keyword
MENU ▼
Read by QxMD icon Read
search

leukoencephalopathies

keyword
https://www.readbyqxmd.com/read/28641055/long-term-safety-evaluation-of-natalizumab-for-the-treatment-of-multiple-sclerosis
#1
Marinella Clerico, Carlo Alberto Artusi, Alessandra Di Liberto, Simona Rolla, Valentina Bardina, Pierangelo Barbero, Stefania Federica De Mercanti, Luca Durelli
Introduction Natalizumab is a humanized monoclonal antibody highly effective in relapsing-remitting multiple sclerosis (MS). Important concerns about its safety have been pointed out because of the risk of progressive multifocal leukoencephalopathy (PML), caused by the opportunistic John-Cunningham virus (JCV). Areas covered This review analyzes all the safety aspects related to the use of natalizumab in MS patients. Fatigue and allergic reactions are not-severe adverse events (AEs) occurring more frequently than placebo and no differences in serious AEs (SAE) have been observed comparing to interferon-β1a during clinical trials...
June 22, 2017: Expert Opinion on Drug Safety
https://www.readbyqxmd.com/read/28635952/yunis-var%C3%A3-n-syndrome-caused-by-biallelic-vac14-mutations
#2
Matthew A Lines, Yoko Ito, Kristin D Kernohan, Wendy Mears, Julie Hurteau-Miller, Sunita Venkateswaran, Leanne Ward, Karine Khatchadourian, Jeff McClintock, Priya Bhola, Philippe M Campeau, Kym M Boycott, Jean Michaud, André Bp van Kuilenburg, Sacha Ferdinandusse, David A Dyment
Yunis-Varón syndrome (YVS) is an autosomal recessive disorder comprising skeletal anomalies, dysmorphism, global developmental delay and intracytoplasmic vacuolation in brain and other tissues. All hitherto-reported pathogenic variants affect FIG4, a lipid phosphatase involved in phosphatidylinositol (3,5)-bisphosphate [PtdIns(3,5)P2] metabolism. FIG4 interacts with PIKfyve, a lipid kinase, via the adapter protein VAC14; all subunits of the resulting complex are essential for PtdIns(3,5)P2 synthesis in the endolysosomal membrane compartment...
June 21, 2017: European Journal of Human Genetics: EJHG
https://www.readbyqxmd.com/read/28635875/-disturbances-of-gait-and-postural-stability-in-chronic-cerebral-ischemia
#3
N N Vakhnina, V V Zakharov
Disturbances of gait and postural stability are characteristic of the chronic progressive vascular lesion of the brain observed in moderate to severe stages of cerebrovascular disease. Disconnections between the prefrontal cortex, basal ganglia and cerebellum due to the damage of the white matter (vascular leukoencephalopathy) underlie disturbances of gait and postural stability (shorter stride length, wider gait). There are difficulties in starting with possible stoppings during the walking, waiting in front of small barriers as well as difficulties in concentration (e...
2017: Zhurnal Nevrologii i Psikhiatrii Imeni S.S. Korsakova
https://www.readbyqxmd.com/read/28629398/anaplastic-astrocytoma-mimicking-progressive-multifocal-leucoencephalopathy-a-case-report-and-review-of-the-overlapping-syndromes
#4
Ema Kantorová, Michal Bittšanský, Štefan Sivák, Eva Baranovičová, Petra Hnilicová, Vladimír Nosáľ, Daniel Čierny, Kamil Zeleňák, Wolfgang Brück, Egon Kurča
BACKGROUND: Co-occurrence of multiple sclerosis (MS) and glial tumours (GT) is uncommon although occasionally reported in medical literature. Interpreting the overlapping radiologic and clinical characteristics of glial tumours, MS lesions, and progressive multifocal leukoencephalopathy (PML) can be a significant diagnostic challenge. CASE PRESENTATION: We report a case of anaplastic astrocytoma mimicking PML in a 27-year-old patient with a 15-year history of MS...
June 19, 2017: BMC Cancer
https://www.readbyqxmd.com/read/28628708/brain-calcifications-in-adult-onset-genetic-leukoencephalopathies-a-review
#5
Xavier Ayrignac, Gaël Nicolas, Clarisse Carra-Dallière, Didier Hannequin, Pierre Labauge
Importance: Adult-onset genetic leukoencephalopathies and leukodystrophies are increasingly recognized as a heterogeneous group of disorders with new diagnostic approaches and potential treatments. In the new era of genomics, the challenging interpretation of individual genetic variations requires an accurate phenotypic description and classification. Clinical and magnetic resonance imaging (MRI)-based approaches have been proposed to improve the diagnostic process of adult-onset leukoencephalopathies...
June 19, 2017: JAMA Neurology
https://www.readbyqxmd.com/read/28625242/-clinical-significance-of-combined-detection-of-peripheral-mir-182-and-il-17-in-the-early-diagnosis-of-patients-with-eclampsia-complicated-with-cerebral-infarction-in-icu
#6
Lina Zhang, Tiejun Wu, Dawei Wu, Yanjun Liu, Min Bai, Shuangfeng Chen
OBJECTIVE: To observe the effect of peripheral blood microRNA-182 (miR-182) combined with interleukin-17 (IL-17) in the early diagnosis of cerebral infarction (CI) in patients with eclampsia. METHODS: A prospective non-randomized controlled study was conducted. The patients with eclampsia admitted to intensive care unit (ICU) of Liaocheng People's Hospital from January 1st, 2013 to September 30th 2016 were enrolled. Cerebral imaging was conducted in 7 days after admission to make a definite diagnosis of the occurrence of CI, excluding patients with cerebral hemorrhage...
June 2017: Zhonghua Wei Zhong Bing Ji Jiu Yi Xue
https://www.readbyqxmd.com/read/28623673/a-prominent-lactate-peak-as-a-potential-key-magnetic-resonance-spectroscopy-mrs-feature-of-progressive-multifocal-leukoencephalopathy-pml-spectrum-pattern-observed-in-three-patients
#7
Duško Kozić, Mladen Bjelan, Jasmina Boban, Jelena Ostojić, Vesna Turkulov, Aleksandar Todorović, Slobodanka Lemajić-Komazec, Snežana Brkić
Progressive multifocal leukoencephalopathy (PML) is a rare, often fatal, opportunistic infection, associated with demyelinating process. PML is caused by John Cunningham (JC) polyomavirus, and predominantly involves patients with human immunodeficiency virus (HIV) disease or other immunocompromised conditions. The purpose of this report was to determine the role of magnetic resonance spectroscopy (MRS) in establishing the diagnosis of PML. MRS with long and short echo time was performed in two patients with PML associated with HIV infection and in one PML patient associated with chronic lymphocytic leukemia...
June 17, 2017: Bosnian Journal of Basic Medical Sciences
https://www.readbyqxmd.com/read/28617385/-differential-diagnosis-of-cerebral-autosomal-dominant-arteriopathy-with-subcortical-infarcts-and-leukoencephalopathy
#8
A A Moroz, N Yu Abramycheva, M S Stepanova, R N Konovalov, S L Timerbaeva, S N Illarioshkin
Cerebral autosomal dominant arteriopathy with subcortical infarctions and leucoencephalopathy (CADASIL) is an inherited CNS disease, which is caused by mutations in the NOTCH3 gene. Selective disorders of small vessels underlie the disease pathogenesis. Clinically CADASIL is characterized by headaches, multiple stroke-like disorders (in most cases transient ischemic attacks and lacunar strokes), and different focal neurological symptoms and dementia. There are specific MRI signs of the disease: multiple lacunar infarctions located in the basal ganglia, brain steam and cerebellum, focal lesions of temporal poles, capsula externa, periventricular and subcortical areas; diffuse white matter changes and leukoaraiosis can be observed as well...
2017: Zhurnal Nevrologii i Psikhiatrii Imeni S.S. Korsakova
https://www.readbyqxmd.com/read/28616447/natalizumab-pml-survivors-with-subsequent-ms-treatment-clinico-radiologic-outcome
#9
Elisabeth Maillart, Jean-Sebastien Vidal, David Brassat, Bruno Stankoff, Agnès Fromont, Jérôme de Sèze, Frédéric Taithe, Pierre Clavelou, Bertrand Bourre, Valérie Delvaux, Audrey Rico, Pierre Labauge, Ayman Tourbah, Christine Lebrun, Jean Pelletier, Thibault Moreau, Céline Louapre, Catherine Lubetzki, Caroline Papeix
OBJECTIVE: To describe the clinico-radiologic outcome of MS patients with natalizumab-related progressive multifocal leukoencephalopathy (Nz-PML) surviving and receiving disease-modifying therapy (DMT). METHODS: We describe clinical and radiologic evolution of Nz-PML survivors in an observational retrospective multicenter cohort to clarify the effect of different subsequent MS DMT strategies. Twenty-three patients from 11 centers were analyzed. Outcomes were (1) clinical efficacy of post-PML MS DMT, (2) radiologic efficacy of post-PML MS DMT, (3) radiologic evolution of PML lesion, and (4) disability progression...
May 2017: Neurology® Neuroimmunology & Neuroinflammation
https://www.readbyqxmd.com/read/28608406/pregnancy-in-cadasil
#10
I Donnini, V Rinnoci, S Nannucci, R Valenti, F Pescini, G Mariani, S Bianchi, M T Dotti, A Federico, D Inzitari, L Pantoni
OBJECTIVES: Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is an inherited cerebral small vessel disease caused by NOTCH3 gene mutations. CADASIL women are frequently considered at high risk of systemic vascular events during pregnancy and often prescribed with antithrombotic drugs. This decision is not evidence-based considering the lack of data about pregnancy outcome in CADASIL. We describe our experience on pregnancy in CADASIL patients...
June 12, 2017: Acta Neurologica Scandinavica
https://www.readbyqxmd.com/read/28601945/cadasil-mutant-notch3-r90c-decreases-the-viability-of-hs683-oligodendrocytes-via-apoptosis
#11
Mibo Tang, Changhe Shi, Bo Song, Jing Yang, Ting Yang, Chengyuan Mao, Yusheng Li, Xinjing Liu, Shuyu Zhang, Hui Wang, Haiyang Luo, Yuming Xu
Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is the most common hereditary cerebral small vessel disease caused by mutations in NOTCH3. Prevailing models suggest that demyelination occurs secondary to vascular pathology. However, in zebrafish, NOTCH3 is also expressed in mature oligodendrocytes. Thus, we hypothesized that in addition to vascular defects, mutant NOTCH3 may alter glial function in individuals with CADASIL. The aim of this study was to characterize the direct effects of a mutant NOTCH3 protein in HS683 oligodendrocytes...
June 10, 2017: Molecular Biology Reports
https://www.readbyqxmd.com/read/28601287/cd4-cd8-ratio-during-natalizumab-treatment-in-multiple-sclerosis-patients
#12
Antonio Carotenuto, Giulia Scalia, Francesco Ausiello, Marcello Moccia, Cinzia Valeria Russo, Francesco Saccà, Anna De Rosa, Chiara Criscuolo, Luigi Del Vecchio, Vincenzo Brescia Morra, Roberta Lanzillo
Although improved, the risk of progressive multifocal leukoencephalopathy is a constant threat for patient affected by multiple sclerosis treated with natalizumab. We performed a 24months longitudinal study aimed to evaluate the total WBC and lymphocytes subsets modifications and their correlations with anti-JCV antibody index after 1 and 2years of natalizumab treatment. Natalizumab induced an increase of WBC, total and C19+ lymphocytes together with a decrease of CD3+, CD4+ T lymphocytes and CD4/CD8 ratio, which was positively related to anti-JCV antibodies index at month 0, 12 and 24...
August 15, 2017: Journal of Neuroimmunology
https://www.readbyqxmd.com/read/28592708/low-signal-intensity-rims-along-the-cerebral-cortex-and-u-fibers-on-susceptibility-weighted-imaging-in-progressive-multifocal-leukoencephalopathy
#13
Yuki Arita, Hirokazu Fujiwara, Jun Kurasawa, Suketaka Momoshima, Tsubasa Takizawa, Masahiro Jinzaki
No abstract text is available yet for this article.
June 8, 2017: Magnetic Resonance in Medical Sciences: MRMS
https://www.readbyqxmd.com/read/28588848/a-case-of-megalencephalic-leukoencephalopathy-with-subcortical-cysts-type-1-was-identified-with-a-novel-compound-heterozygous-alteration-c-135delc-c-423-2dupt-in-china
#14
Cong-Ling Dai, Wen-Bin He, Juan Du, Yue-Qiu Tan, Guang-Xiu Lu, Wen Li
We report a compound heterozygous mutation (c.135delC; c.423+2dupT) of MLC1 gene in a Chinese patient underlying infantile macrocephaly and neurological deterioration in early childhood. Brain MRI revealed diffusion abnormality in swollen white matter and a subcortical cyst. The cDNA sequencing analysis for the c.423+2dupT variant revealed skipping of exon 5.
June 2017: Clinical Case Reports
https://www.readbyqxmd.com/read/28588577/immune-reconstitution-inflammatory-syndrome-unmasking-or-worsening-aids-related-progressive-multifocal-leukoencephalopathy-a-literature-review
#15
REVIEW
Anna Fournier, Guillaume Martin-Blondel, Emmanuèle Lechapt-Zalcman, Julia Dina, Apolline Kazemi, Renaud Verdon, Emmanuel Mortier, Arnaud de La Blanchardière
Incidence of progressive multifocal leukoencephalopathy (PML) in HIV-infected patients has declined in the combined antiretroviral therapy (cART) era although a growing number of acquired immunodeficiency syndrome (AIDS)-related PML-immune reconstitution inflammatory syndromes (PML-IRIS) have been published during the same period. Therapeutic management of PML-IRIS is not consensual and mainly relies on corticosteroids. Our main aim was, in addition to provide a thoughtful analysis of published PML-IRIS cases, to assess the benefit of corticosteroids in the management of PML-IRIS, focusing on confirmed cases...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/28585592/magnetic-resonance-spectroscopy-and-magnetic-resonance-spectroscopic-imaging-in-cerebral-autosomal-dominant-arteriopathy-with-subcortical-infarcts-and-leukoencephalopathy-a-literature-review
#16
Ali Sibtain Farooq Sheikh, Mona Adel Mohamed
This review focuses on the current literature directed towards the brain metabolite findings in Cerebral Autosomal-Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL) disease using magnetic resonance spectroscopy (MRS) and magnetic resonance spectroscopic imaging (MRSI). Using search terms "metabolites", "spectroscopy", and "CADASIL", six articles were found on PubMed database, Scopus and Google Scholar. Changes in metabolites concentrations and relative ratios (RR) were found not only in abnormal but also in normal-appearing brain regions...
June 2017: JPMA. the Journal of the Pakistan Medical Association
https://www.readbyqxmd.com/read/28580052/mri-diagnosis-of-infantile-alexander-disease-in-a-14-month-old-african-boy
#17
Nondumiso Dlamini, Vicci du Plessis
Alexander disease, also known as fibrinoid leukodystrophy, is a rare leukoencephalopathy which occurs due to a mutation in the glial fibrillary acid protein (GFAP) gene. Magnetic resonance imaging (MRI) has proven to be highly sensitive in making the diagnosis. Typical MRI findings, in combination with positive genetic blood analysis, confirm the diagnosis.
October 2016: Journal of Radiology Case Reports
https://www.readbyqxmd.com/read/28578630/neuroaxonal-dystrophy-and-cavitating-leukoencephalopathy-of-chihuahua-dogs
#18
Sara Degl'Innocenti, Nimrod Asiag, Offer Zeira, Cristian Falzone, Carlo Cantile
A novel form of neuroaxonal dystrophy is described in 3 Chihuahua pups, 2 of which were from the same litter. It was characterized not only by accumulation of numerous and widely distributed axonal swellings (spheroids) but also by a severe cavitating leukoencephalopathy. The dogs presented with progressive neurological signs, including gait abnormalities and postural reaction deficits. Magnetic resonance images and gross examination at necropsy revealed dilation of lateral ventricles and cerebral atrophy, accompanied by cavitation of the subcortical white matter...
January 1, 2017: Veterinary Pathology
https://www.readbyqxmd.com/read/28576902/application-of-pharmacogenomics-to-investigate-adverse-drug-reactions-to-the-disease-modifying-treatments-for-multiple-sclerosis-a-case-control-study-protocol-for-dimethyl-fumarate-induced-lymphopenia
#19
Kaarina Kowalec, Elaine Kingwell, Robert Carruthers, Ruth Ann Marrie, Sasha Bernatsky, Anthony Traboulsee, Colin J D Ross, Bruce Carleton, Helen Tremlett
INTRODUCTION: Adverse drug reactions (ADRs) are a global public health issue. The potential for pharmacogenomic biomarkers has been demonstrated in several therapeutical areas, including HIV infection and oncology. Dimethyl fumarate (DMF) is a licensed disease-modifying therapy for the treatment of multiple sclerosis (MS). The use of DMF in MS has been associated with a severe reduction in lymphocyte counts and reports of progressive multifocal leukoencephalopathy. Here, we outline the protocol for a case-control study designed to discover genomic variants associated with DMF-induced lymphopenia...
June 2, 2017: BMJ Open
https://www.readbyqxmd.com/read/28570330/neurologic-complications-of-transplantation
#20
Amy A Pruitt
PURPOSE OF REVIEW: This article describes the diagnosis and management of neurologic problems during hematopoietic cell and solid organ transplantation using time elapsed since transplantation as a guide to expected complications, including drug toxicities, infections, strokes, autoimmune phenomena, disease recurrence, and secondary neoplasms. RECENT FINDINGS: Growing clinical experience in the neurology of transplantation has led to appreciation of the diverse clinical and radiographic spectrum of calcineurin inhibitor-related posterior reversible encephalopathy syndrome (PRES) and progressive multifocal leukoencephalopathy...
June 2017: Continuum: Lifelong Learning in Neurology
keyword
keyword
72206
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"