi:10.1212/WNL.0b013e31828c2fa1.
Jérôme Hodel,
Olivier Outteryck,
Céline Dubron,
Bastien Dutouquet,
Mohamed Amine Benadjaoud,
Emeline Duhin,
Sébastien Verclytte,
Marc Zins,
Alain Luciani,
Alain Rahmouni,
Jean-Pierre Pruvo,
Patrick Vermersch,
and Xavier Leclerc.
Asymptomatic Progressive Multifocal Leukoencephalopathy Associated with Natalizumab: Diagnostic Precision with MR Imaging,
Radiology 2016 278:3,
863-872
M.J.D.
Post,
M.M.
Thurnher,
D.B.
Clifford,
A.
Nath,
R.G.
Gonzalez,
R.K.
Gupta,
K.K.
Post CNS–Immune Reconstitution Inflammatory Syndrome in...
