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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/28507266/mystery-case-csf-1r-mutation-is-a-cause-of-intracranial-cerebral-calcifications-cysts-and-leukoencephalopathy
#3
(no author information available yet)
No abstract text is available yet for this article.
May 16, 2017: Neurology
https://www.readbyqxmd.com/read/28507265/summary-author-response-mystery-case-csf-1r-mutation-is-a-cause-of-intracranial-cerebral-calcifications-cysts-and-leukoencephalopathy
#4
https://www.readbyqxmd.com/read/28507264/author-response-mystery-case-csf-1r-mutation-is-a-cause-of-intracranial-cerebral-calcifications-cysts-and-leukoencephalopathy
#5
Xavier Ayrignac
No abstract text is available yet for this article.
May 16, 2017: Neurology
https://www.readbyqxmd.com/read/28507263/letter-re-mystery-case-csf-1r-mutation-is-a-cause-of-intracranial-cerebral-calcifications-cysts-and-leukoencephalopathy
#6
David S Lynch, Henry Houlden
No abstract text is available yet for this article.
May 16, 2017: Neurology
https://www.readbyqxmd.com/read/28506192/the-expanding-prominence-of-toxic-leukoencephalopathy
#7
Christopher M Filley, Brice V McConnell, C Alan Anderson
Toxic leukoencephalopathy (TL) is a disorder of brain white matter caused by exposure to leukotoxic agents. Magnetic resonance imaging (MRI) can readily identify this syndrome, and, together with diffusion tensor imaging, MRI continues to offer important insights into its nature. Since the first formal description of TL in 2001, many new leukotoxic disorders have been recognized, and the range of leukotoxins has expanded to include more therapeutic drugs, drugs of abuse, and environmental insults. While the understanding of pathophysiology remains incomplete, TL is increasingly common in clinical practice, and the potential long-term cognitive sequelae of toxic white matter injury merit attention...
May 16, 2017: Journal of Neuropsychiatry and Clinical Neurosciences
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
#8
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
#9
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/28500648/bergmann-glia-translocation-a-new-disease-marker-for-vanishing-white-matter-identifies-therapeutic-effects-of-guanabenz-treatment
#10
Stephanie Dooves, Marianna Bugiani, Lisanne Wisse, Truus E M Abbink, Marjo S van der Knaap, Vivi M Heine
Vanishing White Matter (VWM) is a devastating leukoencephalopathy without effective treatment options. Patients have mutations in the EIF2B1-5 genes, encoding the five subunits of eIF2B, a guanine exchange factor that is an important regulator of protein translation. We recently developed mouse models for VWM that replicate the human disease. To study disease improvement after treatment in these mice it is essential to have sensitive biomarkers related to disease stage. The Bergmann glia of the cerebellum, an astrocytic subpopulation, translocate into the molecular layer in symptomatic VWM mice and patients...
May 13, 2017: Neuropathology and Applied Neurobiology
https://www.readbyqxmd.com/read/28497440/-treatment-of-relapsing-remitting-multiple-sclerosis-with-fingolimod-in-routine-clinical-practice
#11
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
#12
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/28487252/capecitabine-induced-acute-toxic-leukoencephalopathy
#13
M Obadia, D Leclercq, Johanna Wasserman, D Galanaud, D Dormont, M Sahli-Amor, Dimitri Psimaras, N Pyatigorskaya, B Law-Ye
A 45-year-old woman was treated by Capecitabine (Xeloda(®)) during 6days for breast cancer with metastatic bone lesions when she presented with nausea, headaches, muscle cramps, dysarthria and swallowing disorders. A stroke was first suspected. Brain CT was normal. MRI showed bilateral and symmetric high signal intensities of deep white matter, corpus callosum and corticospinal tracts on diffusion-weighted imaging and T2 fluid-attenuated inversion recovery (FLAIR) sequence, similar to 5-FU acute leukoencephalopathy...
May 6, 2017: Neurotoxicology
https://www.readbyqxmd.com/read/28481191/natalizumab-associated-progressive-multifocal-leukoencephalopathy-is-not-preceded-by-elevated-drug-concentrations
#14
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/28479817/r141c-mutation-of-notch3-gene-in-cerebral-autosomal-dominant-arteriopathy-with-subcortical-infarcts-and-leukoencephalopathy
#15
Halil Onder, Kemal Kurtcu, Ethem Murat Arsava, Mehmet Akif Topcuoglu
No abstract text is available yet for this article.
April 2017: Journal of Neurosciences in Rural Practice
https://www.readbyqxmd.com/read/28477179/contribution-of-qsm-imaging-to-the-diagnosis-of-the-rare-syndrome-of-leukoencephalopathy-with-cysts-and-calcification-lcc
#16
Peter Raab, Martin Stangel, Heinrich Lanfermann
We describe the typical computed tomography (CT) and magnetic resonance imaging (MRI) characteristics in a rare case of adult leukoencephalopathy with calcifications and cysts (LCC). In addition, we describe the specific findings of quantified susceptibility mapping (QSM) in this case, relate it to known histopathological findings, and point out possible advantages of this method.
May 5, 2017: Clinical Neuroradiology
https://www.readbyqxmd.com/read/28473916/progressive-multifocal-leukoencephalopathy-after-heart-transplantation-4-years-of-clinically-stable-infection-on-low-dose-immunosuppressive-therapy
#17
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/28470822/pericyte-derived-bone-morphogenetic-protein-4-underlies-white-matter-damage-after-chronic-hypoperfusion
#18
Maiko T Uemura, Masafumi Ihara, Takakuni Maki, Takayuki Nakagomi, Seiji Kaji, Kengo Uemura, Tomohiro Matsuyama, Raj N Kalaria, Ayae Kinoshita, Ryosuke Takahashi
Subcortical small vessel disease (SVD) is characterized by white matter damage resulting from arteriolosclerosis and chronic hypoperfusion. Transforming growth factor beta 1 (TGFB1) is dysregulated in the hereditary SVD, CARASIL (cerebral autosomal recessive arteriopathy with subcortical infarcts and leukoencephalopathy). However, very little is known about the role of the largest group in the TGFB superfamily - the bone morphogenetic proteins (BMPs) - in SVD pathogenesis. The aim of this study was to characterize signaling abnormalities of BMPs in sporadic SVD...
May 4, 2017: Brain Pathology
https://www.readbyqxmd.com/read/28469098/study-of-enhanced-depth-imaging-optical-coherence-tomography-in-cerebral-autosomal-dominant-arteriopathy-with-subcortical-infarcts-and-leukoencephalopathy
#19
Xiao-Jing Fang, Meng Yu, Yuan Wu, Zi-Hao Zhang, Wei-Wei Wang, Zhao-Xia Wang, Yun Yuan
BACKGROUND: Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a hereditary small artery disease caused by NOTCH3 gene mutation. We performed enhanced depth imaging optical coherence tomography (EDI-OCT) to evaluate the retinal vessel changes in CADASIL patients and assessed their consonance with brain magnetic resonance imaging (MRI) findings. METHODS: Of 27 genetically confirmed patients and an equal number of controls were recruited at the Peking University First Hospital from January 2015 to August 2016...
May 5, 2017: Chinese Medical Journal
https://www.readbyqxmd.com/read/28468254/natalizumab-in-multiple-sclerosis-long-term-management
#20
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
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