What Is Progressive Multifocal Leukoencephalopathy?

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Progressive multifocal leukoencephalopathy (PML) is a disease that attacks part of your brain. It happens if your body can't fight off disease the way it should. It damages your brain's white matter -- cells that make a substance called myelin. This protects your nerves, and losing it can make it harder for you to move, think, and feel sensations. PML is a very serious illness that can be fatal. It's caused by a virus called the JC virus. Most adults carry it, and it usually doesn't cause any health problems. But it can if you have a weak immune system -- if your body's natural defenses against illness aren't working right. Key word: progressive multifocal leukoencephalopathy
what can be done or drug therapy can stop or cure this affliction?
Scientists have been studying several drugs for PML but in vain. However, there is no doubt that the best way to treat PML is to fight whatever has made your immune system weak. You might take drugs to attack the virus that causes AIDS (HIV) or avoid drugs that can affect your immune system.

Progressive multifocal leukoencephalopathy (PML) is a deadly demyelinating disease of the central nervous system caused by a reactivation of the JC virus that occurs almost exclusively in immunosuppressed individuals.
 

PML is a major opportunistic infection associated with HIV infection and AIDS, with a prevalence of about 5 percent. Outside of HIV infection, PML occurs rarely in patients with lymphoproliferative and myeloproliferative diseases, solid organ malignancies, granulomatous and inflammatory diseases, and organ transplant recipients. Rare cases of PML have been reported in patients treated with the immunomodulatory medication natalizumab.
 

PML typically presents with subacute neurologic deficits including altered mental status, visual symptoms such as hemianopia and diplopia, hemiparesis or monoparesis, and diplopia, hemiparesis or monoparesis, and appendicular or gait ataxia. Seizures occur in up to 18 percent of patients.

New onset or clinical worsening of PML may occur shortly after the initiation of combined antiretroviral therapy (ART) due to the immune reconstitution inflammatory syndrome (IRIS).
 

JC virus granule cell neuronopathy presents with ataxia and cerebellar atrophy in patients with HIV infection who do not have obvious PML lesions in the cerebellum.
 

Unusual manifestations of JC virus include infection of cortical neurons causing encephalopathy and infection of leptomeningeal cells causing meningitis.
 

Clinical suspicion for PML should be heightened if neuroimaging reveals a multifocal process limited to the white matter that does not conform to vascular territories and exhibits neither mass effect nor contrast enhancement. In patients with HIV infection, the differential diagnosis of PML includes HIV encephalopathy and primary central nervous system lymphoma.
 

Brain biopsy is the gold standard for diagnosis of PML but is associated with clinically significant morbidity and mortality. In patients with appropriate neurologic and neuroradiologic features, the diagnosis of PML can usually be established by polymerase chain reaction detection of JC virus DNA in the cerebrospinal fluid if brain biopsy is not desired. However, the sensitivity of polymerase chain reaction for the diagnosis of PML in patients with HIV infection appears to be declining since the advent of combined ART therapy.
 

The differential diagnosis of PML includes HIV encephalopathy, primary central nervous system lymphoma, primary angiitis of the central nervous system, reversible posterior leukoencephalopathy, varicella-zoster virus encephalopathy, and new or recurrent attacks of multiple sclerosis.

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