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Progressive multifocal leukoencephalopathy
1Department of Neurology, University of Kentucky College of Medicine, Lexington 40536-0284, USA.
Abstract:
Before the AIDS epidemic, progressive multifocal leukoencephalopathy (PML) was a rare disorder occurring 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 sufficiently diagnostic to eliminate 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.
Insights
Progressive multifocal leukoencephalopathy (PML) affects up to 5% of AIDS patients, caused by JC virus. While survival is poor, highly active antiretroviral therapy (HAART) shows a positive impact.
Area of Science:
- Neurology
- Virology
- Immunology
Background:
- Progressive multifocal leukoencephalopathy (PML) was historically rare, associated with leukemia/lymphoma.
- PML now affects up to 5% of patients with acquired immunodeficiency syndrome (AIDS).
- The disease is caused by the JC virus (JCV), a papovavirus prevalent in the general population.
Purpose of the Study:
- To describe the characteristics and outcomes of PML in the context of the AIDS epidemic.
- To highlight diagnostic criteria and the impact of treatment on survival.
Main Methods:
- Review of clinical presentation, diagnostic methods (MRI, CSF PCR), and outcomes.
- Analysis of survival data in relation to CD4 counts and treatment with highly active antiretroviral therapy (HAART).
Main Results:
- PML can occur even with CD4 counts above 200 cells/mm3.
- Focal neurological deficits and white matter abnormalities on MRI are key indicators.
- Positive CSF PCR for JCV DNA is a reliable diagnostic marker, often negating the need for brain biopsy.
- Median survival for AIDS-associated PML is approximately 6 months, with some patients experiencing prolonged survival (>12 months).
Conclusions:
- AIDS-associated PML is a significant opportunistic infection with poor prognosis.
- Early diagnosis via MRI and CSF PCR is crucial.
- HAART has demonstrated a beneficial effect on survival for patients with AIDS-associated PML.