A leucoencefalopatia multifocal progressiva (LMP) é um distúrbio neurológico que danifica a mielina que cobre e protege os nervos na substância branca do cérebro. É causada pelo vírus JC (JCV). Aos 10 anos, a maioria das pessoas foi infectada com este vírus, mas ele raramente causa sintomas, a menos que o sistema imunológico fique gravemente enfraquecido. A doença ocorre, raramente, em pacientes transplantados de órgãos; pessoas em tratamento crônico com corticosteroides ou terapia imunossupressora; e indivíduos com câncer, como doença de Hodgkins, linfoma e sarcoidose. A LMP é mais comum entre indivíduos com síndrome da imunodeficiência adquirida (AIDS).
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A leucoencefalopatia multifocal progressiva (LMP) é um distúrbio neurológico que danifica a mielina que cobre e protege os nervos na substância branca do cérebro. É causada pelo vírus JC (JCV). Aos 10 anos, a maioria das pessoas foi infectada com este vírus, mas ele raramente causa sintomas, a menos que o sistema imunológico fique gravemente enfraquecido. A doença ocorre, raramente, em pacientes transplantados de órgãos; pessoas em tratamento crônico com corticosteroides ou terapia imunossupressora; e indivíduos com câncer, como doença de Hodgkins, linfoma e sarcoidose. A LMP é mais comum entre indivíduos com síndrome da imunodeficiência adquirida (AIDS).
Escala de raridade
<1/50kMuito rara
1/20kRara
1/10kPouco freq.
1/5kIncomum
1/2k
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Sinais e sintomas
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[A case of cerebellar-limited HIV-associated progressive multifocal leukoencephalopathy diagnosed with the aid of the shrimp sign].
A 43-year-old man presented with dizziness and slurred speech. A brain MRI with diffusion-weighted imaging and fluid-attenuated inversion recovery imaging revealed an abnormally high signal in the white matter of the left cerebellar hemisphere sparing the dentate nucleus, consistent with the characteristic "shrimp sign". Pathological findings from brain biopsy were non-specific, however, JC virus-DNA was detected in the cerebrospinal fluid and anti-human immunodeficiency virus (HIV) antibodies were positive in the serum, leading to a diagnosis of HIV-associated progressive multifocal leukoencephalopathy (PML) limited to the cerebellum. Antiretroviral therapy was initiated, resulting in a favorable clinical course. The shrimp sign is highly specific to cerebellar PML and may facilitate an early diagnosis.
Outcome Predictors in Progressive Multifocal Leukoencephalopathy Associated With Multiple Sclerosis Treatments: A Multicenter Cohort Study.
JC virus (JCV) reactivation causing progressive multifocal leukoencephalopathy (PML) is a complication in patients with multiple sclerosis (MS) treated with disease-modifying therapies (DMTs). Although natalizumab (NTZ) is most frequently involved, PML also occurs less commonly with sphingosine-1-phosphate receptor modulators (S1P-RM), dimethyl fumarate (DMF), and ocrelizumab. This study aimed to identify factors predicting worse outcomes, focusing on the influence of PML-immune reconstitution inflammatory syndrome (PML-IRIS), plasma exchange (PlEx), corticosteroids, and DMT reintroduction. This retrospective multicenter cohort study analyzed patients with MS who had JCV-associated pathology (PML or granule cell neuronopathy) from 42 centers (2009-2022). The primary outcome was disability at 12 months, measured by the modified Rankin Scale (mRS). Multivariable analyses identified predictors of poor outcomes, PML-IRIS development, and recurrent MS activity. Of 96 identified patients, 94 were analyzed. Most cases occurred under NTZ (77%), followed by S1P-RM (22%) and DMF (1%). Twelve-month survival was 91.5%, with a median mRS of 3 [IQR: 2-4]. Multivariable analysis showed that higher pre-PML disability (OR: 1.95 [95% CI 1.46-2.60], p < 0.001), elevated CSF JCV viral load (OR: 2.45 [95% CI 1.55-3.87], p < 0.001), and symptomatic presentation at onset (OR: 3.93 [95% CI 1.23-12.55], p = 0.021) were associated with worse outcomes. Conversely, PML-IRIS was associated with better outcomes (OR: 0.28 [95% CI 0.09-0.86], p = 0.025). PlEx and corticosteroid use had no negative effect. This study provides valuable insights into the management of iatrogenic PML in patients with MS. The findings may guide clinicians in making informed decisions, particularly regarding the use of PlEx, corticosteroids, and the management of PML-IRIS.
BK polyomavirus-associated progressive multifocal leukoencephalopathy following mogamulizumab therapy for erythrodermic mycosis fungoides.
BK polyomavirus (BKPyV) is a ubiquitous human pathogen that typically causes nephropathy and hemorrhagic cystitis in immunocompromised patients. Although BKPyV shares close genetic and structural similarity with JC polyomavirus (JCPyV), which is responsible for progressive multifocal leukoencephalopathy (PML), its neurotropic potential remains poorly characterized. Rare reports have suggested possible central nervous system (CNS) involvement under conditions of severe immune suppression. Here, we describe the first documented case of BKPyV-associated PML in a patient with erythrodermic mycosis fungoides treated with Mogamulizumab, a CCR4-targeting monoclonal antibody that profoundly alters immune surveillance. We describe a patient with erythrodermic mycosis fungoides and long-standing immunological frailty, who developed neurological symptoms during Mogamulizumab therapy. Brain MRI showed multifocal white matter lesions compatible with PML. BKPyV DNA was detected in plasma, urine, and cerebrospinal fluid (CSF), while JCPyV DNA was absent. Serological testing showed high anti-BKPyV and anti-JCPyV IgG levels in plasma, indicating prior exposure to both viruses, while antibodies were undetectable in CSF, consistent with lack of intrathecal synthesis. This compartmental dissociation between plasma and CSF, together with the detection of BKPyV DNA and the absence of JCPyV DNA in CSF, supports BKPyV as the etiological neurotropic agent responsible for leukoencephalopathy. Sequencing of the VP1 and NCCR regions revealed compartment-specific nucleotide and amino acid variants, including non-conservative substitutions in the CSF isolate, suggesting intra-host viral heterogeneity. Compartment-specific sequence variability of viral protein 1 (VP1) and structural rearrangements of the non-coding control region (NCCR), particularly the loss of the Q and R block in CSF-derived isolates, underscore intra-host heterogeneity of BKV and may contribute to its adaptation and neurotropic potential. This is the first documented case of BKPyV-associated PML in a Mogamulizumab-treated patient. These findings highlight intra-host heterogeneity at the protein level, possibly reflecting compartment-specific viral evolution, and underscore the need for vigilant BKPyV and JCPyV monitoring during Mogamulizumab treatment.
Practical Management of Progressive Multifocal Leukoencephalopathy and Immune Reconstitution Inflammatory Syndrome.
The aim of this narrative review was to provide treating physicians with practical guidance on the management of progressive multifocal leukoencephalopathy (PML), offering a rational, evidence-informed approach to diagnosis and treatment based on published literature and the authors' collective clinical experience. The management of PML requires precise characterization of disease status at presentation and throughout follow-up. We begin by outlining the clinical scenarios in which PML should be suspected, emphasizing that disease presentation is shaped by the strength and quality of the host's antiviral immune response. We then detail the evaluation of patients at the time of diagnosis and during follow-up, with particular attention to defining whether PML is presenting in its classic form, with inflammatory features, or in the context of immune reconstitution inflammatory syndrome. This assessment should incorporate prognostic markers to evaluate the potential for immune recovery and virologic control. We further summarize the current standard of care and identify clinical contexts in which experimental immunotherapeutic interventions to facilitate restoration of JC virus-specific immune functions may be considered, emphasizing the importance of individualized, risk-benefit-based decision making. Notably, such experimental immunotherapies are likely unnecessary and may even be harmful, once immune reconstitution has already been achieved and virologic control is evident. Finally, we address the management of inflammation in PML, underscoring that not all inflammatory presentations warrant corticosteroid therapy. The management of PML remains a significant clinical challenge. Standardizing assessment protocols across centers may enhance data quality and comparability and facilitate future collaborative and data-sharing efforts.
Disproportionality analysis of drug-associated progressive multifocal leukoencephalopathy using spontaneous reports: A 20-year signal detection study based on the FAERS database.
Progressive multifocal leukoencephalopathy (PML) is a rare, often fatal demyelinating disease caused by JC virus reactivation in immunocompromised patients. With the increasing use of immunosuppressants and biologics, PML reporting in non-HIV populations is rising. This study aimed to evaluate drug-associated PML reporting signals using real-world pharmacovigilance data. We analyzed FAERS database from 2004Q1 to 2024Q4. We identified PML reports via MedDRA Terms and manual validation. Four algorithms (ROR, PRR, BCPNN, MGPS) were jointly applied, with drugs showing signals across all four defined as high-risk. 7,244 PML reports involving 298 drugs were identified; 72 drugs showed consistent signals, predominantly immunomodulators (e.g., natalizumab, rituximab), antineoplastics, and biologics. High-risk indications included multiple sclerosis, lymphoma, autoimmune diseases, and organ transplantation. PML reporting increased substantially in non-HIV populations. Time-to -reporting varied widely (49-1343days). Over one-third of reports were associated with life-threatening outcomes or death. This analysis identified 72 drugs with consistent PML reporting signals. However, these findings represent statistical associations in spontaneous reports, not causal relationships or true incidence rates. Inherent limitations-including underreporting, incomplete medication histories, and lack of exposure denominators-require cautious interpretation. Prospective validation studies are essential to establish causality and quantify absolute risks.
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The Indian journal of radiology & imaging[A case of cerebellar-limited HIV-associated progressive multifocal leukoencephalopathy diagnosed with the aid of the shrimp sign].
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HaematologicaProgressive Multifocal Leukoencephalopathy in Patients with HIV-Case Series from Northeastern Romania.
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Neurology. Clinical practice[Progressive multifocal leukoencephalopathy in HIV-negative patients for general practitioners].
Revue medicale suisseHIV-associated neurological infections in a Brazilian tertiary care center: clinical-epidemiological features and predictors of in-hospital mortality.
Revista do Instituto de Medicina Tropical de Sao PauloDisproportionality analysis of drug-associated progressive multifocal leukoencephalopathy using spontaneous reports: A 20-year signal detection study based on the FAERS database.
PloS oneBiopsy-Confirmed Progressive Multifocal Leukoencephalopathy During Epcoritamab Therapy Despite Negative CSF JC Virus PCR: A Case Report.
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PloS oneInter-assay variability in anti-JC virus testing for natalizumab: A Spanish cohort.
Multiple sclerosis and related disordersBK and JC polyomavirus co-infection resulting in polyomavirus nephropathy and progressive multifocal leukoencephalopathy at the same time, a case report.
The Brazilian journal of infectious diseases : an official publication of the Brazilian Society of Infectious DiseasesVirus-Specific T Cells and Response to Checkpoint Inhibitors in Progressive Multifocal Leukoencephalopathy.
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Folia neuropathologicaA real-world safety analysis of infection-related adverse events associated with belimumab, rituximab, and TNF inhibitors using the FAERS database.
Scientific reportsPhenotypic spectrum, etiology and outcomes of infection-related movement disorders: An observational cohort study.
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American journal of kidney diseases : the official journal of the National Kidney FoundationProgressive multifocal leukoencephalopathy during 4 years of Palbociclib for advanced breast cancer with a history of follicular lymphoma patient.
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Journal of neurology, neurosurgery, and psychiatryThe microvascular endothelium of the blood-brain barrier is highly restrictive to JC Polyomavirus neuroinvasion.
Microbiology spectrumProgressive multifocal leukoencephalopathy in rheumatoid arthritis and biological therapies: a case report and review of the literature.
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Cerebellum (London, England)Prolonged survival in HIV-associated Progressive Multifocal Leukoencephalopathy treated with Pembrolizumab: a case series on treatment and long-term follow-up.
Journal of neurovirologyLymphoTEC: a Retrospective Real-World Study on Lymphocyte Reconstitution After Lymphopenia in Patients with Multiple Sclerosis Treated with Dimethyl Fumarate in France.
Advances in therapyPortable ultra-low-field MRI for progressive multifocal leukoencephalopathy: Case studies, sensitivity, and potential applications.
Journal of neurology[Progressive multifocal leukoencephalopathy developed 3 years after related HLA-haploidentical peripheral blood stem cell transplantation].
[Rinsho ketsueki] The Japanese journal of clinical hematologySintilimab for treating progressive multifocal leukoencephalopathy caused by human polyomavirus 2 virus infection following allogeneic hematopoietic cell transplantation: a case report.
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CureusEffectiveness of 6-week dosing of Natalizumab versus continued 4-week treatment for Multiple Sclerosis: An observational registry-based study.
Multiple sclerosis and related disordersJohn Cunningham virus as cause of progressive multifocal leukoencephalopathy.
Neurologia i neurochirurgia polskaAn MRI assessment of mechanisms underlying lesion growth and shrinkage in multiple sclerosis.
Annals of clinical and translational neurologyEvaluating Neural Network Performance in Predicting Disease Status and Tissue Source of JC Polyomavirus from Patient Isolates Based on the Hypervariable Region of the Viral Genome.
VirusesCerebral Microbleeds and Amyloid Pathology Estimates From the Amyloid Biomarker Study.
JAMA network openMixed nontuberculous mycobacteria in an immunocompromised patient with probable progressive multifocal leukoencephalopathy.
IJID regionsDiagnostic Insighhts Into JC Virus-Infected Glial Cells: Intraoperative Cytopathological Analysis of Dot-Shaped Inclusions Associated With PML-NBs.
Cytopathology : official journal of the British Society for Clinical CytologyPerformance of Ultrasensitive Polymerase Chain Reaction Testing for JC Polyomavirus in Cerebrospinal Fluid Compared with Pathological Diagnosis of Progressive Multifocal Leukoencephalopathy.
VirusesAnalysis of Immunological Memory for John Cunningham Virus in a Mexican Population of Patients with Multiple Sclerosis Under Treatment.
BiomedicinesDiagnosing progressive multifocal leukoencephalopathy: Positive predictive value of CSF JC virus quantitative PCR and importance of recognizing suggestive neuroimaging findings.
Journal of the neurological sciencesAnti-JCV antibody index seroconversion in Turkish multiple sclerosis patients treated with natalizumab.
Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical NeurophysiologyProgressive multifocal leukoencephalopathy, the role of the susceptibility-weighted imaging sequence in a case of immunosuppression of an initially unknown cause.
Encephalitis (Seoul, Korea)Antibody-Drug Conjugates: The Toxicities and Adverse Effects That Emergency Physicians Must Know.
Annals of emergency medicineRecent advances in discovery and functional analysis of the small proteins and microRNA expressed by polyomaviruses.
Virology"Pardon My Language": The Curious Case of a Bloody Sandwich and the John Cunningham Virus Reactivation in the Era of Immunomodulatory Drugs.
CureusAntigen-Specific T Cell Receptor Discovery for Treating Progressive Multifocal Leukoencephalopathy.
bioRxiv : the preprint server for biologyReal-World Effectiveness and Safety of Carotegrast Methyl in Japanese Patients with Moderately Active Ulcerative Colitis.
Inflammatory intestinal diseasesA Case of Progressive Multifocal Leukoencephalopathy Caused by Epcoritamab.
CureusProgressive multifocal leukoencephalopathy associated with sphingosine-1-phosphate receptor modulators: A large case series.
Multiple sclerosis and related disordersBrain biopsy and pathological diagnosis for drug-associated progressive multifocal leukoencephalopathy (PML) with inflammatory reactions.
Pathology internationalTransient biopsy-proven progressive multifocal leukoencephalopathy-immune reconstitution inflammatory syndrome (PML-IRIS) in an elderly woman without known immunodeficiency: a case report.
BMC neurologyElevation of serum neurofilament light-chain levels disclose possible occult progressive multifocal leukoencephalopathy and immune reconstitution syndrome in a patient receiving ozanimod: a case report.
Frontiers in immunologyProteome profiling of polyomavirus nuclear replication centers using iPOND.
Journal of virologyStroke Mimics: A Case of John Cunningham Virus-Induced Progressive Multifocal Leukoencephalopathy.
CureusCase report: Progressive multifocal leukoencephalopathy co-occurring with neurosarcoidosis: early brain biopsy and appropriate therapy for PML resulted in a favorable prognosis.
Frontiers in immunologyGPCR Inhibitors Have Antiviral Properties against JC Polyomavirus Infection.
VirusesIdeomotor Apraxia and "Milky Way" Sign in Progressive Multifocal Leukoencephalopathy.
NeurologyIdentification of JC polyomavirus in upper respiratory samples from Portuguese children.
HeliyonCombination of low-dose, long-term immunoglobulin and mirtazapine is effective in progressive multifocal leukoencephalopathy caused by JC virus infection.
BMC neurologyPharmacokinetics and Pharmacodynamics of Natalizumab 6-Week Dosing vs Continued 4-Week Dosing for Relapsing-Remitting Multiple Sclerosis.
Neurology(R) neuroimmunology & neuroinflammationDeciphering Proteomic Expression in Inflammatory Disorders: A Mass Spectrometry Exploration Comparing Infectious, Noninfectious, and Traumatic Brain Injuries in Human Cerebrospinal Fluid.
Neurotrauma reportsDirectly Isolated Allogeneic Virus-Specific T Cells in Progressive Multifocal Leukoencephalopathy.
JAMA neurologyProgressive Multifocal Leukoencephalopathy in a Patient With Cirrhosis and Hepatocellular Carcinoma.
The NeurohospitalistProgressive multifocal leukoencephalopathy in systemic lupus erythematosus treated with pembrolizumab.
BMJ case reportsHIV-Associated Neurocognitive Disorder and CNS Viral Escape: A Case Report.
Cureus[Progressive multifocal leukoencephalopathy in HIV-positive individuals].
Zhurnal nevrologii i psikhiatrii imeni S.S. KorsakovaProgressive Multifocal Leukoencephalopathy confined to the posterior fossa as the presenting manifestation of HIV in a paediatric patient.
IDCasesProgressive multifocal leukoencephalopathy after CAR-T cell therapy.
Bone marrow transplantationAdvances in virus-specific T-cell therapy for polyomavirus infections: A comprehensive review.
International journal of antimicrobial agentsEfficacy and Safety of Bendamustine-Rituximab as Frontline Therapy for Indolent Non-Hodgkin Lymphoma: A Real-World, Single-Center, Retrospective Study.
CureusA rare case of progressive multifocal leukoencephalopathy.
Radiology case reportsSafety of high efficacy therapies in older people with Multiple Sclerosis: A real-world evidence study.
Multiple sclerosis and related disordersNeurosarcoidosis as a rapidly progressive dementia associated with normal pressure hydrocephalus.
MedicinaPresentation and Outcome in S1P-RM and Natalizumab-Associated Progressive Multifocal Leukoencephalopathy: A Multicenter Cohort Study.
Neurology(R) neuroimmunology & neuroinflammationProgressive Multifocal Leukoencephalopathy in Myasthenia Gravis With Selective Hypogammaglobulinemia.
Journal of clinical neuromuscular diseaseUnveiling the Enigma: John Cunningham Virus-Associated Progressive Multifocal Leukoencephalopathy in an Immunocompetent Individual.
CureusUnusual demyelinating disease in a patient with HIV infection.
Journal of neurovirologyProgressive multifocal leukoencephalopathy in sarcoidosis successfully treated with pembrolizumab.
Journal of neurovirology[Case report and literature review of AIDS-related progressive multifocal leukoencephalopathy diagnosed in a psychiatric department].
Orvosi hetilap"One a penny, two a penny", I saw the hot cross bun sign".
Clinical imagingAdvances in diffuse glioma assessment: preoperative and postoperative applications of chemical exchange saturation transfer.
Frontiers in neuroscienceJC virus spread is potentiated by glial replication and demyelination-linked glial proliferation.
Brain : a journal of neurologyJCV granule cell neuronopathy: A rare case manifestation.
Clinical neuropathologyFrequent detection of IFN-gamma -producing memory effector and effector T cells in patients with progressive multifocal leukoencephalopathy.
Frontiers in immunologySafety and effectiveness of disease-modifying therapies after switching from natalizumab.
Multiple sclerosis (Houndmills, Basingstoke, England)Optimizing the post-CAR T monitoring period in recipients of axicabtagene ciloleucel, tisagenlecleucel, and lisocabtagene maraleucel.
Blood advancesAtypical Presentation of a Patient With Progressive Multifocal Leukoencephalopathy.
CureusOcrelizumab-induced organizing pneumonia in multiple sclerosis: case report and literature review.
Archive of clinical casesAssociações
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Referências e fontes
Bases de dados externas citadas neste artigo
Publicações científicas
Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.
- [A case of cerebellar-limited HIV-associated progressive multifocal leukoencephalopathy diagnosed with the aid of the shrimp sign].
- Outcome Predictors in Progressive Multifocal Leukoencephalopathy Associated With Multiple Sclerosis Treatments: A Multicenter Cohort Study.
- BK polyomavirus-associated progressive multifocal leukoencephalopathy following mogamulizumab therapy for erythrodermic mycosis fungoides.
- Practical Management of Progressive Multifocal Leukoencephalopathy and Immune Reconstitution Inflammatory Syndrome.
- Disproportionality analysis of drug-associated progressive multifocal leukoencephalopathy using spontaneous reports: A 20-year signal detection study based on the FAERS database.
- Progressive multifocal leukoencephalopathy - a diagnostic guide for the clinical neurologist.
- Pembrolizumab Experience in a Patient with Progressive Multifocal Leukoencephalopathy Following Multiple Myeloma Treatment: A Case Report and Literature Review.
- Progressive multifocal leukoencephalopathy associated with immune dysregulation.
- Threshold-Dependent Discordance Between STRATIFY JCV™ and IMMUNOWELL™ Assays in Relapsing Multiple Sclerosis.
- Progressive Multifocal Leukoencephalopathy: A Rare Cause of Altered Mentation.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:217260(Orphanet)
- MONDO:0016318(MONDO)
- GARD:7468(GARD (NIH))
- Busca completa no PubMed(PubMed)
- Artigo Wikipedia(Wikipedia)
- Q704930(Wikidata)
Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.
Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar
