Progressive multifocal leukoencephalopathy.
Explore the source record for details and available documents.
SEARCH · PubMed Health
Explore indexed PubMed citations for clinical trials, systematic reviews and public health research. Read source abstracts and follow each citation to its original PubMed record.
Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Progressive Multifocal Leukoencephalopathy (PML) is a subacute demyelinating opportunistic infection of the central nervous system which frequently occurs in AIDS. CT and MR, together with clinical and virological findings, can suggest a correct diagnosis in most cases, thus avoiding stereotactic biopsy which is too invasive considering the lack of therapy and the poor prognosis of this disease. In this study we reviewed the CT and MR findings of 16 proved AIDS-related PML cases. PML lesions appeared as hypodense on CT, hypointense on T1w, hyperintense on PDw and T2w MR images. CT was less sensitive than T2w MR images and underestimated the number of lesions and/or disease extent. On the basis of our findings during the progression of the lesions we observed two different patterns of PML presentation and evolution i.e., "single" and "multifocal". Single lesions generally involve subcortical white matter (arcuate fibers) of parietal lobe and spread to the contralateral hemisphere across the corpus callosum; multiple "patchy" lesions can be localized variably in the cerebral hemispheres and also in the brainstem and cerebellar hemispheres.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
We present the case of a 26-year-old human immunodeficiency virus-seropositive man who developed progressive multifocal leukoencephalopathy as the initial manifestation of AIDS. He appears to have responded dramatically to therapy with 3'-azido-3'-deoxythymidine (AZT). His neurologic status deteriorated shortly after an AZT dose reduction. He has stabilized since resuming his previous AZT dose. Although it remains unclear whether AZT is useful in the treatment of JC virus infection, we think that all AIDS patients with progressive multifocal leukoencephalopathy should be offered treatment with AZT, especially in light of recent reports describing a possible potentiation of human immunodeficiency virus infection of the central nervous system in this setting.
Progressive multifocal leukoencephalopathy (PML) is caused by the human polyomavirus JC (JCV), and there are at least 4 different genotypes of JCV in the United States. Type 1 strains are of European origin, whereas type 2 and 3 strains are of Asian and African origin, respectively. JCV type 4 strains are derived from a type 1/3 recombinant. In this study, the genotype distribution of JCV strains found in brain tissue or cerebrospinal fluid of 50 PML patients was compared with JCV genotypes excreted in the urine of 103 control subjects. Type determination was based on the polymerase chain reaction-amplified partial sequence of the VP1 coding gene and the noncoding region left of ori. Brain tissues from patients with PML were infected with a significantly higher proportion of JCV type 2 strains than were urine samples from the control group (P = .004). This evidence indicates a biologic difference between JCV genotypes and suggests a difference in their potential to cause PML.
We report here a 55-year-old man with progressive multifocal leukoencephalopathy (PML) associated with chronic adult T cell leukemia (ATL). Neurological examination revealed mild dementia, right homonymous hemianopsia and visual agnosia. Serologically anti-HTLV-I antibody was positive. Peripheral blood analysis showed ATL cells up to 23% in white blood cells. Because he did not have symptoms or signs directly related to ATL, it was considered that he had chronic ATL. T2-weighted cranial MRI demonstrated multiple hyperintensity lesions confined to the white matter from the bilateral occipital to parietal lobes, without enhancement after gadolinium administration or mass effect. We performed stereotactic biopsy of the left occipitoparietal white matter. Histological examination of the biopsied specimens showed demyelinated lesions, containing foamy macrophages and bizarre astrocytes. Oligodendrocytes contained nuclear inclusions which reacted with an antibody against the JC virus (JCV) antigen. These findings were consistent with those of PML. The genomic analysis of JCV from the biopsied brain revealed deletions in the regulatory region. We investigated cerebral blood flow, glucose and amino acid metabolism in this patient using positron emission tomography, and obtained the following three characteristic findings in the lesions: 1) luxury perfusion state, 2) decreased fluorodeoxyglucose (FDG) uptake, and 3) increased methionine (Met) uptake. These findings resembled those of low grade tumors.