Progressive multifocal leukoencephalopathy is an opportunistic demyelinating infection of the central nervous system caused by JC virus, a human polyomavirus. It is characterized pathologically by multifocal areas of myelin loss and microscopically by lytic infection of oligodendrocytes. The disorder is rare outside the setting of HIV infection, but 4% of untreated AIDS patients may contract and succumb to the disease. The condition has also become of increasing concern in patients receiving aggressive immunosuppression for organ or stem cell transplantation or treated with natalizumab, rituximab, and other newer immunosuppressive agents.
In this article, Dr. John E Greenlee of the University of Utah School of Medicine reviews the pathogenesis, clinical features, diagnosis, and treatment of this disorder.
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