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PubMed2002

[Progressive multifocal leukoencephalopathy (PML) in chronic lymphatic leukemia (CLL). Review of the literature and case report].

Progressive multifocal leukoencephalopathy (PML) is an infectious disease of the central nervous system caused by the JC virus. Progressive multifocal leukoencephalopathy represents a reactivation of the JC virus after long-standing immunosuppression. Also, PML plays an important role as an opportunistic infection in patients with AIDS. The average time of survival in patients with PML in combination with chronic lymphatic leukemia (CLL) (n = 17 in the literature) is 4.3 months, and therapeutic options are not established. We report the case of a patient with CLL and PML. Clinical symptoms are slight hemiparesis of the right side, mainly appearing as a disturbance of motor function. In MRI, a typical subcortical lesion was shown, and JC virus DNA was positive in the CSF by PCR. Because of first positive results in treatment of PML in patients with AIDS, therapy with cidofovir was started. After treatment for 16 months, symptoms are stable, the PML-induced lesions in MRI are in regression, and JC virus DNA is not detectable in the CSF.

Antiviral Agents↗
PubMed2003

Progressive multifocal leukoencephalopathy in a patient with chronic myelogenous leukemia.

A 42-year-old man with chronic myelogenous leukemia presented to the hospital with hip pain. After undergoing surgical repair for a hip fracture, he developed aphasia, facial droop and fever. He was initially diagnosed with a stroke. A lumbar puncture excluded common infections, and an ELISA test for HIV was negative. Serial MRI scans revealed a progressive demyelinating pattern. Polymerase chain reaction performed on cerebrospinal fluid was positive for the JC virus (JC are the initials of the first person diagnosed with this virus). The patient was treated with cidofovir but died before a full course could be administered. JC virus is present in most adult hosts but is only harmful in immuno-compromised hosts. The active form of the disease is known as progressive multifocal leukoencephalopathy. This is the first reported case of a patient developing progressive multifocal leukoencephalopathy. with chronic myelogenous leukemia as the only underlying condition.

Adult↗
PubMed1997

Progressive multifocal leukoencephalopathy presenting as human immunodeficiency virus type 1 (HIV)-associated dementia.

Progressive multifocal leukoencephalopathy (PML) is a demyelinating disorder of the CNS that usually causes hemiparesis or hemianopsia. Dementia occurs in combination with other neurologic abnormalities. We report a human immunodeficiency virus type 1 (HIV)-infected man whose only manifestation of proven PML was dementia that was clinically indistinguishable from HIV-associated dementia.

AIDS Dementia Complex↗
PubMed1988

Positron emission tomography in a patient with progressive multifocal leukoencephalopathy.

A 56-year-old man with chronic lymphocytic leukemia, progressive multifocal leukoencephalopathy, and a dense left homonymous hemianopia had 18F-fluorodeoxyglucose positron emission tomography. Cortical glucose metabolism was decreased in the right cerebral hemisphere and the left cerebellar hemisphere. To our knowledge, this is the first demonstration of cerebral and cerebellar hypometabolism due solely to white matter disease.

Aged↗
PubMedDate not supplied

Cytologic findings in progressive multifocal leukoencephalopathy. Report of two cases.

Cytologic preparations from two cases of progressive multifocal leukoencephalopathy (PML) were obtained by stereotactically guided needle biopsies using computerized tomograms of the brain. Case 1 was a 32-year-old man with an acquired immunodeficiency syndrome-related complex. Case 2 was a 71-year-old man with chronic lymphocytic leukemia. Smears showed moderate cellularity, consisting of moderately to markedly atypical cells with enlarged hyperchromatic nuclei. The chromatin pattern showed smudging, with or without clumping, similar in pattern to the human polyomavirus-infected "decoy" cells seen in urine cytology. Nuclei were predominantly round to oval, smoothly contoured and often stripped of cytoplasm. Occasional bizarre lobulated or multinucleated forms were seen. Some atypical cells had abundant cytoplasm exhibiting stellate projections. Histologic sections of the biopsy material confirmed the diagnosis in each case. In both cases, electron microscopy demonstrated intranuclear polyoma-type virus particles. The present findings suggest that PML should be considered in the differential diagnosis of marked cytologic atypia in brain aspirates from immunocompromised patients.

AIDS-Related Complex↗
PubMed1996

DNA amplification in glial cells of progressive multifocal leukoencephalopathy: an image analysis study.

JC virus (JCV), the agent of progressive multifocal leukoencephalopathy (PML), has been shown by both immunohistochemistry and flow cytometry to be associated with p53 protein stabilization. Since stabilization/inactivation of p53 is associated with the development of genomic instability, abnormal cell DNA contents are to be expected in JCV-infected cells of PML. This work explores that possibility by image analysis evaluation of DNA content in PML-infected oligodendrocytes and bizarre astrocytes. Brain paraffin sections of PML lesions from five adult male patients with the acquired immune deficiency syndrome (AIDS) were treated with the Feulgen technique to obtain a stochiometric staining of DNA and analyzed with a microscope image processor. Inclusion-bearing oligodendrocytes exhibited near tetraploid DNA indices in each of the five cases, whereas atypical astrocytes were in the hypertetraploid range in all cases and were polyploid in four instances. This evidence of DNA amplification in PML glial cells is congruent with the functional abolition of p53 protein in association with JCV infection and lends further support to the role of p53 as a keeper of diploid status and guardian of genomic stability.

Adult↗
PubMed2005

Progressive multifocal leukoencephalopathy (PML) presenting as intractable dystonia in an HIV-infected child.

Progressive multifocal leukoencephalopathy (PML) has been described in adult immunocompromised patients and has a progressive downhill course. Though dementia and motor disturbances have been described, intractable dystonia is a very unusual manifestation. In children, PML is very rare and often misdiagnosed as other central nervous system (CNS) encephalopathy. No definitive treatment is available.

Brain↗
PubMed2001

[Progressive multifocal leukoencephalopathy. Demyelinating viral disease--common complication of AIDS].

PML is a chronic, progressive, fatal disease in the CNS of humans. Characteristic pathologic features are spotty demyelination, enlarged oligodendrocytes with nuclear inclusions, and transformed astrocytes. It is caused by the polyoma virus JCV, which has worldwide distribution and usually is harmless. In some individuals with impaired cell-mediated immunity, most commonly in aids, virus changes into a pathogenic form. Hence, PML is a slow, viral, opportunistic infection. The infection is productive in astrocytes and destructive in oligodendrocytes. Lysis of the latter causes demyelination. The discovery of PML in 1958 opened the door to extensive research in several fields of biomedicine.

AIDS-Related Opportunistic Infections↗
PubMed1992

Progressive multifocal leukoencephalopathy and gliomas in a HIV-negative patient.

A case of progressive multifocal leukoencephalopathy (PML) is reported, detected at autopsy of a 30-year-old patient. The clinical picture was characterized by a progressive course of mental deterioration and ingravescent neurological symptoms. The patient was HIV-negative. He died of bronchopneumonia, after a clinical course of 13 months. Autopsy disclosed pulmonary tuberculosis with involvement of regional lymph nodes. In the brain, besides numerous PML-foci of varying age and structure, a pleomorphic astrocytoma was found in the white matter of the right parietal lobe. In the brain stem glial proliferation resembling diffuse gliomatosis was also present. In situ hybridization revealed Papova-virus (JCV) in oligoglial nuclei, but not in neoplastic astrocytes. This is the third report on the concomitant occurrence of PML and glioma in man.

Adult↗
PubMedDate not supplied

Primary progressive multifocal leukoencephalopathy: report of a case.

The authors describe a case of primary progressive multifocal leukoencephalopathy (pPML). Unlike previous similar reports, our patient underwent up-to-date and extensive in vivo and post-mortem investigations that established beyond doubt the competence of his immune system and the absence of underlying predisposing disorders. The various implications of this case, both clinical and related to the possible pathogenetic mechanisms of JC virus infection, are discussed.

Aged↗
PubMed1995

Diagnostic value of detecting JC virus DNA in cerebrospinal fluid of patients with progressive multifocal leukoencephalopathy.

JC virus DNA was detected by PCR in the cerebrospinal fluid of 17 of 23 (73.9%) patients with confirmed cases of progressive multifocal leukoencephalopathy and 2 of 48 (4.2%) controls without progressive multifocal leukoencephalopathy. The sensitivity and specificity of this PCR were 74 and 95.8%, respectively, while the positive and negative predictive values were 89.5 and 88.5%, respectively.

DNA, Viral↗
PubMed1990

Detection of JC virus DNA by polymerase chain reaction in patients with progressive multifocal leukoencephalopathy.

Polymerase chain reaction (PCR) was used in the detection of JC virus (JCV) DNA in formalin-fixed, paraffin-embedded brain tissue sections from 24 patients with progressive multifocal leukoencephalopathy. Brain sections from normal autopsies (n = 17) and other neurologic conditions (n = 4) were used as controls. Specific amplified products were detected in 20 (83%) of 24 patients with progressive multifocal leukoencephalopathy. PCR did not amplify JCV or human beta-globin gene sequences in four patients with characteristic demyelinating lesions of progressive multifocal leukoencephalopathy that were positive for JCV by in situ hybridization or immunocytochemistry. PCR from biopsy sections resulted in more intense amplification signals than PCR from autopsy tissue. Normal brain sections from 17 autopsies were negative by PCR. Low-grade amplification of JCV was observed in one patient with herpes simplex virus encephalitis. PCR served as a more rapid test for confirmation of progressive multifocal leukoencephalopathy than in situ hybridization. However, PCR performance was altered by prolonged formalin fixation of tissue and undetermined inhibitors of the amplification reaction. Laboratories and clinicians should be aware of the difficulties encountered when using paraffin-embedded tissue for PCR.

Base Sequence↗
PubMed1996

Cytomorphology of progressive multifocal leukoencephalopathy (PML): review of sixteen cases occurring in HIV-positive patients.

Progressive multifocal leukoencephalopathy (PML) is a fatal demyelinating disorder of the central nervous system (CNS) resulting from infection of oligodendrocytes by JC virus. Although all patients immunocompromised by any congenital, acquired, or iatrogenic condition are at risk, the population which currently accounts for the majority of new cases is that infected with the human immunodeficiency virus (HIV). Though the clinical/radiologic presentation is characteristic, biopsy confirmation is necessary, as these patients are at risk for other primary CNS disorders which may produce similar clinical findings. Immediate assessment of tissue adequacy by cytologic smear is generally preferred in these specimens due to its relative reduced risk of disease transmission when compared to conventional frozen section. We report here the cytologic findings seen in touch imprints and squash preparations of 16 cases of PML, all occurring in HIV-positive patients and obtained by stereotactic guided needle biopsy. Typical cytomorphologic findings are described and correlated with histologic sections. In addition, features useful in the exclusion of other differential diagnostic possibilities are discussed.

Adult↗
PubMed2006

Two cases of progressive multifocal leukoencephalopathy after allogeneic hematopoietic cell transplantation and a review of the literature.

Progressive multifocal leukoencephalopathy (PML) is a rare subacute demyelinating disorder of the central nervous system (CNS) caused by the DNA JC human polyomavirus. In immunocompromised hosts, PML is caused by reactivation of a latent infection rather than de novo primary exposure. PML in the setting of hematopoietic cell transplantation (HCT) is exceedingly rare. PML should be considered in the differential diagnosis of HCT recipients, autologous or allogeneic, presenting with worsening of neurological symptoms, especially associated with post-transplant neurodegenerative findings. Although DNA polymerase chain reaction (PCR) of the cerebrospinal fluid (CSF) has emerged as a promising tool for detecting JC virus, a negative result does not rule out PML. Brain biopsy remains the most reliable and accurate method for diagnosing JC virus-associated PML. Presently, there is no universally effective antiviral therapy against JC virus and outcome is fatal in the majority of cases. We hereby describe two cases of PML developing after allogeneic HCT and provide a comprehensive review of the literature.

Adult↗
PubMed2003

Early events in the life cycle of JC virus as potential therapeutic targets for the treatment of progressive multifocal leukoencephalopathy.

The human polyomavirus, JC virus (JCV), is the etiological agent of progressive multifocal leukoencephalopathy (PML). PML occurs almost exclusively in the setting of severe and prolonged immunosuppression and it remains an important and life-threatening complication in the acquired immunodeficiency syndrome (AIDS) population. Several drugs that target DNA replication have shown efficacy at inhibiting JCV replication in vitro but none to date have shown in vivo efficacy. The authors' laboratory has been studying early events that contribute to infection of susceptible cells by JCV. They previously demonstrated that infection of glial cells by JCV requires clathrin-dependent endocytosis and that this early step in the viral life cycle can be blocked by the antipsychotic drug, chlorpromazine. As chlorpromazine is associated with the development of extrapyramidal symptoms that may be heightened in AIDS patients, the authors sought to test the atypical antipsychotic, clozapine, for antiviral activity against JCV. In this report, the authors show that clozapine is as effective as chlorpromazine at inhibiting infection. They further demonstrate that low-dose combinations of both drugs synergistically inhibit infection.

Antipsychotic Agents↗
PubMed1999

Remission of progressive multifocal leukoencephalopathy following highly active antiretroviral therapy in a patient with HIV infection.

Progressive multifocal leukoencephalopathy (PML) is a demyelinating disease resulting from lytic infection of oligodendrocytes by the papovavirus JC (JCV). PML has also been recognized as an AIDS-defining illness. The incidence of PML has increased since 1987 and it occurs in up to 4% of patients with AIDS. To date, there is no treatment available for PML and it usually results in death within 3-6 months of diagnosis. However, there are some reports of remission of PML after antiretroviral therapy. We report a 12-year-old child with hemophilia B and developing AIDS with the onset of PML. With highly active antiretroviral therapy, PML subsided with an increase of CD4 count from 10 to 300/microl in spite of about 1.0 X 10(4) human immunodeficiency virus (HIV)-1-RNA copies. He has survived more than 1 year without specific therapy against JCV. Highly active antiretroviral therapy appears to have improved his prognosis in HIV-associated PML.

Acquired Immunodeficiency Syndrome↗
PubMed1991

Progressive multifocal leukoencephalopathy in persons infected with human immunodeficiency virus, San Francisco, 1981-1989.

Progressive multifocal leukoencephalopathy (PML), a rare neurological disease, has been sporadically reported in persons infected with human immunodeficiency virus (HIV), the causative agent of acquired immune deficiency syndrome (AIDS). From January 1981 through February 1989, in San Francisco, we identified 94 HIV-infected persons with PML, of whom 48 (51%) were pathologically confirmed (as required for AIDS case reporting). These 48 patients were significantly older when diagnosed with AIDS (20% older than 50 years) than patients with AIDS without PML. The remaining 46 (49%) patients, diagnosed clinically and by neuroimaging, did not differ significantly from definitive patients in demographic or survival characteristics after PML diagnosis. We detected antibodies to JC virus, the causative agent of PML, in 9 of 14 (64%) AIDS-related patients with PML, and in 9 of 14 (64%) matched control subjects, suggesting that determination of JC virus antibody status before AIDS diagnosis does not reliably indicate which patients will contract PML. Our study shows that the proportion of patients with AIDS who contracted PML remained stable between 1981 and 1988, but increased in the first 2 months of 1989. Our findings further indicate that PML in HIV-infected patients may be underestimated by as much as 50%.

Adolescent↗