Progressive multifocal leukoencephalopathy in HIV-1 infection

• Published in: The Lancet infectious diseases Vol. 9; no. 10; pp. 625 - 636
• Main Authors: Cinque, Paola, Koralnik, Igor J, Gerevini, Simonetta, Miro, Jose M, Price, Richard W
• Format: Journal Article
• Language: English
• Published: London Elsevier Ltd 01.10.2009; Lancet Publishing Group; Elsevier Limited
• Subjects: Antiretroviral agents; Biological and medical sciences; Cerebrospinal Fluid - virology; HIV Infections - complications; Human immunodeficiency virus 1; Human viral diseases; Humans; Immunodeficiencies; Immunodeficiencies. Immunoglobulinopathies; Immunopathology; Infectious Disease; Infectious diseases; JC virus; JC Virus - isolation & purification; Lesions; Leukoencephalopathy, Progressive Multifocal - diagnosis; Leukoencephalopathy, Progressive Multifocal - epidemiology; Leukoencephalopathy, Progressive Multifocal - pathology; Leukoencephalopathy, Progressive Multifocal - therapy; Medical sciences; Polyomavirus; Viral diseases; Viral diseases of the lymphoid tissue and the blood. Aids; Viral diseases of the nervous system; Immunopathology; Nervous system diseases; HIV-1 virus; Retroviridae; AIDS; Immune deficiency; Lentivirus; Inflammatory disease; Cerebral disorder; Progressive multifocal leukoencephalopathy; Infection; Virus; Viral disease; Central nervous system disease; Human immunodeficiency virus; Slow virus
• ISSN: 1473-3099; 1474-4457; 1474-4457
• DOI: 10.1016/S1473-3099(09)70226-9

Progressive multifocal leukoencephalopathy is caused by the JC polyomavirus (JCV) and is one of the most feared complications of HIV-1 infection. Unlike other opportunistic infections, this disease can present when CD4 counts are higher than those associated with AIDS and when patients are receiving combined antiretroviral therapy, either shortly after starting or, more rarely, during long term successful treatment. Clinical suspicion of the disease is typically when MRI shows focal neurological deficits and associated demyelinating lesions; however, the identification of JCV in cerebrospinal fluid or brain tissue is needed for a definitive diagnosis. Although no specific treatment exists, the reversal of immunosuppression by combined antiretroviral therapy leads to clinical and MRI stabilisation in 50–60% of patients with the disease, and JCV clearance from cerebrospinal fluid. A substantial proportion of patients treated with combined antiretroviral therapy develop inflammatory lesions, which can be associated with either a favourable outcome or clinical worsening. The reasons for variability in the natural history of progressive multifocal leukoencephalopathy and treatment responses are largely undefined, and more specific and rational approaches to management are needed.