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Medicine (MD/DO)
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USMLE Step 2
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Neurology, Ophthalmology & Otology
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Infectious Nervous System Disorders
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Progressive Multifocal Leukoencephalopathy (PML)

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Progressive Multifocal Leukoencephalopathy (PML)

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Progressive Multifocal Leukoencephalopathy (PML)

PML-license-plate
sesanch1
11 KEY FACTS
PATHOPHYSIOLOGY
Demyelination of CNS
Unraveling-myelin on CNS-brain

PML is a disease of the central nervous system that involves demyelination and is typically caused by the reactivation of the JC virus in immunosuppressed individuals.

Reactivation of JC Virus
JC-on-a-penny

PML is affiliated with the reactivation of those previously infected with the JC virus. The immunosuppressive state (e.g., HIV) of an individual triggers the reactivation of the virus, thereby allowing for the destruction of oligodendrocytes and, thus, the demyelination of the CNS.

CLINICAL FEATURES
Neurologic Deficits
Defective-neuron

PML can involve myriad symptoms, including neurological deficits (e.g., hemiplegia, ataxia, aphasia), seizures, vision changes, encephalopathy, and altered mental status. These symptoms can be slow in onset and progress over several weeks.

Seizure
Caesar

PML can involve various symptoms revolving around neurological deficits, seizures, vision changes, encephalopathy, and altered mental status. These symptoms can be slow in onset and progress over several weeks.

Vision Changes
Delta Eyes

PML can involve a myriad of symptoms, including neurological deficits (e.g., hemiplegia, ataxia, aphasia), seizures, vision changes (e.g., hemianopsia), encephalopathy, and altered mental status. These symptoms can be slow in onset and progress over several weeks.

Encephalopathy
Altered Brain

PML can involve a myriad of symptoms, including neurological deficits (e.g., hemiplegia, ataxia, aphasia), seizures, vision changes, and encephalopathy, which can lead to AMS and dementia. These symptoms can be slow in onset and progress over several weeks.

DIAGNOSIS
Brain MRI or CT
Brain M-R-eyes and Cat-scanner

The preferred method of diagnosis involves an MRI with and without the presence of gadolinium contrast. An MRI is more sensitive to white matter lesions, though a CT with contrast can also be ordered.

CD4 Count
CD (4) Fork-Helper T Cell

A CD4 count should be obtained in those with suspected PML in order to assess immunological and HIV status. In those with HIV, PML is usually in those with a CD4 < 200.

CSF Sample
Brain-Spine-Fluid Sampler

The diagnosis of PML can also involve a sampling of CSF, which can detect the JC virus via PCR. CSF, in combination with clinical and radiological findings, can also be sufficient for diagnosis.

TREATMENT
Supportive Care
Supportive IV Bags

PML treatment revolves around supportive care to address clinical symptoms. In those with HIV, ART should be started immediately. Unfortunately, despite treatment, PML has a high mortality rate with a median survival of less than one year.

ART Therapy
Graffiti Art

PML treatment revolves around supportive care to address clinical symptoms. In those with HIV, ART should be started immediately. Unfortunately, despite treatment, PML has a high mortality rate with a median survival of less than one year.

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