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Updated: Jul 13, 2026

Induction and Clinical Scoring of Chronic-Relapsing Experimental Autoimmune Encephalomyelitis
Published on: July 4, 2007
[Review of progressive multifocal leukoencephalopathy]
1Department of Neurology, Tokyo Metropolitan Cancer and Infectious Disease Center, Komagome Hospital.
Abstract:
Progressive multifocal leukoencephalopathy (PML) is a fatal demyelinating disease of the central nervous system caused by a reactivation of the JC virus (JCV). PML occurs almost exclusively in the setting of cellular immune deficiency and its prevalence has recently increased greatly, in line with the AIDS (acquired immunodeficiency syndrome) epidemic in Western countries. A Japanese epidemiological survey showed an increasing trend in the number of AIDS-PML cases. To date, there are no effective and specific therapies for PML. As patients with AIDS-PML may benefit from combined antiretroviral therapy, efforts to improve the immune status of such patients is therefore considered to be a key factor in achieving a successful outcome for PML cases. However, PML deficits are nevertheless expected to be permanent and most cases of PML result in death within a few months. It is therefore hoped that improvements in diagnostic modalities can be made which can eventually allow for the earlier detection of PML while, at the same time, improved curative therapies for PML can also be developed in the near future.
Insights
Progressive multifocal leukoencephalopathy (PML), a fatal brain disease, is caused by JC virus reactivation in immune-deficient individuals, particularly those with AIDS. Current treatments focus on immune restoration, as specific therapies are lacking and outcomes remain poor.
Area of Science:
- Neurology
- Virology
- Immunology
Context:
- Progressive multifocal leukoencephalopathy (PML) is a rare, fatal demyelinating disease of the central nervous system.
- PML is caused by the reactivation of the JC virus (JCV) in immunocompromised individuals.
- The prevalence of PML has increased significantly, particularly in patients with acquired immunodeficiency syndrome (AIDS).
Purpose:
- To review the current understanding of PML, its association with AIDS, and epidemiological trends.
- To highlight the challenges in treating PML and the importance of immune reconstitution.
- To emphasize the need for improved diagnostic and therapeutic strategies for PML.
Summary:
- PML is a severe neurological condition resulting from JC virus reactivation, predominantly affecting individuals with compromised cellular immunity, such as those with AIDS.
- While combined antiretroviral therapy (cART) can improve immune status in AIDS-PML patients, it does not directly treat PML, and deficits are often permanent.
- The Japanese epidemiological survey indicates a rising incidence of AIDS-associated PML.
Impact:
- PML poses a significant threat to immunocompromised populations, necessitating urgent development of effective treatments.
- Early detection through improved diagnostic modalities is crucial for potentially better patient outcomes.
- Further research into specific anti-JCV therapies and immune-based strategies is essential for combating PML.
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