Progressive multi-focal leucoencephalopathy (PML) is a demyelinating disease of the central nervous system caused by the JC virus. Despite many people being infected by the JC virus PML is very rare, generally only occurring in association with conditions such as:
- HIV
- CLL
- patients on natalizumab
Presentations include:
- motor weakness
- impaired vision
- mental changes
- speech abnormalities
- seizures
Investigations
- CT: subcortical hypodensities
- MRI: hyperintense lesions on T2-weighted images, hypointense on T1-weighted images
- CSF: often normal; do PCR on CSF for JC virus
Management:
- in patients with HIV: start HAART
- in patients on natalizumab: stop natalizumab and initiate plasmapheresis
Prognosis: generally very poor.
References
Berger, J. The clinical features of PML. Cleve Clin J Med. 2011; 78 Suppl 2: S8-12.
Berger, J. and Khalil, K. The pathogenesis of progressive multifocal leukocencephalopathy. Discov Med. 2011; 12(67): 495-503.
Calabrese, L. A rational approach to PML for the clinician. Cleve Clin J Med. 2011; 78 Suppl 2 S38-41.
Berger, J. The clinical features of PML. Cleve Clin J Med. 2011; 78 Suppl 2: S8-12.
Berger, J. and Khalil, K. The pathogenesis of progressive multifocal leukocencephalopathy. Discov Med. 2011; 12(67): 495-503.
Calabrese, L. A rational approach to PML for the clinician. Cleve Clin J Med. 2011; 78 Suppl 2 S38-41.
