Case of the Week
Section Editors: Matylda Machnowska1 and Anvita Pauranik2
1University of Toronto, Toronto, Ontario, Canada
2BC Children's Hospital, University of British Columbia, Vancouver, British Columbia, Canada
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January 31, 2011 (MAIN)
Progressive Multifocal Leukoencephalopathy
- Progressive multifocal leukoencephalopathy (PML) is a demyelinating disease caused by JC papovavirus.
- Most commonly seen in the HIV population. 20% of the cases are seen in other immunocompromised states, including rheumatologic, hematologic and oncologic conditions. Rarely, seen in immunocompetent population.
- Diagnosis can be confirmed only on histology. However, the diagnosis can be established if there is a correlation between the clinical pr, imaging appearance, and positive PCR on CSF analysis.
- Key Diagnostic Features: MR is the imaging modality of choice. Large, multifocal, asymmetric, predominantly white matter lesions, extending to involve the subcortical white matter, without any obvious mass effect are seen. Diffusion restriction and enhancement can be seen along the advancing edge of demyelination.
- DDx: HIV encephalopathy, Lymphoma, Tumefactive MS.
- Rx: HAART
