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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

Synopsis

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.

Codes

ICD10CM:
A81.2 – Progressive multifocal leukoencephalopathy

SNOMEDCT:
22255007 – Progressive multifocal leukoencephalopathy

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

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Therapy

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Drug Reaction Data

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References

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Last Updated:09/11/2018
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Progressive multifocal leukoencephalopathy
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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.