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Progressive multifocal leukoencephalopathy: an update on clinical and laboratory spectrum and recent developments.
Progressive multifocal leukoencephalopathy (PML) is a demyelinating disorder of the brain parenchyma caused by JC virus, and is usually observed in patients with immunocompromised conditions. The clinical presentation is heterogeneous, and includes ataxia, aphasia, limb and speech dysfunctions, and altered mental status. Various laboratory tests may be helpful in the diagnosis, including serology and PCR for JC virus DNA. Supportive care is the mainstay of the management. Allogeneic hematopoietic stem cell transplantation is considered the most effective treatment. Other modalities of therapy include various immunosuppressive treatments, such as interferon beta, and monoclonal antibodies against TNF-alpha. Although this is still a diagnosis of exclusion, most clinicians agree that brain biopsy or autopsy is required for an accurate diagnosis of PML. This is often not feasible in clinical practice, and non-invasive methods, such as magnetic resonance imaging and JC virus testing of cerebrospinal fluid, are widely used. Although the treatment remains supportive, there has been a growing interest in the development of new treatment modalities and immunosuppressive regimens. Recent advances in molecular biology have contributed significantly to this area, but we are far from a complete understanding of the pathogenesis of this disorder.Q:

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