scholarly journals Progressive multifocal leukoencephalopathy: new concepts

2013 ◽  
Vol 71 (9B) ◽  
pp. 699-702 ◽  
Author(s):  
Marco A. Lima
Keyword(s):  
Progressive Multifocal Leukoencephalopathy ◽  
Demyelinating Disease ◽  
Jc Virus ◽  
Treatment Strategies ◽  
Antiretroviral Drugs ◽  
Transplant Recipients ◽  
New Concepts ◽  
Immunosuppressive Medications ◽  
New Treatment ◽  
The Impact

Progressive multifocal leukoencephalopathy (PML) is a demyelinating disease of the CNS caused by reactivation of JC virus (JCV) in a setting of cellular immunosuppression. Originally, PML was observed in patients with advanced HIV infection, lymphoproliferative disorders and transplant recipients. However, the widespread use of HIV antiretroviral drugs and the new selective immunomodulatory and immunosuppressive medications, such as Rituximab and Natalizumab, has recently modified the epidemiology, clinical presentation and prognosis of PML. Herein, we discuss the new concepts on PML, emphasizing the recent modification in the epidemiology; the impact of new immunomodulatory treatments in the disease, PML-IRIS (Immune reconstitution inflammatory síndrome), new treatment strategies and other JCV related CNS diseases.

Progressive multifocal leukoencephalopathy after a kidney transplantation: based on literature review and own clinical case

2021 ◽  
Vol 2021 (2b) ◽  
pp. 113-116
Author(s):  
T.A. Litovchenko ◽  
◽  
O.V. Vostrotin ◽  
O.L. Tondiy ◽  
V.V. Lebedynets ◽  
...  
Keyword(s):  
Kidney Transplantation ◽  
Literature Review ◽  
Progressive Multifocal Leukoencephalopathy ◽  
Immunosuppressive Therapy ◽  
Clinical Case ◽  
Demyelinating Disease ◽  
Jc Virus ◽  
Transplant Recipients ◽  
History Of ◽  
The Central Nervous System

This article discusses the development of progressive multifocal leukoencephalopathy after a kidney transplantation on the background of immunosuppressive therap. It’s the example of a clinical case. Based on the literature review, it is known that progressive multifocal leukoencephalopathy is a progressive demyelinating disease of the central nervous system, which is caused by reactivation of the latent JC virus; such reactivation usually occurs in immunodeficient conditions. In the clinical case, the appearance of this disease was demonstrated and its severe and potentially fatal multifocal lesion of the white matter of the brain was proved. The disease was developed on the background of immunosuppressive therapy after a history of kidney transplantation. The understanding of the clinical course of progressive multifocal leukoencephalopathy, the absence of specific neurological manifestations and course were expanded. It is noted that MRI results in addition to the clinical picture and anamnestic data help to establish the diagnosis of PML and make a differential diagnosis. Progressive multifocal leukoencephalopathy in transplant recipients receiving immunosuppressive therapy have an unfavorable prognosis.

scholarly journals Progressive multifocal leukoencephalopathy in an HIV patient was diagnosed by 3 times lumbar punctures and 2 times brain biopsies

2020 ◽  
Vol 26 (6) ◽  
pp. 952-956
Author(s):  
Mengyan Wang ◽  
Zhongdong Zhang ◽  
Jinchuan Shi ◽  
Hong Liu ◽  
Binhai Zhang ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Progressive Multifocal Leukoencephalopathy ◽  
Demyelinating Disease ◽  
Jc Virus ◽  
Clinical Manifestations ◽  
Brain Biopsy ◽  
Hiv Positive ◽  
Negative Results ◽  
The Central Nervous System ◽  
Rule Out

AbstractProgressive multifocal leukoencephalopathy (PML) is a rare demyelinating disease of the central nervous system caused by JC virus (JCV) and is difficult to diagnose. We report on a male HIV-positive patient with PML finally diagnosed by 3 times lumbar punctures and 2 times brain biopsies. Negative results of JCV-PCR in cerebrospinal fluid (CSF) do not rule out the diagnosis of PML when clinical manifestations and neuroimaging features suspected PML. It is necessary to obtain new CSF and make repeat tests and even perform brain biopsy.

scholarly journals Association between disease-modifying therapies for multiple sclerosis and healthcare utilisation on a population level: a retrospective cohort study

BMJ Open ◽  
2019 ◽  
Vol 9 (11) ◽  
pp. e033599 ◽  
Author(s):  
Lina Al-Sakran ◽  
Ruth Ann Marrie ◽  
David Blackburn ◽  
Katherine Knox ◽  
Charity Evans
Keyword(s):  
Multiple Sclerosis ◽  
Retrospective Cohort ◽  
Negative Binomial ◽  
Demyelinating Disease ◽  
Treatment Strategies ◽  
Cost Benefit ◽  
Healthcare Utilisation ◽  
Population Level ◽  
Disease Modifying ◽  
The Impact

ObjectiveDisease-modifying therapy (DMT) use in multiple sclerosis (MS) has increased significantly. However, the impact of DMTs on healthcare use is limited and conflicting, and rarely examined at a population level. This study examined the association between DMTs and healthcare utilisation at the population level.DesignRetrospective cohort.SettingHealth administrative data from Saskatchewan, Canada (1997–2016).ParticipantsTo test for associations at the population level, we identified two cohorts. The general population cohort included all Saskatchewan residents ≥18 years who were drug plan beneficiaries. The MS cohort included individuals ≥18 years, identified using a validated definition (≥3 hospital, physician or drug claims for MS).Main outcome measures and methodsTo test for an association between the total number of DMT dispensations per year and the total number of hospitalisations we used negative binomial regression fitted with generalised estimating equations (GEE); only hospitalisations that occurred after the date of MS diagnosis (date of first claim for MS or demyelinating disease) were extracted. To test for an association between the number of DMT dispensations and physician claims, negative binomial distributions with GEE were fit as above. Results were reported as rate ratios (RR), with 95% CIs, and calculated for every 1000 DMT dispensations.ResultsThe number of DMT dispensations was associated with a decreased risk for all-cause (RR=0.994; 95% CI 0.992 to 0.996) and MS-specific (RR=0.909; 95% CI 0.880 to 0.938) hospitalisations. The number of DMT dispensations was not associated with the number of all-cause (RR=1.006; 95% CI 0.990 to 1.022) or MS-specific (RR=0.962; 95% CI 0.910 to 1.016) physician claims.ConclusionIncreased DMT use in Saskatchewan was associated with a reduction in hospitalisations, but did not impact the number of physician services used. Additional research on cost-benefit and differing treatment strategies would provide further insight into the true impact of DMTs on healthcare utilisation at a population level.

scholarly journals Progress in the Application of Drugs for the Treatment of Multiple Sclerosis

2021 ◽  
Vol 12 ◽  
Author(s):  
Weipeng Wei ◽  
Denglei Ma ◽  
Lin Li ◽  
Lan Zhang
Keyword(s):  
Multiple Sclerosis ◽  
Demyelinating Disease ◽  
Treatment Strategies ◽  
New Drugs ◽  
Focal Lesion ◽  
Disease Modifying Drugs ◽  
Physical Disorders ◽  
The Central Nervous System ◽  
Control Disease ◽  
New Treatment

Multiple sclerosis (MS) is an autoimmune and chronic inflammatory demyelinating disease of the central nervous system (CNS), which gives rise to focal lesion in CNS and cause physical disorders. Although environmental factors and susceptibility genes are reported to play a role in the pathogenesis of MS, its etiology still remains unclear. At present, there is no complete cure, but there are drugs that decelerate the progression of MS. Traditional therapies are disease-modifying drugs that control disease severity. MS drugs that are currently marketed mainly aim at the immune system; however, increasing attention is being paid to the development of new treatment strategies targeting the CNS. Further, the number of neuroprotective drugs is presently undergoing clinical trials and may prove useful for the improvement of neuronal function and survival. In this review, we have summarized the recent application of drugs used in MS treatment, mainly introducing new drugs with immunomodulatory, neuroprotective, or regenerative properties and their possible treatment strategies for MS. Additionally, we have presented Food and Drug Administration-approved MS treatment drugs and their administration methods, mechanisms of action, safety, and effectiveness, thereby evaluating their treatment efficacy.

scholarly journals Progressive multifocal leukoencephalopathy in human immunodeficiency virus type 1-infected patients: absence of correlation between JC virus neurovirulence and polymorphisms in the transcriptional control region and the major capsid protein loci

2001 ◽  
Vol 82 (4) ◽  
pp. 899-907 ◽  
Author(s):  
Monica Sala ◽  
Jean-Pierre Vartanian ◽  
Pascale Kousignian ◽  
Jean-François Delfraissy ◽  
Yassine Taoufik ◽  
...  
Keyword(s):  
Progressive Multifocal Leukoencephalopathy ◽  
Capsid Protein ◽  
Control Region ◽  
Transcriptional Control ◽  
Major Capsid Protein ◽  
Demyelinating Disease ◽  
Jc Virus ◽  
Capsid Protein Gene ◽  
Transcriptional Control Region ◽  
Genomic Marker

Progressive multifocal leukoencephalopathy (PML) is a rapidly fatal demyelinating disease of the central nervous system related to JC polyomavirus (JCV) replication in oligodendrocytes. PML usually occurs in immunocompromised individuals, especially in the setting of AIDS. Administration of highly active anti-retroviral therapy (HAART) may improve survival prognosis in some, but not all, patients with AIDS-related PML. This observation might be explained by the outgrowth of some JCV variants of increased fitness. To evaluate this hypothesis, two subgroups of five patients with AIDS-related PML, started on HAART after PML diagnosis, were analysed. The non-responder (NR) patients died rapidly despite HAART, while responders (R) had a positive outcome and were still alive. JCV DNA was extracted from cerebrospinal fluid biopsies and two regions of the genome were analysed, the transcriptional control region (TCR) and the major capsid protein gene (VP1). Both regions show different degrees of polymorphism and are recognized as evolving independently. Sequence analysis demonstrated that (i) extensive TCR rearrangements were present in both subgroups of patients, (ii) VP1 sequence polymorphisms could be identified in the BC loop, suggesting the absence of immune selection, and (iii) no genomic marker for JCV specific neurovirulence could be identified in the TCR and VP1 loci.

scholarly journals A Review on JC Virus Infection in Kidney Transplant Recipients

2013 ◽  
Vol 2013 ◽  
pp. 1-7 ◽  
Author(s):  
Serena Delbue ◽  
Mariano Ferraresso ◽  
Luciana Ghio ◽  
Camilla Carloni ◽  
Silvia Carluccio ◽  
...  
Keyword(s):  
Renal Transplantation ◽  
Demyelinating Disease ◽  
Jc Virus ◽  
Graft Function ◽  
Kidney Tissue ◽  
Bk Virus ◽  
Pathological Features ◽  
Transplant Recipients ◽  
Kidney Transplant Recipients ◽  
Dna Virus

The polyomavirus (PyV), JC virus (JCV), is a small nonenveloped DNA virus that asymptomatically infects about 80% of healthy adults and establishes latency in the kidney tissue. In case of immunodeficient hosts, JCV can lytically infect the oligodendrocytes, causing a fatal demyelinating disease, known as progressive multifocal leukoencephalopathy (PML). Although the reactivation of another human PyV, BK virus (BKV), is relatively common and its association with the polyomavirus associated nephropathy (PyVAN) following renal transplantation is proven, JCV replication and its impact on graft function and survival are less well studied. Here we describe the biology of JCV and its pathological features and we review the literature regarding the JCV infection analyzed in the setting of transplantations.

scholarly journals Refractory T-Cell Anergy and Rapidly Fatal Progressive Multifocal Leukoencephalopathy After Prolonged CTLA4 Therapy

2017 ◽  
Vol 4 (2) ◽  
Author(s):  
Manon Dekeyser ◽  
Marie-Ghislaine de Goër de Herve ◽  
Houria Hendel-Chavez ◽  
Céline Labeyrie ◽  
David Adams ◽  
...  
Keyword(s):  
T Cell ◽  
Progressive Multifocal Leukoencephalopathy ◽  
Demyelinating Disease ◽  
Jc Virus ◽  
Human Polyomavirus ◽  
Therapeutic Approaches ◽  
Cell Responses ◽  
T Cell Anergy ◽  
Immunosuppressed Patients ◽  
Cell Anergy

Abstract Progressive multifocal leukoencephalopathy (PML) is a deadly demyelinating disease due to central nervous system replication of the human polyomavirus JC virus (JCV) in immunosuppressed patients. The only effective therapeutic approach is to restore anti-JCV T-cell responses. In this study, we describe a case of rapidly fatal PML with JCV T-cell anergy in a renal transplant patient treated with CTLA4-Ig (belatacept, a CD28-B7 costimulation blocker and T-cell anergy inducer). T-cell anergy could not be reversed despite several therapeutic approaches. Progressive multifocal leukoencephalopathy secondary to biotherapy-induced T-cell anergy may thus represent a subset of PML with major resistance to anti-JCV immune recovery.

scholarly journals JC Virus Granule Cell Neuronopathy as AIDS-Presenting Illness

2018 ◽  
Vol 45 (4) ◽  
pp. 466-469 ◽  
Author(s):  
Simon Grandjean Lapierre ◽  
Xin Dang ◽  
Danielle Gilbert ◽  
Sylvie Lauzier ◽  
Igor J. Koralnik ◽  
...  
Keyword(s):  
Human Immunodeficiency Virus ◽  
Virus Infection ◽  
Human Immunodeficiency Virus Infection ◽  
Progressive Multifocal Leukoencephalopathy ◽  
Granule Cell ◽  
Demyelinating Disease ◽  
Jc Virus ◽  
Clinical Manifestations ◽  
Immunodeficiency Virus ◽  
Granule Cell Neuronopathy

AbstractJC virus is the etiological agent of progressive multifocal leukoencephalopathy, a white matter demyelinating disease that mostly affects immunocompromised patients. JC virus can also infect neurons and meningeal cells and cause encephalitis, meningitis and granule cell neuronopathy. We report a patient with JC virus granule cell neuronopathy, without concomitant progressive multifocal leukoencephalopathy, presenting as inaugural acquired immune deficiency syndrome-related illness. This patient’s human immunodeficiency virus infection remained undiagnosed for several months after neurological symptoms onset. We review JC virus pathophysiology, clinical manifestations, treatment and prognosis, and emphasize the importance of considering human immunodeficiency virus infection and related opportunistic infections in the differential diagnosis of new-onset isolated cerebellar disease.

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