What Is Progressive Multifocal Leukoencephalopathy?
Progressive multifocal leukoencephalopathy is a progressive demyelinating disorder of the central nervous system (brain) caused by JC virus that leads to rapid death.
Progressive multifocal leukoencephalopathy usually occurs as an opportunistic infection in patients with underlying depression of cell-mediated immunity. It has been recognized that the JC virus is highly prevalent in the adult population, with 50–90% of healthy individuals having been exposed to the virus. Approximately 85% of the population has antibodies to JC virus. The virus’ purported site of latency in the human body is currently under debate.1
Progressive multifocal leukoencephalopathy is characterized by tissue loss of subcortical white matter (brain tissue) and active perivascular inflammatory foci (locations in blood vessels) with numerous eosinophilic granulocytes (white blood cells).
Q: What is demyelinating?
A: Demyelinating means there is damage to the myelin sheath of nerve cells called oligodendrites in the brain. In this disorder the damaged, irregular areas caused by the infection get progressively bigger.
The myelin sheath is a fatty substance that surrounds and protects nerve cells and enhances the transmission of nerve impulses much like the covering of a lamp cord keeps the electricity flowing within it from the plug to the light bulb. Damage to the myelin sheath impairs transmission of nerve impulses in the way that fraying an electric cord impairs the flow of electricity.
What Is Progressive Multifocal Leukoencephalopathy In Celiac Disease and/or Gluten Sensitivity?
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Gourineni VC, Juvet T, Kumar Y, Bordea D, Sena KN. Progressive multifocal leukoencephalopathy in a 62-year-old immunocompetent woman. Case Rep Neurol Med. 2014;2014:549271. doi: 10.1155/2014/549271. ↩