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Progressive multifocal leukoencephalopathy
From WikEM
Contents
Background
- Progressive multifocal leukoencephalopathy (PML) is a rare, demyelinating CNS disorder
- Associated with reactivated JC virus
- Seen almost exclusively in immunosuppressed patients, usually due to:
- AIDS
- Leukemia, lymphoma, myeloproliferative disorders (with or without chemo)
- Post solid organ or stem-cell transplant
- Autoimmune disorders (with or without immune-modulating medications)
- Medications: Natalizumab, rituximab, infliximab, mycophenolate mofetil
Clinical Features
- Altered mental status
- Aphasia
- Ataxia
- Hemiparesis
- Hemiplegia and visual field disturbances
- Seizure
- Cranial nerve palsies (rare)
- Cervical spine involvement (rare)
Differential Diagnosis
Evaluation
- Evaluate for other (especially easily treatable) causes of symptoms
- MRI brain: non-enhancing, multifocal areas of white matter demyelination
- PCR for JC virus (CSF, blood, urine)
Management
- Limit duration of immunosuppression
- HIV/AIDS patients, antiretroviral therapy is key
- Cidofovir may be useful in non-AIDS patients
