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Amyotrophic lateral sclerosis
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Spinocerebellar ataxia (NORD)
Tethered spinal cord syndrome
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Adult brain tumors: Pathology review
Central nervous system infections: Pathology review
Cerebral vascular disease: Pathology review
Congenital neurological disorders: Pathology review
Dementia: Pathology review
Demyelinating disorders: Pathology review
Headaches: Pathology review
Movement disorders: Pathology review
Neurocutaneous disorders: Pathology review
Neuromuscular junction disorders: Pathology review
Pediatric brain tumors: Pathology review
Seizures: Pathology review
Spinal cord disorders: Pathology review
Traumatic brain injury: Pathology review
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PML Progressive Multifocal Leukoencephalopathy
HIV-positive adults p. 174
polyomaviruses p. 161
rituximab p. 445
In progressive multifocal leukoencephalopathy, progressive means that the disease worsens over time, multifocal means the disease affects multiple locations, leuko refers to white, and encephalopathy refers to a disease of the brain.
So progressive multifocal leukoencephalopathy or simply PML, is a disease of the white matter of the brain that affects multiple locations and worsens over time.
If we look at a cross section of the brain, it can be divided into two areas: the outermost area is the grey matter, which is made up of neuron cell bodies, and the innermost area is the white matter, which is made up of the axons that come off of the neuron cell bodies.
The axons transmit electrical impulses to the next neuron in the series.
The axons are surrounded by a fatty protective sheath called myelin that helps increase the speed at which electrical impulses are sent.
Myelin is produced by a special type of cells called oligodendrocytes.
The cause of progressive multifocal leukoencephalopathy is the John Cunningham virus, or simply JC virus, named after the first patient in whom the virus was identified.
JC virus is a non-enveloped virus with closed circular double-stranded DNA genome.
It’s thought that the virus is transmitted from person to person through the respiratory and gastrointestinal tract, and that it then moves through the bloodstream and eventually reaches kidney epithelial cells.
JC virus enters these kidney cells and starts replicating, but the cytotoxic CD8+ T cells of our immune system keep the virus in check by killing any cell that has replicating JV virus.
So to be clear, the virus is not eliminated, but instead it remains latent in the kidneys, meaning that it isn’t dividing and causing disease.
And It turns out that the vast majority of the population is infected by JC virus - that might include you!
Most people with a healthy immune system are able to keep JC virus in the latent phase in the kidney epithelial cells for their entire life. But things can change if the immune system gets weakened.
This can happen for various reasons, like an HIV-infected individual who loses T cells, or individuals taking immunosuppressive medications like monoclonal antibodies such as natalizumab, which is typically used to treat multiple sclerosis, and rituximab, which is used to treat various cancers and autoimmune diseases.
Progressive multifocal leukoencephalopathy (PML) is a rare, often fatal demyelinating disease of the brain, caused by JC virus. JC virus is a non-enveloped, double-stranded DNA virus that can be found latent and harmless in most adults.
However, in people with weakened immune systems, the virus can cause PML, in which the white matter in the brain gets destroyed, leading to progressive and irreversible axon demyelination that presents with motor and cognitive neurological symptoms
There is no specific treatment for PML and it is usually fatal. However, some people with PML may improve with treatment for their underlying condition (e.g., HIV/AIDS).
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