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Progressive multifocal leukoencephalopathy

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https://www.readbyqxmd.com/read/28526178/late-onset-progressive-multifocal-leukoencephalopathy-in-hodgkin-lymphoma
#1
Whitley W Aamodt, James E Siegler, Angela N Viaene, Michael N Rubenstein
Progressive multifocal leukoencephalopathy (PML) is a demyelinating disease resulting from infection of oligodendrocytes in the central nervous system with John Cunningham virus. Although PML is commonly diagnosed in immunocompromised patients with human immunodeficiency virus, it can also arise in other immunodeficient states. In this report, we present an unusual case of PML occurring 40years after chemoradiation therapy for Hodgkin lymphoma in a patient with normal total lymphocyte counts on annual surveillance...
May 16, 2017: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
https://www.readbyqxmd.com/read/28523195/disseminated-necrotizing-leukoencephalopathy-complicating-septic-shock-in-an-immunocompetent-patient
#2
Pedro Gaspar-da-Costa, Sofia Reimão, Sandra Braz, João Meneses Santos, Rui M M Victorino
Disseminated necrotizing leukoencephalopathy (DNL) is characterized by multiple microscopic foci of white matter necrosis. DNL was initially thought to be exclusively associated with immunosuppression conditions but it has been recently described in immunocompetent patients in septic shock. A 90-year-old immunocompetent woman with no previous neurological impairment presented with septic shock and drowsiness that responded well to therapy with clinical improvement and a full neurological recovery. Unexpectedly deterioration with progression to coma occurred...
2017: Case Reports in Critical Care
https://www.readbyqxmd.com/read/28502942/brain-biopsy-is-more-reliable-than-the-dna-test-for-jc-virus-in-cerebrospinal-fluid-for-the-diagnosis-of-progressive-multifocal-leukoencephalopathy
#3
Junji Ikeda, Akira Matsushima, Wataru Ishii, Tetuya Goto, Kenta Takahashi, Kazuo Nakamichi, Masayuki Saijo, Yoshiki Sekijima, Shu-Ichi Ikeda
The current standard diagnostic approach for progressive multifocal leukoencephalopathy (PML) is to perform a DNA test to identify the presence of the JC virus in cerebrospinal fluid (CSF). A 32-year-old woman with a 5-year history of systemic lupus erythematosus developed right hemiplegia and motor aphasia. MRI revealed a large white matter lesion in the left frontal lobe. JC virus DNA was undetectable in the CSF, but a brain biopsy showed typical histopathology and a high DNA load of the JC virus. The patient was treated with mefloquine and mirtazapine, and is currently alive at 24 months after onset...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28502940/pet-imaging-of-18-f-fdg-11-c-methionine-11-c-flumazenil-and-11-c-4dst-in-progressive-multifocal-leukoencephalopathy
#4
Kenji Ishibashi, Yoshiharu Miura, Ken Matsumura, Yusuke Kanemasa, Kazuo Nakamichi, Masayuki Saijo, Jun Toyohara, Kenji Ishii
The use of positron emission tomography (PET) imaging in progressive multifocal leukoencephalopathy (PML) has rarely been reported. We herein report a set of PET images in a 63-year-old patient with PML. In PML lesions, the uptake of (18)F-fluorodeoxyglucose, (11)C-methionine, (11)C-flumazenil, and [methyl-(11)C]4'-thiothymidine was decreased, increased, decreased, and unchanged, respectively. These results suggest that glucose metabolism decreased, protein synthesis increased, neuronal integrity decreased, and the DNA synthesis and cellular proliferation of host cells were not activated in PML lesions...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28497440/-treatment-of-relapsing-remitting-multiple-sclerosis-with-fingolimod-in-routine-clinical-practice
#5
C Alcala-Vicente, F C Perez-Miralles, F Gascon-Gimenez, I Bosca-Blasco, A Navarre-Gimeno, F Coret-Ferrer, B Casanova-Estruch
INTRODUCTION: Fingolimod is a selective immunosuppressant that targets the S1P receptor, and is indicated in the treatment of aggressive relapsing-remitting multiple sclerosis (RRMS) and following treatment failure with first-order drugs. AIM: To investigate the safety and effectiveness of fingolimod under the conditions of routine clinical practice. PATIENTS AND METHODS: We conducted an observational study with prospective follow-up of patients with RRMS who received fingolimod from January 2011 until February 2014...
May 16, 2017: Revista de Neurologia
https://www.readbyqxmd.com/read/28493815/the-biology-of-jc-polyomavirus
#6
Benedetta Assetta, Walter J Atwood
JC polyomavirus (JCPyV) is the causative agent of a fatal central nervous system demyelinating disease known as progressive multifocal leukoencephalopathy (PML). PML occurs in people with underlying immunodeficiency or in individuals being treated with potent immunomodulatory therapies. JCPyV is a DNA tumor virus with a double stranded DNA genome and encodes a well-studied oncogene, large T antigen. Its host range is highly restricted to humans and only a few cell types support lytic infection in vivo or in vitro...
May 10, 2017: Biological Chemistry
https://www.readbyqxmd.com/read/28481191/natalizumab-associated-progressive-multifocal-leukoencephalopathy-is-not-preceded-by-elevated-drug-concentrations
#7
Clemens Warnke, Hans-Peter Hartung
No abstract text is available yet for this article.
May 1, 2017: Multiple Sclerosis: Clinical and Laboratory Research
https://www.readbyqxmd.com/read/28473916/progressive-multifocal-leukoencephalopathy-after-heart-transplantation-4-years-of-clinically-stable-infection-on-low-dose-immunosuppressive-therapy
#8
Per Sundbom, Laila Hubbert, Lena Serrander
Progressive multifocal leukoencephalopathy (PML), caused by reactivation of JC-virus is a relatively rare complication seen in patients with compromised immune system. There are no evidence-based treatment available and prognosis is poor. Withdrawal of immunosuppressant can result in further neurological deterioration and for patients with solid organ transplantations, fatal graft rejection. We report a 52-year-old women that presented with seizures within 1 month after heart transplantation. Initial diagnosis was vascular disease...
February 2017: Oxford Medical Case Reports
https://www.readbyqxmd.com/read/28468254/natalizumab-in-multiple-sclerosis-long-term-management
#9
REVIEW
Marinella Clerico, Carlo Alberto Artusi, Alessandra Di Liberto, Simona Rolla, Valentina Bardina, Pierangelo Barbero, Stefania Federica De Mercanti, Luca Durelli
Natalizumab is a monoclonal antibody highly effective in the treatment of relapsing remitting multiple sclerosis (RRMS) patients. Despite its effectiveness, there are growing concerns regarding the risk of progressive multifocal leukoencephalopathy (PML), a brain infection caused by John Cunningham virus (JCV), particularly after 24 doses and in patients who previously received immunosuppressive drugs. Long-term natalizumab treated, immunosuppressive-pretreated, and JCV antibody-positive patients are asked to rediscuss natalizumab continuation or withdrawal after 24 doses...
April 29, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/28462542/clinical-profile-and-outcome-of-progressive-multifocal-leukoencephalopathy-in-hiv-infected-indian-patients
#10
Vipul Shah, Harsh Toshniwal, Manoj Shevkani
BACKGROUND AND OBJECTIVE: Progressive multifocal leukoencephalopathy (PML) is a fatal demyelinating disease of the Central nervous system (CNS) caused by the human polyoma virus JC (JCV). Human Immunodeficiency Virus (HIV) infection predisposes to PML. Very sparse data is available regarding the effect of Highly Active Anti Retroviral Therapy (HAART) on clinical outcome of PML in Indian settings. This study was carried out to look into clinical profile, survival and neurological outcome of HIV infected PML patients in HAART era...
March 2017: Journal of the Association of Physicians of India
https://www.readbyqxmd.com/read/28453853/single-molecule-sequencing-revealing-the-presence-of-distinct-jc-polyomavirus-populations-in-patients-with-progressive-multifocal-leukoencephalopathy
#11
Hanna Seppälä, Elina Virtanen, Mika Saarela, Pia Laine, Lars Paulín, Laura Mannonen, Petri Auvinen, Eeva Auvinen
Background.: Progressive multifocal leukoencephalopathy (PML) is a fatal disease caused by reactivation of JC polyomavirus (JCPyV) in immunosuppressed individuals and lytic infection by neurotropic JCPyV in glial cells. The exact content of neurotropic mutations within individual JCPyV strains has not been studied to our knowledge. Methods.: We exploited the capacity of single-molecule real-time sequencing technology to determine the sequence of complete JCPyV genomes in single reads...
March 15, 2017: Journal of Infectious Diseases
https://www.readbyqxmd.com/read/28445498/metabolic-profiles-by-1h-magnetic-resonance-spectroscopy-in-natalizumab-associated-post-pml-lesions-of-multiple-sclerosis-patients-who-survived-progressive-multifocal-leukoencephalopathy-pml
#12
Ruth Schneider, Barbara Bellenberg, Robert Hoepner, Eva-Maria Kolb, Gisa Ellrichmann, Aiden Haghikia, Ralf Gold, Carsten Lukas
PURPOSE: Early diagnosis and treatment of multiple sclerosis-related progressive multifocal leukoencephalopathy (PML) significantly improve clinical outcomes. However, there is a lack of information regarding the restart of immunomodulatory therapy in the post-PML setting, when multiple sclerosis activity reappears. We aimed at the examination of metabolic differences using 1H-magnetic resonance spectroscopy (1H-MRS) in multiple sclerosis patients at various post-PML stages and at the exploration of differences according to their disease and JC virus (JCV) status...
2017: PloS One
https://www.readbyqxmd.com/read/28439420/an-unusual-cause-of-fatal-rapid-onset-ataxia-plus-syndrome
#13
Ivan Kmezic, Jan Weinberg, Dan Hauzenberger, Farouk Hashim, Evangelia Kollia, Monika Klimkowska, Inger Nennesmo, Martin Paucar
BACKGROUND: Progressive multifocal leukoencephalopathy (PML) is a demyelinating disorder of the central nervous system caused by reactivation of the JC-virus and is in most cases associated with underlying immunosuppression. Acquired immune deficiency syndrome (AIDS) and hematological malignancies are well-known predisposing factors for PML. However, in the past ten years, various pharmacological agents have been associated with increased risk of PML. Based on the phenomenology PML can be divided into the cerebral form and the rare cerebellar form...
2017: Cerebellum & Ataxias
https://www.readbyqxmd.com/read/28431609/benefit-risk-perception-of-natalizumab-therapy-in-neurologists-and-a-large-cohort-of-multiple-sclerosis-patients
#14
Christoph Heesen, Ingo Kleiter, Sven G Meuth, Julia Krämer, Jürgen Kasper, Sascha Köpke, Wolfgang Gaissmaier
BACKGROUND: Natalizumab (NAT) is associated with the risk of progressive multifocal leukoencephalopathy (PML). Risk stratification algorithms have been developed, however, without detectable reduction of PML incidence. OBJECTIVE: To evaluate to which extent patients and physicians understand and accept risks associated with NAT treatment. METHODS: Prospective observational cohort study in German MS centers (n=73) among NAT-treated MS patients (n=801) and their neurologists (n=99)...
May 15, 2017: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/28428206/current-and-emerging-therapies-in-multiple-sclerosis-implications-for-the-radiologist-part-2-surveillance-for-treatment-complications-and-disease-progression
#15
REVIEW
C McNamara, G Sugrue, B Murray, P J MacMahon
An understanding of the new generation of MS drugs in conjunction with the key role MR imaging plays in the detection of disease progression, opportunistic infections, and drug-related adverse effects is of vital importance to the neuroradiologist. Part 1 of this review outlined the current treatment options available for MS and examined the mechanisms of action of the various medications. It also covered specific complications associated with each form of therapy. Part 2, in turn deals with the subject of pharmacovigilance and the optimal frequency of MRI monitoring for each individual patient, depending on his or her unique risk profile...
April 20, 2017: AJNR. American Journal of Neuroradiology
https://www.readbyqxmd.com/read/28413713/catastrophic-outcome-of-patients-with-a-rebound-after-natalizumab-treatment-discontinuation
#16
Inés González-Suarez, Luis Rodríguez de Antonio, Aida Orviz, Sara Moreno-García, María D Valle-Arcos, Jordi A Matias-Guiu, Cristina Valencia, Manuela Jorquera Moya, Celia Oreja-Guevara
INTRODUCTION: Natalizumab (NTZ) is an effective drug for the treatment of relapsing-remitting multiple sclerosis. In some patients discontinuation is mandatory due to the risk of progressive multifocal leukoencephalopathy. However, severe clinical and radiological worsening has been described after drug cessation. Our aim was to describe the clinical and radiological features of the rebound phenomenon. MATERIAL AND METHODS: Patients switched from NTZ to Fingolimod (FTY) who had presented a rebound after discontinuation were selected...
April 2017: Brain and Behavior
https://www.readbyqxmd.com/read/28413313/additional-risk-minimisation-measures-for-medicinal-products-in-the-european-union-a-review-of-the-implementation-and-effectiveness-of-measures-in-the-united-kingdom-by-one-marketing-authorisation-holder
#17
Elaine Agyemang, Lorna Bailey, John Talbot
INTRODUCTION: Additional risk minimisation measures (aRMMs) for medicinal products are necessary to address specific important safety issues which may not be practically achieved through routine risk management measures alone. The implementation and determination of effectiveness for aRMMs can be a challenge as it involves multiple stakeholders. It is therefore important to have concise objectives to avoid undue burden on patients, healthcare professionals and the healthcare system. AIM: The aim of this study was to examine how aRMMs are implemented and how effectiveness is assessed in the European Union (EU) using practical examples from Roche Products Limited in the United Kingdom (UK) (referred to as the 'Company')...
2017: Pharmaceutical Medicine
https://www.readbyqxmd.com/read/28406921/is-the-risk-of-progressive-multifocal-leukoencephalopathy-the-real-reason-for-natalizumab-discontinuation-in-patients-with-multiple-sclerosis
#18
MULTICENTER STUDY
Julia Krämer, Jan-Gerd Tenberge, Ingo Kleiter, Wolfgang Gaissmaier, Tobias Ruck, Christoph Heesen, Sven G Meuth
BACKGROUND: Progressive multifocal leukoencephalopathy (PML) is one of the major risks of natalizumab therapy. Despite introduction of the currently employed PML risk stratification algorithm, the incidence of natalizumab-associated PML cases is not decreasing. OBJECTIVES: We addressed the following questions: How do natalizumab-treated multiple sclerosis patients and their treating physicians assess and deal with PML risk? Is PML risk the real reason for natalizumab discontinuation? METHODS: 699 natalizumab-treated multiple sclerosis patients and 99 physicians were included in this prospective observational study...
2017: PloS One
https://www.readbyqxmd.com/read/28378895/monitoring-cd49d-receptor-occupancy-a-method-to-optimize-and-personalize-natalizumab-therapy-in-multiple-sclerosis-patients
#19
Joan Puñet-Ortiz, José Vicente Hervás-García, Aina Teniente-Serra, Antonio Cano-Orgaz, Maria José Mansilla, Bibiana Quirant-Sánchez, Juan Navarro-Barriuso, Marco A Fernández-Sanmartín, Silvia Presas-Rodríguez, Cristina Ramo-Tello, Eva María Martínez-Cáceres
BACKGROUND: In natalizumab-treated relapsing-remitting MS (RRMS) patients, various extended interval dosing strategies are under evaluation to minimize severe treatment-associated side effects, mainly progressive multifocal leukoencephalopathy development. Up to now, it has not been presented any approach, even in form of assay design, to determine the optimal percentage of CD49d receptor occupancy (RO) associated with a favorable clinical, radiological, and immunological response. METHODS: A multiparametric quantitative flow cytometry method was settled to measure CD49d RO on peripheral blood lymphocytes...
April 5, 2017: Cytometry. Part B, Clinical Cytometry
https://www.readbyqxmd.com/read/28362268/progressive-multifocal-leukoencephalopathy-due-to-john-cunningham-jc-virus-following-allogeneic-hematopoietic-cell-transplantation
#20
Wilson Lam, Zeyad Al-Shaibani, Deepali Kumar, Auro Viswabandya, Santhosh Thyagu, Fotios V Michelis, Jeffrey H Lipton, Hans A Messner, Uday Deotare
Progressive multifocal leukoencephalopathy (PML) is an uncommon infectious complication post allogeneic stem cell transplant (allo-SCT). We present a case report of a patient developing this complication with a review of the current literature. It also describes the first use of artesunate in a clinical case of PML with no beneficial effect.
March 31, 2017: Antiviral Therapy
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