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Progressive multifocal leukoencephalopathy
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Progressive multifocal leukoencephalopathy

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Contributors: Andrea Wasilewski MD, Jamie Adams MD, Richard L. Barbano MD, PhD
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Synopsis

Progressive multifocal leukoencephalopathy : Aphasia, Ataxia, Extremities weakness, Mental status alteration, Visual impairment, Asthenia, Cognitive decline
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

Look For

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

Best Tests

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Management Pearls

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Therapy

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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.

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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 © 2023 VisualDx®. All rights reserved.