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Progressive multifocal leukoencephalopathy

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https://www.readbyqxmd.com/read/29231623/progressive-multifocal-leukoencephalopathy
#1
Karl Bjørnar Alstadhaug, Kjell-Morten Myhr, Christine Hanssen Rinaldo
No abstract text is available yet for this article.
December 12, 2017: Tidsskrift for Den Norske Lægeforening: Tidsskrift for Praktisk Medicin, Ny Række
https://www.readbyqxmd.com/read/29230221/editorial-immune-control-of-jc-virus-infection-and-immune-failure-during-progressive-multifocal-leukoencephalopathy
#2
EDITORIAL
Yassine Taoufik, Marie-Ghislaine de Goër de Herve
No abstract text is available yet for this article.
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/29221753/pura-the-gene-encoding-pur-alpha-member-of-an-ancient-nucleic-acid-binding-protein-family-with-mammalian-neurological-functions
#3
REVIEW
Dianne C Daniel, Edward M Johnson
The PURA gene encodes Pur-alpha, a 322 amino acid protein with repeated nucleic acid binding domains that are highly conserved from bacteria through humans. PUR genes with a single copy of this domain have been detected so far in spirochetes and bacteroides. Lower eukaryotes possess one copy of the PUR gene, whereas chordates possess 1-4 PUR family members. Human PUR genes encode Pur-alpha (Pura), Pur-beta (Purb) and two forms of Pur-gamma (Purg). Pur-alpha is a protein that binds specific DNA and RNA sequence elements...
December 5, 2017: Gene
https://www.readbyqxmd.com/read/29200254/-lack-of-knowledge-is-no-excuse-hiv-positive-patient-with-progressive-multifocal-leukoencephalopathy-case-report-from-kenya
#4
(no author information available yet)
Progressive multifocal leukoencephalopathy (PML) is a severe demyelinating disease that affects the central nervous system, which has high morbidity and mortality and no effective, targeted therapies are available. According to the data from developing countries, it affects about 3 to 5% patients who are HIV positive. We present a case of a 27-year-old patient, who got infected with the HIV virus from his mother. The patient had poor compliance to the therapy since its initiation. Due to poor compliance and immunological and virological failure of the first line ARVs, the patient developed PML...
November 4, 2017: Neuro Endocrinology Letters
https://www.readbyqxmd.com/read/29159704/neuroimmune-regulation-of-jc-virus-by-intracellular-and-extracellular-agnoprotein
#5
Michael Craigie, Stephanie Cicalese, Ilker Kudret Sariyer
JC virus (JCV) is a human polyomavirus and the etiologic agent of the demyelinating disease progressive multifocal leukoencephalopathy (PML). PML is observed in patients with underlying immunocompromising conditions, suggesting that neuro-immune interactions between peripheral immune cells and neuro-glia play an important role in controlling viral reactivation in the brain. There is little known about the immunobiology of JCV reactivation in glial cells and the role of immune, glial, and viral players in this regulation...
November 20, 2017: Journal of Neuroimmune Pharmacology: the Official Journal of the Society on NeuroImmune Pharmacology
https://www.readbyqxmd.com/read/29152583/inhibition-of-retrograde-transport-limits-polyomavirus-infection-in-vivo
#6
Saumya Maru, Ge Jin, Dhimant Desai, Shantu Amin, Shwetank, Matthew D Lauver, Aron E Lukacher
Polyomaviruses (PyVs) silently infect most humans, but they can cause life-threatening diseases in immunocompromised individuals. The JC polyomavirus (JCPyV) induces progressive multifocal leukoencephalopathy, a severe demyelinating disease in multiple sclerosis patients receiving immunomodulatory therapy, and BK polyomavirus (BKPyV)-associated nephropathy is a major cause of kidney allograft failure. No effective anti-PyV agents are available. Several compounds have been reported to possess anti-PyV activity in vitro, but none have shown efficacy in clinical trials...
November 2017: MSphere
https://www.readbyqxmd.com/read/29146954/multiple-sclerosis-progressive-multifocal-leukoencephalopathy-risk-stratification
#7
Kalliopi Pitarokoili, Ralf Gold
No abstract text is available yet for this article.
December 2017: Nature Reviews. Neurology
https://www.readbyqxmd.com/read/29143589/biomarkers-in-the-evolution-of-multiple-sclerosis
#8
Thomas Berger
Nonimaging biomarkers can be applied in differential diagnosis, evaluation of disease progression and therapy monitoring of multiple sclerosis (MS). Presence of oligoclonal IgG bands in cerebrospinal fluid is a diagnostic element and a negative predictor of MS evolution. AQP4 antibodies are pathogenic and diagnostic for neuromyelitis optica spectrum disorder. Antibodies to myelin oligodendrocyte glycoprotein develop in about 50% of predominantly pediatric patients with acute disseminated encephalomyelitis, but their possible role in pathogenesis is unknown...
November 2017: Neurodegenerative Disease Management
https://www.readbyqxmd.com/read/29142146/inflammatory-natalizumab-associated-pml-baseline-characteristics-lesion-evolution-and-relation-with-pml-iris
#9
Mike P Wattjes, Martijn T Wijburg, Jeroen van Eijk, Stephan Frequin, Bernard M J Uitdehaag, Frederik Barkhof, Clemens Warnke, Joep Killestein
BACKGROUND AND OBJECTIVE: Natalizumab-associated progressive multifocal leukoencephalopathy (NTZ-PML) patients may show imaging signs suggestive of inflammation at diagnosis ('inflammatory PML'), reminiscent of PML-immune reconstitution inflammatory syndrome (PML-IRIS). We investigated the imaging characteristics of inflammatory NTZ-PML lesions and PML-IRIS to determine differentiating and overlapping features. METHODS: We scored the presence, localisation and pattern of imaging characteristics of inflammation on brain MRI scans of inflammatory NTZ-PML patients...
November 15, 2017: Journal of Neurology, Neurosurgery, and Psychiatry
https://www.readbyqxmd.com/read/29142140/imaging-and-csf-analyses-effectively-distinguish-cjd-from-its-mimics
#10
Peter Rudge, Harpreet Hyare, Alison Green, John Collinge, Simon Mead
OBJECTIVE: To review clinical and investigation findings in patients referred to a specialist prion clinic who were suspected to have sporadic Creutzfeldt-Jakob disease (sCJD) and yet were found to have an alternative final diagnosis. METHODS: Review the clinical findings and investigations in 214 patients enrolled into the UK National Prion Monitoring Cohort Study between October 2008 and November 2015 who had postmortem confirmed sCJD and compare these features with 50 patients referred over the same period who had an alternative final diagnosis (CJD mimics)...
November 15, 2017: Journal of Neurology, Neurosurgery, and Psychiatry
https://www.readbyqxmd.com/read/29139004/progressive-multifocal-leukoencephalopathy-in-the-absence-of-immunosuppression
#11
Benjamin E Zucker, Sybil R L Stacpoole
A 69-year-old woman presented with a cortical hand syndrome progressing over several weeks. MRI brain showed characteristic appearances of progressive multifocal leukoencephalopathy (PML), confirmed by detection of the JC virus in CSF, despite the absence of any evidence of immunosuppression. Treatment with mirtazapine, mefloquine and cidofovir did not affect the progression of the disease, which was fatal within 7 months of presentation. This report adds to the small case literature that suggests that PML can occur in immunocompetent people, albeit extremely rarely...
November 14, 2017: Journal of Neurovirology
https://www.readbyqxmd.com/read/29132903/progressive-multifocal-leukoencephalopathy-in-patients-receiving-rituximab-and-cyclophosphamide-after-haplo-identical-t-cell-replete-transplantation-and-review-of-the-literature
#12
REVIEW
R Salem, R Massoud, S S Kanj, M Hamdan, R Salman, A Bazarbachi, J El-Cheikh
John Cunningham virus (JCV) reactivation, occurring mainly in immunocompromised patients, leads to progressive multifocal leukoencephalopathy, an uncommon but lethal disease. JCV reactivation after T-cell replete haploidentical stem cell transplantation, in the pre-cyclophosphamide era, is poorly represented in the literature. We therefore describe two cases of acute myeloid leukemia who developed JCV reactivation after receiving cyclophosphamide and rituximab post haploidentical stem cell transplantation, and review the literature, aiming to a better understanding of the disease course and its risk factors...
November 2017: Current Research in Translational Medicine
https://www.readbyqxmd.com/read/29120025/prevention-and-therapy-of-jc-polyomavirus-mediated-progressive-multifocal-leukoencephalopathy-a-realistic-possibility
#13
Ivan Jelcic, Benoit Combaluzier, Ilijas Jelcic, Mireia Sospedra, Jan Grimm, Roland Martin
Progressive multifocal leukoencephalopathy (PML) is a serious opportunistic infection of the brain caused by the JC polyomavirus (JCPyV). PML occurs when immune control of persistent infection with JCPyV fails, the virus mutates and changes its cellular tropism, enters the brain and infects astrocytes, oligodendrocytes and, in particular cases, also neurones. Currently, there is no therapy for this often fatal disease. A number of approaches have failed, and only the restoration of protective immunity, if possible, can lead to clearance of the virus once PML has occurred...
November 9, 2017: Swiss Medical Weekly
https://www.readbyqxmd.com/read/29119538/safety-and-effectiveness-of-natalizumab-first-report-of-interim-results-of-post-marketing-surveillance-in-japan
#14
Takahiko Saida, Kazumasa Yokoyama, Ryusuke Sato, Haruki Makioka, Yukihiko Iizuka, Masakazu Hase, Yan Ling, Shinichi Torii
INTRODUCTION: Natalizumab, a humanized anti-α4 integrin monoclonal antibody, received marketing approval in Japan in 2014 for the treatment of multiple sclerosis (MS). Because the previous large-scale clinical trials of natalizumab were mainly conducted in Europe and North American countries, and data in patients with MS from Japan were limited, we conducted an all-case post-marketing surveillance of natalizumab-treated MS patients from Japan to investigate the safety and effectiveness of natalizumab in a real-world clinical setting in Japan...
November 8, 2017: Neurology and Therapy
https://www.readbyqxmd.com/read/29091757/infectious-entry-and-neutralization-of-pathogenic-jc-polyomaviruses
#15
Eileen M Geoghegan, Diana V Pastrana, Rachel M Schowalter, Upasana Ray, Wei Gao, Mitchell Ho, Gary T Pauly, Dina M Sigano, Campbell Kaynor, Ellen Cahir-McFarland, Benoit Combaluzier, Jan Grimm, Christopher B Buck
Progressive multifocal leukoencephalopathy (PML) is a lethal brain disease caused by uncontrolled replication of JC polyomavirus (JCV). JCV strains recovered from the brains of PML patients carry mutations that prevent the engagement of sialylated glycans, which are thought to serve as receptors for the infectious entry of wild-type JCV. In this report, we show that non-sialylated glycosaminoglycans (GAGs) can serve as alternative attachment receptors for the infectious entry of both wild-type and PML mutant JCV strains...
October 31, 2017: Cell Reports
https://www.readbyqxmd.com/read/29068484/the-clinical-utility-of-jc-virus-antibody-index-measurements-in-the-context-of-progressive-multifocal-leukoencephalopathy
#16
REVIEW
A Q Reuwer, M Heron, D van der Dussen, T Schneider-Hohendorf, J L Murk
In natalizumab-treated patients without previous immunosuppressive treatment, the JCV antibody index is used to stratify PML risk. A high index value indicates that the risk to develop PML is significantly elevated, although probably about 99% of patients with this index value will not develop PML. This minireview aimed to provide an overview of the basic virology and immunology relevant to understanding JCV infections in MS patients, with a focus on what is presently known about antibodies to JCV and how they could be of use to predict and diagnose PML...
November 2017: Acta Neurologica Scandinavica
https://www.readbyqxmd.com/read/29055444/progressive-multifocal-leukoencephalopathy-immune-reconstitution-inflammatory-syndrome-pml-iris-in-liver-transplant-recipient
#17
J Avsenik, A Horvat Ledinek, K Šurlan Popovič
65-year-old liver transplant recipient presented with progressive neurologic dysfunction. CSF analysis revealed high JC virus load and MRI findings suggested the diagnosis of progressive multifocal leukoencephalopathy (PML). Cidofovir and mirtazapine were initiated and patient's regular immunosuppressants were reduced. Subsequently patient developed left sided hemiplegia, drowsiness and severe neglect syndrome. MRI revealed enlargement of PML lesions with contrast enhancement and worsening of oedema, consistent with immune reconstitution inflammatory syndrome (IRIS)...
October 2017: Multiple Sclerosis and related Disorders
https://www.readbyqxmd.com/read/29044559/discovery-and-characterization-of-novel-trans-spliced-products-of-human-polyoma-jc-virus-late-transcripts-from-pml-patients
#18
A Sami Saribas, Julia DeVoto, Akhil Golla, Hassen S Wollebo, Martyn K White, Mahmut Safak
Although the human neurotropic polyomavirus, JC virus (JCV), was isolated almost a half century ago, understanding the molecular mechanisms governing its biology remains highly elusive. JCV infects oligodendrocytes and astrocytes in the central nervous system (CNS) and causes a fatal brain disease known as progressive multifocal leukoencephalopathy (PML) in immunocompromised individuals including AIDS. It has a small circular DNA genome (∼ 5 kb) and generates two primary transcripts from its early and late coding regions, producing several predicted alternatively spliced products mainly by cis-splicing...
October 16, 2017: Journal of Cellular Physiology
https://www.readbyqxmd.com/read/29034436/natalizumab-therapy-is-associated-with-changes-in-serum-jc-virus-antibody-indices-over-time
#19
John Peters, Eric Williamson
To examine changes in anti-JC Virus (JCV) index measurements over time in multiple sclerosis (MS) patients to better understand this test, which is used in assessing risk of progressive multifocal leukoencephalopathy (PML) with natalizumab. We aim to describe and compare seroconversion rates, variability of JCV antibody index values, and changes in index values over time between patients on natalizumab therapy and patients naïve to natalizumab. Anti-JCV index values are used to help decide whether to start, continue, or stop treatment...
December 2017: Journal of Neurology
https://www.readbyqxmd.com/read/29025774/posterior-fossa-progressive-multifocal-leukoencephalopathy-first-presentation-of-an-unknown-autoimmune-disease
#20
Paulette Scholten, Peter Kralt, Bram Jacobs
We present a case of a 57-year-old man who presented with progressive cerebellar dysarthria and cerebellar ataxia. Additional investigations confirmed the diagnosis of progressive multifocal leukoencephalopathy (PML) in the posterior fossa. This is a demyelinating disease of the central nervous system, caused by an opportunistic infection with John Cunningham virus. PML has previously been considered a lethal condition, but because of careful monitoring of patients with HIV and of patients using immunosuppressive drugs it is discovered in earlier stages and prognosis can be improved...
October 11, 2017: BMJ Case Reports
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