SynopsisCodesLook ForDiagnostic PearlsDifferential Diagnosis & PitfallsBest TestsManagement PearlsTherapyDrug Reaction DataReferences
Progressive multifocal leukoencephalopathy
Other Resources UpToDate PubMed

Progressive multifocal leukoencephalopathy

Contributors: Andrea Wasilewski MD, Jamie Adams MD, Richard L. Barbano MD, PhD
Other Resources UpToDate PubMed


Progressive multifocal leukoencephalopathy (PML) is a demyelinating disease caused by reactivation of John Cunningham (JC) virus, which destroys oligodendrocytes. It is associated with human immunodeficiency virus (HIV) / AIDS, usually in patients with CD4 counts less than 200, and can also occur after initiation of highly active antiretroviral therapy. In addition, the disease is seen after treatment with immune-modulating therapies such as natalizumab.

Focal neurologic symptoms are typically subacute and evolve over weeks. Symptoms can include weakness, sensory loss, ataxia, vision changes, aphasia, cognitive decline, or behavioral changes. Seizures can also occur.

PML classically affects the subcortical white matter and U-fibers. Prognosis depends on severity of the underlying disease, although it is generally poor, and the disease is often fatal within months.


A81.2 – Progressive multifocal leukoencephalopathy

22255007 – Progressive multifocal leukoencephalopathy

Look For

Subscription Required

Diagnostic Pearls

Subscription Required

Differential Diagnosis & Pitfalls

  • Multiple sclerosis
  • Acute demyelinating encephalomyelitis
  • Creutzfeldt-Jakob disease
  • Paraneoplastic encephalomyelitis
  • Central nervous system Lymphoma
  • Central nervous system Vasculitis
  • Radiation-induced leukoencephalopathy
  • Reversible posterior leukoencephalopathy syndrome
  • Human immunodeficiency virus disease
  • Adrenomyelopathy (see Adrenoleukodystrophy)

Best Tests

Subscription Required

Management Pearls

Subscription Required


Subscription Required

Drug Reaction Data

Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.

Subscription Required


Subscription Required

Last Updated:09/11/2018
Copyright © 2024 VisualDx®. All rights reserved.
Progressive multifocal leukoencephalopathy
A medical illustration showing key findings of Progressive multifocal leukoencephalopathy : Aphasia, Ataxia, Extremities weakness, Mental status alteration, Visual impairment, Asthenia, Cognitive decline
Copyright © 2024 VisualDx®. All rights reserved.