keyword
MENU ▼
Read by QxMD icon Read
search

Leukoencephalopathy

keyword
https://www.readbyqxmd.com/read/28228569/no-evidence-of-beneficial-effects-of-plasmapheresis-in-natalizumab-associated-pml
#1
Doriana Landi, Nicola De Rossi, Sara Zagaglia, Cristina Scarpazza, Luca Prosperini, Maria Albanese, Fabio Buttari, Francesco Mori, Girolama Alessandra Marfia, Maria Pia Sormani, Ruggero Capra, Diego Centonze
OBJECTIVE: To examine retrospectively the effects of plasmapheresis (PLEX) on the survival and clinical outcomes of patients with multiple sclerosis (MS) and natalizumab (NTZ)-associated progressive multifocal leukoencephalopathy (PML). METHODS: The medical literature was searched for the terms natalizumab and progressive multifocal leukoencephalopathy. A total of 193 international and 34 Italian NTZ-PML cases were included. Clinical outcome was determined by comparing the patients' clinical status at PML diagnosis with status after PML resolution...
February 22, 2017: Neurology
https://www.readbyqxmd.com/read/28228564/natalizumab-associated-pml-challenges-with-incidence-resulting-risk-and-risk-stratification
#2
Nicholas Schwab, Tilman Schneider-Hohendorf, Nico Melzer, Gary Cutter, Heinz Wiendl
Progressive multifocal leukoencephalopathy (PML) associated with natalizumab treatment continues to be a severe problem of clinically successful therapy. This is an update of risk stratification developments and discusses the current approach to depict and calculate PML incidence and PML risk. (1) PML incidence and resulting risk used in today's clinical practice are potentially outdated and the risk for patients with prior immunosuppression might have been underestimated. (2) Risk stratification according to treatment duration epochs likely suggests lower risk due to patients stopping treatment within a given epoch...
February 22, 2017: Neurology
https://www.readbyqxmd.com/read/28225293/rituximab-associated-progressive-multifocal-leukoencephalopathy
#3
Juraj Sokol, Lenka Lisá, Jana Zeleňáková, Tomáš Balhárek, Ivana Plameňová, Ján Staško, Peter Kubisz
The definition "Progressive Multifocal Leukoencephalopathy" (PML) was first used in 1958 to describe a fatal demyelinating central nervous system (CNS) disease in patients with lymphoproliferative disorders. In 1971, the virus responsible for the disease was isolated and named John Cunningham virus (JCV). We present a rare case of a 62-year-old male with chronic lymphocytic leukemia and PML. In our work, we discuss the diagnostic and therapeutic challenges and offer suggestions for preventing PML development...
2017: Vnitr̆ní Lékar̆ství
https://www.readbyqxmd.com/read/28216420/evaluation-of-rag1-mutations-in-an-adult-with-combined-immunodeficiency-and-progressive-multifocal-leukoencephalopathy
#4
Claudia Schröder, Niklas Baerlecken, Ulrich Pannicke, Thilo Dörk, Torsten Witte, Roland Jacobs, Matthias Stoll, Klaus Schwarz, Bodo Grimbacher, Reinhold E Schmidt, Faranaz Atschekzei
Here we describe novel mutations in recombination activation gene 1 (RAG1) in a compound heterozygous male patient with combined T and B cell immunodeficiency (CID). Clinical manifestations besides antibody deficiency included airway infections, granulomatosis and autoimmune features. He died at the age of 37 due to PML caused by JC virus infection. By targeted next-generation sequencing we detected post mortem in this patient three mutations in RAG1. One allele harbored two novel mutations (c.1123C>G, p...
February 16, 2017: Clinical Immunology: the Official Journal of the Clinical Immunology Society
https://www.readbyqxmd.com/read/28214184/-systemic-lupus-erythematosus-and-lymphopenia-clinical-and-pathophysiological-features
#5
M Martin, A Guffroy, X Argemi, T Martin
Lymphopenia is frequent in systemic lupus erythematosus (SLE) and profound (<500/mm(3)) in 10% of cases. T lymphocytes, especially CD4+, are more affected than B cells. The pathophysiological mechanisms are complex, involving lymphocytotoxic antibodies, excess of apoptosis, increased susceptibility of T cells to complement mediated cytolysis, as well as lymphopoiesis impairment and lymphocyte sequestration. Lymphopenia in SLE is independent from other cytopenia and immunosuppressive drug regiments, and associated with disease activity, risk of flare and damage scores...
February 14, 2017: La Revue de Médecine Interne
https://www.readbyqxmd.com/read/28212801/notch-signaling-in-vascular-smooth-muscle-cells
#6
J T Baeten, B Lilly
The Notch signaling pathway is a highly conserved pathway involved in cell fate determination in embryonic development and also functions in the regulation of physiological processes in several systems. It plays an especially important role in vascular development and physiology by influencing angiogenesis, vessel patterning, arterial/venous specification, and vascular smooth muscle biology. Aberrant or dysregulated Notch signaling is the cause of or a contributing factor to many vascular disorders, including inherited vascular diseases, such as cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy, associated with degeneration of the smooth muscle layer in cerebral arteries...
2017: Advances in Pharmacology
https://www.readbyqxmd.com/read/28203182/treatable-leukoencephalopathy-in-a-patient-with-hypophosphatemia
#7
Masahiro Okazaki, Makito Hirano, Tomoki Iwatsu, Masaki Yamana, Hidekazu Suzuki, Takao Satou, Susumu Kusunoki
We report the first patient with pathologically proven leukoencephalopathy associated with hypophosphatemia. A 61-year-old woman had repetitive episodes of decreased consciousness with pontine and pallidal lesions and extensive leukoencephalopathy on MRI, later found to be associated with hypophosphatemia. Although hypophosphatemia has been linked to central pontine and extrapontine myelinolysis (osmotic myelinolysis), lesions in the deep white matter have not been reported. Brain biopsy performed during the first diagnosis process revealed nonspecific demyelination with gliosis, a finding similar to that of chronic osmotic myelinolysis...
September 2016: Case Reports in Neurology
https://www.readbyqxmd.com/read/28202707/cadasil-accelerated-by-acute-hypotension-arterial-and-venous-contribution-to-leukoaraiosis
#8
Jacqueline A Pettersen, Julia Keith, Fuqiang Gao, J David Spence, Sandra E Black
OBJECTIVE: To underline the importance of blood pressure regulation in cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) and to describe changes that occur in the veins in this condition, specifically venous collagenosis associated with leukoaraiosis. METHODS: Case report with neuroimaging and pathologic data. RESULTS: A 61-year-old man with genetically confirmed CADASIL was initially lucid following a motor vehicle accident but subsequently became hypotensive (60/40 mm Hg) due to an open femur fracture and required intubation...
February 15, 2017: Neurology
https://www.readbyqxmd.com/read/28202068/the-dna-damage-response-promotes-polyomavirus-jc-infection-by-nucleus-to-cytoplasm-nf-kappab-activation
#9
Martyn K White, Anna Bellizzi, Gabriele Ibba, Valeria Pietropaolo, Anna T Palamara, Hassen S Wollebo
BACKGROUND: Infection of glial cells by human neurotropic polyomavirus JC (JCV), the causative agent of the CNS demyelinating disease progressive multifocal leukoencephalopathy (PML), rapidly inflicts damage to cellular DNA. This activates DNA damage response (DDR) signaling including induction of expression of DNA repair factor Rad51. We previously reported that Rad51 co-operates with the transcription factor NF-κB p65 to activate JCV early transcription. Thus Rad51 induction by JCV infection may provide positive feedback for viral activation early in JCV infection...
February 15, 2017: Virology Journal
https://www.readbyqxmd.com/read/28193754/author-response-acute-intermittent-porphyria-related-leukoencephalopathy
#10
Marjo S van der Knaap, Sietske H Kevelam
No abstract text is available yet for this article.
February 14, 2017: Neurology
https://www.readbyqxmd.com/read/28193753/letter-re-acute-intermittent-porphyria-related-leukoencephalopathy
#11
Wladimir B V R Pinto, Paulo Victor Sgobbi de Souza, Thiago Bortholin, Marco Antonio Troccoli Chieia, Acary S B Oliveira
No abstract text is available yet for this article.
February 14, 2017: Neurology
https://www.readbyqxmd.com/read/28190435/neurologic-complications-of-transplantation
#12
R Dhar
Major neurologic morbidity, such as seizures and encephalopathy, complicates 20-30% of organ and stem cell transplantation procedures. The majority of these disorders occur in the early posttransplant period, but recipients remain at risk for opportunistic infections and other nervous system disorders for many years. These long-term risks may be increasing as acute survival increases, and a greater number of "sicker" patients are exposed to long-term immunosuppression. Drug neurotoxicity accounts for a significant proportion of complications, with posterior reversible leukoencephalopathy syndrome, primarily associated with calcineurin inhibitors (i...
2017: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/28181126/anti-angiogenic-tyrosine-kinase-inhibitors-and-reversible-posterior-leukoencephalopathy-syndrome-could-hypomagnesaemia-be-the-trigger
#13
REVIEW
Rashmi R Shah
Reversible posterior leukoencephalopathy syndrome (RPLS), also known frequently as posterior reversible encephalopathy syndrome (PRES), is a characteristic acute neuro-radiology syndrome with clinical presentation that typically includes acute hypertension, seizures and other neurological symptoms and signs. Many patients with RPLS have (a history of) pre-existing hypertension and in receipt of diuretics. It is being diagnosed more frequently and in association with an increasing number of morbidities and medications...
February 9, 2017: Drug Safety: An International Journal of Medical Toxicology and Drug Experience
https://www.readbyqxmd.com/read/28177126/identification-of-novel-snord118-mutations-in-seven-patients-with-leukoencephalopathy-with-brain-calcifications-and-cysts
#14
Kazuhiro Iwama, Takeshi Mizuguchi, Jun-Ichi Takanashi, Hidehiro Shibayama, Minobu Shichiji, Susumu Ito, Hirokazu Oguni, Toshiyuki Yamamoto, Akiko Sekine, Shun Nagamine, Yoshio Ikeda, Hiroya Nishida, Satoko Kumada, Takeshi Yoshida, Tomonari Awaya, Ryuta Tanaka, Ryo Chikuchi, Hisayoshi Niwa, Yu-Ichi Oka, Satoko Miyatake, Mitsuko Nakashima, Atsushi Takata, Noriko Miyake, Shuichi Ito, Hirotomo Saitsu, Naomichi Matsumoto
Leukoencephalopathy with brain calcifications and cysts (LCC) is neuroradiologically characterized by leukoencephalopathy, intracranial calcification, and cysts. Coats plus syndrome is also characterized by the same neuroradiological findings together with defects in retinal vascular development. Indeed, LCC and Coats plus were originally considered the same clinical entity that was termed cerebroretinal microangiopathy with calcifications and cysts, but evidence now suggests that they are genetically distinct...
February 8, 2017: Clinical Genetics
https://www.readbyqxmd.com/read/28176617/progressive-multifocal-leukoencephalopathy-in-a-kidney-transplant-recipient
#15
Federico Cicora, Javier Roberti
Progressive multifocal leukoencephalopathy (PML) is a debilitating and often fatal viral disease of the central nervous system. A 39-year-old male kidney transplant recipient presented to our hospital with weakness of the left arm and leg, his immunosuppressive regimen consisted of tacrolimus, mycophenolate sodium, and meprednisone. Magnetic resonance imaging of the brain revealed hypercellularity in neural tissue and a lesion affecting the right ventral and lateral areas of the thalamus and the posterior limb of the internal capsule...
February 7, 2017: Experimental and Clinical Transplantation
https://www.readbyqxmd.com/read/28174598/review-on-the-role-of-the-human-polyomavirus-jc-in-the-development-of-tumors
#16
REVIEW
Serena Delbue, Manola Comar, Pasquale Ferrante
Almost one fifth of human cancers worldwide are associated with infectious agents, either bacteria or viruses, and this makes the possible association between infections and tumors a relevant research issue. We focused our attention on the human Polyomavirus JC (JCPyV), that is a small, naked DNA virus, belonging to the Polyomaviridae family. It is the recognized etiological agent of the Progressive Multifocal Leukoencephalopathy (PML), a fatal demyelinating disease, occurring in immunosuppressed individuals...
2017: Infectious Agents and Cancer
https://www.readbyqxmd.com/read/28168372/high-b-value-diffusion-weighted-imaging-in-progressive-multifocal-leukoencephalopathy-in-hiv-patients
#17
Claudia Godi, Enrico De Vita, Enrico Tombetti, Indran Davagnanam, Lewis Haddow, Hans Rolf Jäger
OBJECTIVES: An ill-defined hyperintense edge and hypointense core on diffusion-weighted imaging (DWI) is typical of progressive multifocal leukoencephalopathy (PML). We aimed to investigate whether a b-value of 3,000 s/mm(2) (b3000) can improve visualisation of PML, or provide different structural information compared to 1,000 s/mm(2) (b1000). METHODS: We retrospectively identified HIV-positive patients with confirmed PML studied under a clinical protocol including both b1000 and b3000 DWI...
February 6, 2017: European Radiology
https://www.readbyqxmd.com/read/28167648/natalizumab-for-multiple-sclerosis-a-case-in-point-for-the-impact-of-translational-neuroimmunology
#18
Afsaneh Shirani, Olaf Stüve
Advances in translational neuroimmunology over the last two decades have revolutionized the treatment of relapsing forms of multiple sclerosis. A pathological hallmark of multiple sclerosis is the presence of leukocytes in the areas of disease activity in the CNS. Natalizumab inhibits the trafficking of lymphocytes from the blood into the brain and spinal cord by blocking the adhesion molecule α4-integrin. Representing the enormous success of a molecular targeted approach, natalizumab was the first mAb approved for the treatment of relapsing-remitting multiple sclerosis...
February 15, 2017: Journal of Immunology: Official Journal of the American Association of Immunologists
https://www.readbyqxmd.com/read/28165319/progressive-multifocal-leukoencephalopathy-in-two-natalizumab-treated-stepsisters-an-intriguing-coincidence
#19
Frédéric Bacchetta, Amandine Mathias, Myriam Schluep, Renaud Du Pasquier
BACKGROUND: Natalizumab, a treatment used in multiple sclerosis (MS), is associated with cases of progressive multifocal leukoencephalopathy (PML). OBJECTIVE: We describe two cases of PML in related but not genetically apparented natalizumab-treated MS patients who are stepsisters. Reported cases/outcomes: While Patient 1 developed PML, Patient 2 was on natalizumab and had contacts with Patient 1. Patient 2 was diagnosed with PML 5 months after Patient 1. CONCLUSION: The clinical and temporal data highly suggest that there was JC virus (JCV) transmission from one patient to the other with development of PML as primo-infection in Patient 2...
February 2017: Multiple Sclerosis: Clinical and Laboratory Research
https://www.readbyqxmd.com/read/28163944/immune-reconstitution-inflammatory-syndrome-opening-pandora-s-box
#20
Mariana Meireles, Conceição Souto Moura, Margarida França
One of the purposes of antiretroviral therapy (ART) is to restore the immune system. However, it can sometimes lead to an aberrant inflammatory response and paradoxical clinical worsening known as the immune reconstitution inflammatory syndrome (IRIS). We describe a 23-year-old male, HIV1 infected with a rapid progression phenotype, who started ART with TCD4+ of 53 cells/mm(3) (3,3%) and HIV RNA = 890000 copies/mL (6 log). Four weeks later he was admitted to the intensive care unit with severe sepsis...
2017: Case Reports in Infectious Diseases
keyword
keyword
45903
1
2
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read
×

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"