progressive multifocal leukoencephalopathy
Also found in: Dictionary, Acronyms, Encyclopedia, Wikipedia.
Related to progressive multifocal leukoencephalopathy: subacute sclerosing panencephalitis
Progressive Multifocal Leukoencephalopathy
Progressive multifocal leukoencephalopathy (PML) is a rapidly progressive neuromuscular disease caused by opportunistic infection of brain cells (oligodendrocytes and astrocytes) by the JC virus (JCV).
PML is an opportunistic infection associated with AIDS and certain cancers. It occurs in people with inadequate immune response and carries a poor prognosis. The incidence of PML, once quite rare, is rising as the numbers of people living with persistently compromised immune systems rises. An estimated 2-7% of people with HIV disease will develop PML. The infection also occurs among people undergoing long-term chemotherapy for cancer. PML is not considered a contagious disease. According to the Centers for Disease Control definition of AIDS, PML in the presence of HIV infection is sufficient to form a diagnosis of AIDS.
Causes and symptoms
Although at least 80% of the adults in the United States have been exposed to JC virus (as evidenced by the presence of antibodies to this virus), very few will develop PML. Little is certain about what causes JCV to produce active disease, but the virus persists in the kidneys of otherwise healthy people without making them ill. Recent evidence suggests that after prolonged compromise of the immune system, the virus changes into a form that can reach brain tissue and cause disease. In PML, the JCV infects and kills the cells (oligodendrocytes) that produce myelin, which is needed to form the sheath that surrounds and protects nerves.
About 45% of people with PML experience vision problems, most often a blindness affecting half of the visual field of each eye. Mental impairment affects about 38% of people with PML. Eventually, about 75% experience extreme weakness. Other symptoms include lack of coordination, paralysis on one side of the body (hemiparesis), and problems in speaking or using language.
Diagnosis is difficult, but usually relies on a neurologist and radiologist assessing the white matter of the brain on a computed tomography scan or a magnetic resonance imaging (MRI). Tests of the cerebrospinal fluid can help distinguish between PML and other diseases, such as multiple sclerosis and acute hemorrhagic leukoencephalopathy. The rapid clinical progression in immunocompromised patients is another distinguishing factor.
Currently, there is no known cure for PML, although it sometimes responds to treatment in patients with AIDS who are taking anti-HIV drugs (such as AZT, alpha-interferon, and peptide T). Although several agents have shown some potential in the last few years, such as the highly toxic cancer drug cytarabine, none are safe enough or sufficiently effective to be approved for PML.
PML is usually a very aggressive disease. The time between the onset of symptoms and death can be as little as one to six months. However, some patients infected with HIV have improved without receiving treatment specifically for PML.
Royal III, Walter. "Update on Progressive MultifocalLeukoencephalopathy." The Hopkins HIV Report 9 (March 1997).
Multifocal — Having many focal points. In progressive multifocal leukoencephalopathy, it means that damage caused by the disease occurs at multiple sites.
Opportunistic infection — A illness caused by infecting organisms that would not be able to produce disease in a person with a healthy immune system, but are able to take advantage of an impaired immune response.
pro·gress·ive mul·ti·fo·cal leu·ko·en·ceph·a·lop·a·thy (PML),
a rare, afebrile, fatal disease caused by the human polyomavirus JC; clinically characterized by progressive, widespread neurologic deficits, including impairment of higher cortical functions, visual problems, hemiparesis, and cerebellar dysfunction; pathologically consists of multiple disseminated areas of demyelination surrounded by markedly altered neuroglia; typically occurs in patients with disturbed immune systems (for example, those with AIDS, leukemia, lymphoma, or those receiving immunosuppressive therapy).
Synonym(s): progressive subcortical encephalopathy
progressive multifocal leukoencephalopathyA demyelinising CNS lesion, caused by a human papovavirus, usually JC virus (rarely also BK virus), which affects ±4% of AIDS patients.
Dementia, spastic paralysis, blindness, opportunistic infections; PML occurs in immunocompromised hosts—e.g., with leukaemia or AIDS.
Cytarabine; antiretrovirals are very ineffective.
progressive multifocal leukoencephalopathyNeurology A demyelinizing CNS lesion, caused by a human papovavirus, usually JC virus, rarely also BK virus, affecting ±4% of AIDS Pts Clinical Dementia, spastic paralysis, blindness, opportunistic infections; PML occurs in immunocompromised hosts–eg, with leukemia, AIDS Management Cytarabine, antiretrovirals are useless
pro·gres·sive mul·ti·fo·cal leu·ko·en·ceph·a·lop·a·thy(PML) (prŏ-gres'iv mŭl'tē-fō'kăl lū'kō-en-sef'ă-lop'ă-thē)
A rare, subacute, afebrile disease characterized by areas of demyelinization surrounded by markedly altered neuroglia, including inclusion bodies in glial cells; it occurs usually in people with AIDS, leukemia, lymphoma, or other debilitating diseases, or in those who have been receiving immunosuppressive treatment. Caused by JC virus, a human polyoma virus.