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Leukoencephalopathy

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https://www.readbyqxmd.com/read/29328996/acute-and-sub-acute-neurological-toxicity-in-children-treated-for-acute-lymphoblastic-leukemia
#1
REVIEW
Natalia C Millan, Analía Pastrana, Myriam R Guitter, Pedro A Zubizarreta, María S Monges, María S Felice
Eighty percent of children with acute lymphoblastic leukemia (ALL) survive with current treatments. Neurotoxicity is an infrequent adverse event. We describe clinical presentations of neurological toxicity, phases of treatment when these adverse events were more frequent and patients ́ outcome. From January-1995 to December-2015, 1379 ALL cases were admitted. Neurotoxicity was diagnosed in 49 patients (3.6%) and classified according to neurological syndromes. Medical records, laboratory-tests and images were reviewed...
January 2, 2018: Leukemia Research
https://www.readbyqxmd.com/read/29323725/serum-igg-antibodies-from-healthy-subjects-up-to-100-years-old-react-to-jc-polyomavirus
#2
Ilaria Bononi, Elisa Mazzoni, Silvia Pietrobon, Marco Manfredini, Elena Trregiani, Marika Rossini, Francesca Lotito, Giovanni Guerra, Paola Rizzo, Fernanda Martini, Mauro Tognon
JC polyomavirus (JCPyV) was identified in 1971 in the brain tissue of a patient (J.C.) affected by the progressive multifocal leukoencephalopathy (PML). JCPyV encodes for the oncoproteins large T antigen (Tag) and small t-antigen (tag). These oncoproteins are responsible of the cell transformation and tumorigenesis in experimental animals. JCPyV is ubiquitous in human populations. After the primary infection, which is usually asymptomatic, JCPyV remains lifelong in the host in a latent phase. Its reactivation may occur in heathy subjects and immunocompromised patients...
January 11, 2018: Journal of Cellular Physiology
https://www.readbyqxmd.com/read/29322824/detection-and-analysis-of-variants-of-jc-polyomavirus-in-urine-samples-from-hiv-1-infected-patients-in-china-s-zhejiang-province
#3
Caiqin Hu, Ying Huang, Junwei Su, Mengyan Wang, Qihui Zhou, Biao Zhu
Objectives Human JC polyomavirus (JCPyV) infection has an increased risk of developing progressive multifocal leukoencephalopathy (PML). Different JCPyV subtypes differ in the virulence with which they cause PML. Currently, the JCPyV infection status and subtype distribution in patients with human immunodeficiency virus-1 (HIV-1) in China are still unclear. This study aimed to investigate the epidemiology and subtype distribution of JCPyV in HIV-1-infected patients in China. Methods Urine samples from 137 HIV-1-infected patients in Zhejiang Province in China were tested for the presence of JCPyV DNA...
January 1, 2018: Journal of International Medical Research
https://www.readbyqxmd.com/read/29321332/erk-is-a-critical-regulator-of-jc-polyomavirus-infection
#4
Jeanne K DuShane, Michael P Wilczek, Colleen L Mayberry, Melissa S Maginnis
The human JC polyomavirus (JCPyV) infects the majority of the population worldwide and presents as an asymptomatic, persistent infection in the kidney. In individuals who are immunocompromised, JCPyV can become reactivated and cause a lytic infection in the central nervous system resulting in the fatal, demyelinating disease progressive multifocal leukoencephalopathy (PML). Infection is initiated by interactions between the capsid protein viral protein 1 (VP1) and the α2,6-linked sialic acid on LSTc, while JCPyV internalization is facilitated by 5-hydroxytryptamine 2 receptors (5-HT2Rs)...
January 10, 2018: Journal of Virology
https://www.readbyqxmd.com/read/29321230/progressive-multifocal-leukoencephalopathy-are-you-aware-of-it
#5
EDITORIAL
David B Clifford, Thomas Berger
No abstract text is available yet for this article.
January 10, 2018: Neurology
https://www.readbyqxmd.com/read/29321229/the-national-incidence-of-pml-in-sweden-1988-2013
#6
Ellen Iacobaeus, Sarah Burkill, Shahram Bahmanyar, Ramil Hakim, Camilla Byström, Michael Fored, Tomas Olsson, Lou Brundin, Scott Montgomery
OBJECTIVE: To investigate the incidence of progressive multifocal leukoencephalopathy (PML) and patient characteristics in Sweden between 1988 and 2013. METHODS: All PML diagnoses in Sweden between 1988 and 2013 were identified in the National Patient Register. Information to validate the diagnosis and patient characteristics was obtained from medical records. RESULTS: Medical record review classified 108 out of 250 patients (43%) as definite (n = 84), probable (n = 4), or possible (n = 20) PML according to diagnostic criteria...
January 10, 2018: Neurology
https://www.readbyqxmd.com/read/29320313/acute-radiologic-manifestations-of-america-s-opioid-epidemic
#7
David D B Bates, Katherine Gallagher, HeiShun Yu, Jennifer Uyeda, Akira M Murakami, Bindu N Setty, Stephan W Anderson, Mariza O Clement
The United States is in the midst of an opioid use epidemic, which has severe medical, social, and economic consequences. Addictions to and abuse of prescription and illicit opioids are increasing, and emergency department radiologists are increasingly being faced with the task of examining patients who present with opioid-related complications. These complications may be the result of direct drug toxicity or nonsterile injection of the drugs. Neurologic, musculoskeletal, cardiopulmonary, genitourinary, and gastrointestinal complications may be evident at diagnostic imaging in emergent settings...
January 2018: Radiographics: a Review Publication of the Radiological Society of North America, Inc
https://www.readbyqxmd.com/read/29304918/progressive-multifocal-leukoencephalopathy
#8
Melissa M Blessing, Aditya Raghunathan, Lori A Erickson
No abstract text is available yet for this article.
January 2018: Mayo Clinic Proceedings
https://www.readbyqxmd.com/read/29302065/adulthood-leukodystrophies
#9
REVIEW
Wolfgang Köhler, Julian Curiel, Adeline Vanderver
The leukodystrophies are a group of inherited white matter disorders with a heterogeneous genetic background, considerable phenotypic variability and disease onset at all ages. This Review focuses on leukodystrophies with major prevalence or primary onset in adulthood. We summarize 20 leukodystrophies with adult presentations, providing information on the underlying genetic mutations and on biochemical assays that aid diagnosis, where available. Definitions, clinical characteristics, age of onset, MRI findings and treatment options are all described, providing a comprehensive overview of the current knowledge of the various adulthood leukodystrophies...
January 5, 2018: Nature Reviews. Neurology
https://www.readbyqxmd.com/read/29296850/progressive-multifocal-leukoencephalopathy-and-hematologic-malignancies-a-single-cancer-center-retrospective-review
#10
Elizabeth C Neil, Lisa M DeAngelis
Progressive multifocal leukoencephalopathy (PML) is an uncommon opportunistic infection with high morbidity and mortality. This is an institutional review board-approved retrospective review of medical records identified by diagnostic coding for PML or John Cunningham virus (JCV) from 2000 to 2015. Inclusion criteria were cerebrospinal fluid (CSF) positive for JCV by polymerase chain reaction or brain biopsy-proven PML in non-HIV patients. There were 16 patients, 12 of whom were men (75%); the median age was 56 years (range, 31-71 years)...
October 24, 2017: Blood Advances
https://www.readbyqxmd.com/read/29288492/-leukodystrophy-like-phenotype-in-children-with-myelin-oligodendrocyte-glycoprotein-antibody-associated-disease
#11
Yael Hacohen, Thomas Rossor, Kshitij Mankad, Wk 'Kling' Chong, Andrew Lux, Evangeline Wassmer, Ming Lim, Frederik Barkhof, Olga Ciccarelli, Cheryl Hemingway
AIM: To review the demographics and clinical and paraclinical parameters of children with myelin oligodendrocyte glycoprotein (MOG) antibody-associated relapsing disease. METHOD: In this UK-based, multicentre study, 31 children with MOG antibody-associated relapsing disease were studied retrospectively. RESULTS: Of the 31 children studied, 14 presented with acute disseminated encephalomyelitis (ADEM); they were younger (mean 4.1y) than the remainder (mean 8...
December 30, 2017: Developmental Medicine and Child Neurology
https://www.readbyqxmd.com/read/29283439/homozygous-boricua-tbck-mutation-causes-neurodegeneration-and-aberrant-autophagy
#12
Xilma R Ortiz-González, Jesus A Tintos-Hernández, Kierstin Keller, Xueli Li, A Reghan Foley, Diana X Bharucha-Goebel, Sudha K Kessler, Sabrina W Yum, Peter B Crino, Miao He, Douglas C Wallace, Carsten G Bönnemann
OBJECTIVE: Autosomal recessive mutations in TBCK cause intellectual disability of variable severity. Although the physiologic function of TBCK remains unclear, loss-of-function mutations are associated with inhibition of mTORC1 signaling. As mTORC1 signaling is known to regulate autophagy, we hypothesized that TBCK-encephalopathy patients with a neurodegenerative course have defects in autophagic-lysosomal dysfunction. METHODS: Children (n=8) of Puerto Rican (Boricua) descent affected with homozygous TBCK p...
December 28, 2017: Annals of Neurology
https://www.readbyqxmd.com/read/29279639/posterior-reversible-leukoencephalopathy-syndrome-pres-in-intensive-care-unit-case-series
#13
Antara Gokhale, Atheel Kimona, Sandeep Kantor, S Prakash, Yogesh Manhas
Introduction: Posterior reversible encephalopathy syndrome (PRES) is characterized by a range of clinico neuro radiological manifestation along with hypertension due to varied etiology. Contrary to its name, a small number of patients have residual neurological deficits. Patients with severe manifestation of PRES may get admitted to the Intensive Care Unit (ICU) due to coma or status epilepticus. Small case series and large multicenter studies have been reported. Materials and Methods: We report a single center series of ten cases admitted to ICU over a 5 year period in a tertiary care centre...
November 2017: Indian Journal of Critical Care Medicine
https://www.readbyqxmd.com/read/29260032/longitudinal-clinical-and-neuro-radiological-findings-in-a-patient-with-leukoencephalopathy-with-brain-calcifications-and-cysts-labrune-syndrome
#14
Yasushi Iwasaki, Ken-Ichiro Hoshino, Keiko Mori, Masumi Ito, Yoshinari Kawai, Maya Mimuro, Tamao Tsukie, Takeshi Ikeuchi, Mari Yoshida
Since she was 4 years old, the patient had exhibited frequent convulsive seizures, and she experienced severe headaches and depression in adulthood. At the age of 37 years, cerebral calcifications were detected, but she exhibited no cognitive or motor problems. She suffered a cerebral haemorrhage at 49 years old and experienced cognitive dysfunction, dysarthria, dysphagia, and left-hemiparesis as sequelae. After undergoing gastrostomy, she exhibited very slow cognitive deterioration associated with speech disturbance over more than 10 years...
September 2017: ENeurologicalSci
https://www.readbyqxmd.com/read/29259854/microglia-express-abi3-in-the-brains-of-alzheimer-s-disease-and-nasu-hakola-disease
#15
Jun-Ichi Satoh, Yoshihiro Kino, Motoaki Yanaizu, Youhei Tosaki, Kenji Sakai, Tsuyoshi Ishida, Yuko Saito
Nasu-Hakola disease (NHD) is a rare autosomal recessive leukoencephalopathy caused by a loss-of-function mutation of either TYROBP (DAP12) or TREM2 expressed in microglia. A rare variant of the TREM2 gene encoding p.Arg47His causes a 3-fold increase in the risk for late-onset Alzheimer's disease (LOAD). A recent study demonstrated that a rare coding variant p.Ser209Phe in the ABI family member 3 (ABI3) gene, a regulator of actin cytoskeleton organization, confers risk of developing of LOAD, although the pattern of ABI3 expression in AD and NHD brains with relevance to microglial pathology remains to be characterized...
November 2017: Intractable & Rare Diseases Research
https://www.readbyqxmd.com/read/29245268/posterior-reversible-encephalopathy-syndrome-after-postpartum-hemorrhage-and-uterine-artery-embolization-a-case-report
#16
Fangfang Shi, Liwei Shen, Yonghui Shi, Lei Shi, Xiaoli Yang, Zhi Jin, Wenpeng Liu, Danhong Wu
RATIONALE: Posterior reversible encephalopathy syndrome (PRES) is characterized by clinical and radiological features, including headache, disturbed consciousness, seizures, and cortical blindness associated with findings indicating posterior leukoencephalopathy on imaging studies. Ours is the first case of PRES developing after postpartum hemorrhage and uterine artery embolization. PATIENT CONCERNS: An 18-year-old patient had postpartum hemorrhage after a normal delivery...
December 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29244098/clemastine-rescues-myelination-defects-and-promotes-functional-recovery-in-hypoxic-brain-injury
#17
Bruce A C Cree, Jianqin Niu, Kimberly K Hoi, Chao Zhao, Scott D Caganap, Roland G Henry, Dang Q Dao, Daniel R Zollinger, Feng Mei, Yun-An A Shen, Robin J M Franklin, Erik M Ullian, Lan Xiao, Jonah R Chan, Stephen P J Fancy
Hypoxia can injure brain white matter tracts, comprised of axons and myelinating oligodendrocytes, leading to cerebral palsy in neonates and delayed post-hypoxic leukoencephalopathy (DPHL) in adults. In these conditions, white matter injury can be followed by myelin regeneration, but myelination often fails and is a significant contributor to fixed demyelinated lesions, with ensuing permanent neurological injury. Non-myelinating oligodendrocyte precursor cells are often found in lesions in plentiful numbers, but fail to mature, suggesting oligodendrocyte precursor cell differentiation arrest as a critical contributor to failed myelination in hypoxia...
December 13, 2017: Brain: a Journal of Neurology
https://www.readbyqxmd.com/read/29243131/analysis-of-variability-of-urinary-excreted-jc-virus-strains-in-patients-infected-with-hiv-and-healthy-donors
#18
Danijela Karalic, Ivana Lazarevic, Ana Banko, Maja Cupic, Djordje Jevtovic, Tanja Jovanovic
In immunocompromised individuals, especially in patients with T cell immunodeficiency, reactivation of JCPyV can cause serious life-threatening diseases. Nowadays, HIV infection is one of the most important factor for reactivation of JCPyV and the development of of the progressive multifocal leukoencephalopathy (PML). Mutations in the outer loops of the VP1 region can lead to the selection of the viral variants with changed tropism and increased pathological potential. The aims of this study were to determine sequence variation and amino acid changes within VP1 loops and the structure of non-coding control region (NCCR) of urinary excreted JCPyV isolates among HIV-infected patients and healthy donors...
December 14, 2017: Journal of Neurovirology
https://www.readbyqxmd.com/read/29231623/progressive-multifocal-leukoencephalopathy
#19
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
#20
EDITORIAL
Yassine Taoufik, Marie-Ghislaine de Goër de Herve
No abstract text is available yet for this article.
2017: Frontiers in Immunology
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