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PubMed2005

AIDS-related progressive multifocal leukoencephalopathy.

A case of progressive multifocal leukoencephalopathy associated with human immunodeficiency virus infection with a fatal outcome is presented. The disease has not been reported from our region before. The patient presented initially with hemiparesis and non-enhancing lesion on computed tomography scan that was thought to be an infarct. After a delay of 4 months, the diagnosis was made by brain biopsy. This delay probably contributed to his unfortunate outcome. The case is described, and methods of diagnosis and treatment are discussed.

Acquired Immunodeficiency Syndrome↗
PubMed2005

AIDS-associated progressive multifocal leukoencephalopathy : current management strategies.

Progressive multifocal leukoencephalopathy (PML) is a rare, opportunistic infection of the CNS by the ubiquitous JC virus (JCV). PML is only seen in the context of severe and prolonged immunosuppression, a phenomenon now frequently encountered since the AIDS pandemic. PML is characterised by progressive lysis of oligodendrocytes with demyelination. A rapid clinical course ensues with focal neurological deficits and a median time to death of 3.5 months without treatment. Prior to highly active antiretroviral therapy (HAART), there was no effective therapy. Since the advent of HAART, the prognosis for PML has much improved; however, a significant number of patients appear unresponsive to antiretrovirals and some worsen because of the development of immune reconstitution disease. A better understanding of the biology of JCV and its interactions with host cells is leading to new anti-JCV-specific agents that await evaluation in randomised, controlled trials. Improved diagnostic tools and the possibility of immunotherapy and gene therapy are further advancing the field.

Acquired Immunodeficiency Syndrome↗
PubMed1981

Two cases of progressive multifocal leukoencephalopathy after renal transplantation.

Progressive multifocal leukoencephalopathy (PML) occurred in two patients after kidney transplantation. Less than 2 years after such a transplantation associated with immunosuppressive chemotherapy a 54-year-old male developed polyneuropathy then clinical diffuse alteration of the central nervous system. He died three months later with the suspicion of hypertensive encephalopathy due to progressive renal failure. A 45-year-old female had a kidney transplantation first rapidly complicated by Listeria monocytogenes meningoencephalitis. She was cured from this disease and had a satisfactory social rehabilitation during two years. Afterwards, she suffered various neurological troubles, including epilepsy, that were attributed to combined renal failure and developing hydrocephalus. One year after the onset of these neurological symptoms, the grafted kidney was removed and chemotherapy was discontinued. She died three months later. Both patients had typical PML with eosinophilic intranuclear inclusions in presumptive oligodendroglial cells. By electron microscopy, performed on formalin fixed brain tissue, round particles (40-50 nm) could be recognized in some glial cell nuclei. These two cases are confronted with the four published observations of PML following organ transplantation.

Astrocytes↗
PubMed2004

Progressive multifocal leukoencephalopathy in transplant recipients.

Progressive multifocal leukoencephalopathy (PML) is a demyelinating infection caused by the JC virus. It is an emerging disease in transplant recipients; however, it remains poorly defined. Twenty-four cases of PML reported in the literature in transplant recipients were reviewed. Of the 24 cases, nine occurred in renal, six in bone marrow, four in liver, three in heart and two in lung transplant recipients. Median time to onset was 17 months; 71% occurred within 24 months of transplantation. PML tended to occur later in the kidney recipients (P=0.04). Seventy-five percent had subacute presentation; hemiparesis (50%), apathy (46%) and confusion (38%) were the most frequently presented features. Treatment included reduction of immunosuppression and chemotherapy, mainly cidofovir. Death occurred within 2.5 months of the onset of symptoms in 17 patients (71%). PML is a unique entity that should be considered in any transplant recipient with neurological symptoms. The outcome is usually fatal, although regression has been reported.

Adult↗
PubMed2001

Detection of human herpesvirus 6 and JC virus in progressive multifocal leukoencephalopathy complicating follicular lymphoma.

Progressive multifocal leukoencephalopathy (PML), a demyelinating infectious disease caused by JC virus (JCV), occurs almost exclusively in immunocompromised patients usually with malignant diseases. We report here a Japanese female with follicular lymphoma who subsequently developed PML. In addition to JCV, human herpesvirus 6 (HHV-6) was detected in the affected brain lesions of the patient by polymerase chain reaction and by in situ hybridization. HHV-6, recognized as a neurotropic virus, is known to be reactivated during immunosuppression and can cause fatal complications such as encephalitis/encephalopathy. It is likely that impaired immunity associated with lymphoma and the additional immunosuppression following cytopenia-inducing chemotherapies predisposed the patient to reactivated HHV-6 infection. Although it remains to be clarified whether HHV-6 plays an important role as a co-agent with JCV in causing demyelination of the brain, our observation alerts physicians to the possible association of HHV-6 with the pathogenesis of PML.

Adult↗
PubMed2006

Review of progressive multifocal leukoencephalopathy and natalizumab.

BACKGROUND: Progressive multifocal leukoencephalopathy (PML), a destructive demyelinating infection which lytically infects oligodendrocytes, has occurred in patients treated with natalizumab. Magnetic resonance imaging (MRI) scan imaging of the brain gives clues to diagnosis but is nonspecific in distinguishing multiple sclerosis from PML. Spinal fluid detection of JC virus is specific but incompletely sensitive. Associated immunosuppression is typically of the cell-mediated type but can be poorly defined on clinical grounds. REVIEW SUMMARY: It is apparent that natalizumab is a predisposing factor for developing PML from the 3 cases of natalizumab-treated patients. There is no reliable presymptomatic way to detect PML or JC virus infection of the brain by virologic or imaging surveillance techniques. One patient with multiple sclerosis and natalizumab treatment has survived, indicating that withdrawal of antibody, possibly in combination with antiviral therapy, may permit survival. However, immune reconstitution disease is a risk after immune restoration and withdrawal of natalizumab. PML deficits would be expected to be permanent. The estimate of incidence of PML in natalizumab-treated patients is 1 per 1000. The duration of natalizumab treatment may be an independent risk factor for development of PML. CONCLUSION: PML, a usually fatal neurologic infection, should be considered as a risk factor when using natalizumab. The treatment of multiple sclerosis patients with natalizumab is a matter of informed risk, individualized for each multiple sclerosis patient.

Antibodies, Monoclonal↗
PubMed2005

Atypical radiological presentation of progressive multifocal leukoencephalopathy following liver transplantation.

Progressive multifocal leukoencephalopathy (PML), a demyelinating disease of the brain caused by JC virus (JCV), occurs following transplantation and other conditions associated with immunosuppression. On magnetic resonance imaging (MRI), PML lesions typically appear as hyperintense signal on T2-weighted and FLAIR images located in the subcortical white matter, which are devoid of contrast enhancement or mass effect. The prognosis is poor, but unusual inflammatory forms of PML characterized by contrast enhancement have been associated with a cellular immune response against JCV and a better prognosis. The authors report an atypical presentation of PML with contrast-enhancing lesions and mass effect on the MRI in a liver transplant recipient,who had a progressive course and fatal outcome.

Adult↗
PubMed2003

Inflammatory reaction in progressive multifocal leukoencephalopathy: harmful or beneficial?

Progressive multifocal leukoencephalopathy (PML) occurs in patients with profound immunosuppression. Although lesions are usually devoid of lymphoplasmocytic infiltrates, inflammatory forms of PML have been described, in both human immunodeficiency virus (HIV)-seropositive (HIV+) and -seronegative (HIV-) patients. In addition, PML has been shown to develop in HIV+ patients shortly after introduction of highly active antiretroviral therapy (HAART), despite a recovery of the immune system. Therefore, one could postulate that PML might arise in the context of an immune reconstitution syndrome. To examine the clinical and neuroradiological characteristics of inflammatory forms of PML, the authors performed a retrospective analysis of the patients seen at their institution since 1996 as well as a review of the literature. Of 39 HIV+ and HIV- PML patients, 5 (13%) presented with an inflammatory form of this disease. Two HIV+ patients developed PML soon after the onset of HAART, concomitant to immune recovery, as demonstrated by a decrease of HIV viral load (VL) and an increase of CD4+ T-cell count. Three patients (2 HIV+ and 1 HIV-) had signs of inflammation in the central nervous system (CNS) characterized by contrast-enhancing lesions on neuroimaging studies, and/or inflammatory infiltrates on brain biopsy. The presence of JC virus-specific cytotoxic T lymphocytes was demonstrated in 4/4 patients tested and the outcome was favorable in 3 of them. In agreement with previously published case reports, the data indicate that inflammatory reactions in PML are not infrequent, and that they are generally associated with a favorable prognosis. Therefore clinicians should not disregard the diagnosis of PML in presence of contrast-enhancing brain lesions, and should use caution before treating these immunosuppressed individuals with steroids.

Adult↗
PubMed2004

Polyoma nephropathy and progressive multifocal leukoencephalopathy in a renal transplant recipient.

Progressive multifocal leukoencephalopathy is a progressive and ultimately fatal white-matter disease of the brain that is associated with polyomavirus infection. It is uncommon in the general population, and even in the immunosuppressed patient, who is inherently at greatest risk for active infection with the virus, it is rare. The causative agent in progressive multifocal leukoencephalopathy, JC virus, has become increasingly important in recent years as its role in nephropathy in the renal transplant recipient has become better understood. We present a young renal transplant patient who developed nephropathy with renal biopsy changes consistent with polyomavirus lesions and then developed mental status changes and was diagnosed with progressive multifocal leukoencephalopathy.

Adolescent↗
PubMed1976

Progressive multifocal leukoencephalopathy in renal transplant recipients.

Progressive multifocal leukoencephalopathy, a slowly progressive papovavirus infection of the CNS, has been reported in more than 110 patients to date, over half of whom have had either leukemia or lymphoma. Our patient is the fourth case of PML occurring after long-term immunosuppression for renal transplantation. We believe that PML is emerging as a potentially serious problem in renal transplant recipients.

Adult↗
PubMed1986

Progressive multifocal leukoencephalopathy and magnetic resonance imaging.

Progressive multifocal leukoencephalopathy developed in a homosexual man with underlying Hodgkin's disease. Computed tomography and magnetic resonance imaging of the brain demonstrated multiple lesions, more in gray than white matter. Brain biopsy established the diagnosis of progressive multifocal leukoencephalopathy. Magnetic resonance imaging was found useful for detecting brain lesions and for localizing an accessible lesion for biopsy.

Brain↗
PubMed2005

Diffusion tensor imaging in progressive multifocal leukoencephalopathy: early predictor for demyelination?

Progressive multifocal leukoencephalopathy is a severe demyelinating disease of the central nervous system caused by a JC papovavirus infection. Prognosis is poor with fatal outcome within 1 year in 90% of patients. Our case report shows that diffusion tensor imaging can give early and specific information about disease status and extent in a 15-year-old girl with progressive multifocal leukoencephalopathy associated with congenital human immunodeficiency virus (HIV) infection.

Adolescent↗
PubMed1982

Primary cerebellopontine progressive multifocal leukoencephalopathy diagnosed premortem by cerebellar biopsy.

A subacute progressive cerebellar brainstem syndrome developed in a patient with systemic lupus erythematosus in remission. Cerebellar biopsy documented the diagnosis of progressive multifocal leukoencephalopathy (PML). Data from this patient and 10 others in the literature emphasize the need to consider this diagnosis when ataxia develops in any patient with underlying malignancy, chronic infection, or other disease that involves immunological incompetence. Although the ataxic form of PML is not of nosological relevance, early diagnosis may eventually have therapeutic importance.

Biopsy↗
PubMed1999

Quantification of JC virus DNA in the cerebrospinal fluid of patients with human immunodeficiency virus-associated progressive multifocal leukoencephalopathy--a longitudinal study.

In progressive multifocal leukoencephalopathy (PML) the JC virus (JCV) load in the cerebrospinal fluid (CSF) is discussed as a parameter for disease progression. To investigate the evolution of viral shedding into the CSF, the JCV DNA concentration was quantified by competitive polymerase chain reaction (PCR) in multiple CSF samples from prior to and during an unsuccessful intrathecal salvage therapy in 2 human immunodeficiency virus-infected patients with biopsy-proven PML. With continuous clinical progression the virus load varied considerably intra- and interindividually, ranging from nondetectable to 1.2x108 genome equivalents/10 microliter CSF. Whereas an overall increase during progressive disease was confirmed, the virus burden was either constant or fluctuated irregularly during the intermediate stage of disease. This shows a variability of viral shedding during active disease that must be taken into account when the JCV load is measured by quantitative PCR for both the diagnosis of PML and monitoring under investigational treatment.

AIDS-Related Opportunistic Infections↗
PubMed1979

Cerebellar form of progressive multifocal leukoencephalopathy (PML).

A case of progressive multifocal leukoencephalopathy (PML) with selective involvement of cerebellum and brain stem is compared with nine other published cases. Recognition of PML presenting with ataxia before cerebral manifestations is stressed especially, since promising results with some antiviral drugs have been claimed.

Ataxia↗
PubMed2003

Progressive multifocal leukoencephalopathy complicating Waldenström's macroglobulinaemia.

Progressive multifocal leukoencephalopathy (PML) is a rare demyelinating neurological disorder caused by JC virus. Immunocompromised patients such as those with chronic lymphocytic leukemia, AIDS and following organ transplantation are at increased risk. We report a patient with PML complicating longstanding Waldenström's macroglobulinaemia. Although PML is a rare occurrence, the newer highly immunosuppressive treatment approaches for patients with lymphoproliferative disorders necessitate a high index of clinical suspicion. The diagnosis should be considered in patients with compatible clinical features who have received long-term immunosuppressive treatments recognized to impair cellular immunity.

Humans↗
PubMed1994

In situ polymerase chain reaction demonstration of JC virus in progressive multifocal leukoencephalopathy, including an index case.

Progressive multifocal leukoencephalopathy (PML) is a demyelinating disease that is caused by JC papovavirus. The virus can be demonstrated in brains with PML using a variety of techniques. In situ polymerase chain reaction (PCR) is a new method that combines the sensitivity of PCR with the histological localization of in situ hybridization. We here show that in situ PCR can detect JC virus in archival tissue sections of 3 cases of PML, including 1 of the original cases described in 1958 and 1 case that was fixed in formalin for 7 weeks. JC virus DNA was amplified directly on tissue and demonstrated in the characteristic enlarged oligodendrocytes and bizarre astrocytes. These data illustrate the utility of the in situ PCR technique in detecting JC virus in archival sections, including those fixed for prolonged periods, and provide a historical footnote to the original report of PML.

Adult↗