Biomaterial Database

Update on progressive multifocal leukoencephalopathy. 2012

Israel Steiner, and Joseph R Berger

Department of Neurology, Rabin Medical Center-Beilinson Campus, 49100 Petach Tikva, Israel. isteiner@cc.huji.ac.il

Progressive multifocal leukoencephalopathy (PML) is a severe, often fatal, opportunistic viral infection of the central nervous system that is mainly seen in the context of AIDS and certain monoclonal immune-suppressive therapies. The causative agent, a polyoma virus, named JC virus infects only humans and there is no animal model for PML. This update focuses on information gathered in recent years on the pathogenesis of the disorder, on several clinical aspects associated with diagnosis and therapy, and on the immune reconstitution inflammatory syndrome (IRIS), a complication associated with removal of immunosuppressive therapy in PML.

UI	MeSH Term	Description	Entries
D007577	JC Virus	A species of POLYOMAVIRUS, originally isolated from the brain of a patient with progressive multifocal leukoencephalopathy. The patient's initials J.C. gave the virus its name. Infection is not accompanied by any apparent illness but serious demyelinating disease can appear later, probably following reactivation of latent virus.	Human Polyomavirus JC,JC polyomavirus,Polyomavirus, JC,John Cunningham Virus,Polyomavirus hominis 2,Polyomavirus JC, Human,Virus, John Cunningham
D007968	Leukoencephalopathy, Progressive Multifocal	An opportunistic viral infection of the central nervous system associated with conditions that impair cell-mediated immunity (e.g., ACQUIRED IMMUNODEFICIENCY SYNDROME and other IMMUNOLOGIC DEFICIENCY SYNDROMES; HEMATOLOGIC NEOPLASMS; IMMUNOSUPPRESSION; and COLLAGEN DISEASES). The causative organism is JC Polyomavirus (JC VIRUS) which primarily affects oligodendrocytes, resulting in multiple areas of demyelination. Clinical manifestations include DEMENTIA; ATAXIA; visual disturbances; and other focal neurologic deficits, generally progressing to a vegetative state within 6 months. (From Joynt, Clinical Neurology, 1996, Ch26, pp36-7)	Encephalitis, JC Polyomavirus,Progressive Multifocal Leukoencephalopathy,JC Polyomavirus Encephalopathy,Encephalopathies, JC Polyomavirus,Encephalopathy, JC Polyomavirus,JC Polyomavirus Encephalitis,Leukoencephalopathies, Progressive Multifocal,Multifocal Leukoencephalopathies, Progressive,Multifocal Leukoencephalopathy, Progressive,Progressive Multifocal Leukoencephalopathies
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D054019	Immune Reconstitution Inflammatory Syndrome	Exuberant inflammatory response towards a previously acquired or undiagnosed opportunistic infection. It is frequently seen in AIDS patients following antiretroviral treatment.	Immune Reconstitution Disease,Immune Restoration Syndrome,Immune Reconstitution Syndrome,Immune Restoration Disease,Immune Reconstitution Syndromes,Immune Restoration Diseases,Reconstitution Disease, Immune,Reconstitution Syndrome, Immune,Restoration Syndrome, Immune
D061067	Antibodies, Monoclonal, Humanized	Antibodies from non-human species whose protein sequences have been modified to make them nearly identical with human antibodies. If the constant region and part of the variable region are replaced, they are called humanized. If only the constant region is modified they are called chimeric. INN names for humanized antibodies end in -zumab.	Antibodies, Humanized,Humanized Antibodies