Semin Neurol 1999; 19(2): 193-200
DOI: 10.1055/s-2008-1040837
© 1999 by Thieme Medical Publishers, Inc.

Progressive Multifocal Leukoencephalopathy

Joseph R. Berger1 , Eugene O. Major2
  • 1Department of Neurology, University of Kentucky College of Medicine, Lexington, Kentucky
  • 2Laboratory of Molecular Medicine and Neuroscience, National Institute of Neurological Diseases and Stroke, National Institutes of Health, Bethesda, Maryland
Further Information

Publication History

Publication Date:
19 March 2008 (online)

ABSTRACT

Before the AIDS epidemic, progressive multifocal leukoencephalopathy (PML) was a rare disorder occuring most often in association with leukemia and lymphoma. Current estimates indicate that PML ultimately develops in up to 5% of all patients with AIDS. This demyelinating disease results from infection with JC virus, a papova virus, that most of the world's population is exposed to prior to adulthood. Although PML commonly occurs in the setting of advanced immunosuppression, it may be observed in patients with CD4 lymphocyte counts in excess of 200 cells/mm3. Focal neurological symptoms and signs coupled with hyperintense signals abnormalities of the white matter on T2-weighted cranial magnetic resonance imaging are highly suggestive of the disease. In this setting, a positive CSF polymerase chain reaction for JCV DNA has been felt to be sufficently diagnostic to elimate the need for brain biopsy. Survival of AIDS-associated PML is poor with median survivals averaging just 6 months. However, as many as 10% of AIDS patients with PML will have prolonged (>12 months) survival and partial recovery. Highly active antiretroviral therapy (HAART) has been demonstrated to have a salutary effect on survival.