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https://www.readbyqxmd.com/read/28545192/atypical-teratoid-rhabdoid-tumor-of-brain-a-clinicopathologic-study-of-eleven-patients-and-review-of-literature
#1
Nasir Ud Din, Abrar Barakzai, Aisha Memon, Sheema Hasan, Zubair Ahmad
Background: Atypical teratoid/ rhabdoid tumor (AT/RT) is a rare aggressive embryonal central nervous system (CNS) tumor of infancy and early childhood. Majority of the cases arise in the posterior fossa, and remaining in the cerebrum. Aims: To analyze the clinicopathologic features of AT/RT on a cohort of cases. Materials and methods: All reported cases of AT/RT at the Department of Pathology and Laboratory Medicine, Aga Khan University Hospital (AKUH) from 2007 to 2016 were reviewed for clinical and pathological features...
April 1, 2017: Asian Pacific Journal of Cancer Prevention: APJCP
https://www.readbyqxmd.com/read/28544746/clinical-outcomes-following-proton-therapy-for-children-with-central-nervous-system-tumors-referred-overseas
#2
Daniel J Indelicato, Julie A Bradley, Eric S Sandler, Philipp R Aldana, Amy Sapp, Jennifer E Gains, Adrian Crellin, Ronny L Rotondo
BACKGROUND: International, multidisciplinary care of children with central nervous system (CNS) tumors presents unique challenges. The aim of this study is to report patient outcomes of U.K. children referred for proton therapy to a North American facility. METHODS: From 2008 to 2016, 166 U.K. children with approved CNS tumors were treated with proton therapy at a single academic medical center in the United States. Median age was 7 years (range, 1-19). Median follow-up was 2...
May 24, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28543180/traf6-participates-in-early-brain-injury-after-subarachnoid-hemorrhage-in-rats-through-inhibiting-autophagy-and-promoting-oxidative-stress
#3
Yang Dou, Haitao Shen, Dongxia Feng, Haiying Li, Xiaodi Tian, Jian Zhang, Zhong Wang, Gang Chen
Tumor necrosis factor receptor-associated factor 6 (TRAF6) is a member of the TRAF family and an important multifunctional intracellular adaptin of the tumor necrosis factor (TNF) superfamily and toll/IL-1 receptor (TIR) superfamily. TRAF6 has been studied in several central nervous system (CNS) diseases, including ischemic stroke, traumatic brain injury and neurodegenerative diseases, but its role in subarachnoid hemorrhage (SAH) has not been fully illustrated. This study was designed to explore changes of expression level and potential roles and mechanisms of TRAF6 in early brain injury (EBI) after SAH by using a Sprague-Dawley rat model of SAH induced in 0...
May 24, 2017: Journal of Neurochemistry
https://www.readbyqxmd.com/read/28543098/brain-endothelial-cell-expression-of-sparcl-1-is-specific-to-chronic-multiple-sclerosis-lesions-and-is-regulated-by-inflammatory-mediators-in-vitro
#4
C Bridel, M J A Koel-Simmelink, L Peferoen, C Derada Troletti, S Durieux, R Gorter, E Nutma, P Gami, E Iacobeus, L Brundin, J Kuhle, H Vrenken, J Killestein, S R Piersma, T V Pham, H E De Vries, S Amor, C R Jimenez, C E Teunissen
AIMS: Cell matrix modulating protein SPARCL-1 is highly expressed by astrocytes during CNS development and following acute CNS damage. Applying NanoLC-MS/MS to CSF of RRMS and SPMS patients, we identified SPARCL-1 as differentially expressed between these two stages of MS, suggesting a potential as CSF biomarker to differentiate RRMS from SPMS and a role in MS pathogenesis. METHODS: This study examines the potential of SPARCL-1 as CSF biomarker discriminating RRMS from SPMS in 3 independent cohorts (n=249), analyses its expression pattern in MS lesions (n= 26), and studies its regulation in cultured human brain microvasculature endothelial cells (BEC) after exposure to MS-relevant inflammatory mediators...
May 24, 2017: Neuropathology and Applied Neurobiology
https://www.readbyqxmd.com/read/28542592/fatigue-in-patients-with-neuromyelitis-optica-spectrum-disorder-and-its-impact-on-quality-of-life
#5
Jin Myoung Seok, Misong Choi, Eun Bin Cho, Hye Lim Lee, Byoung Joon Kim, Kwang Ho Lee, Pamela Song, Eun Yeon Joo, Ju-Hong Min
Fatigue is a prevalent symptom and major burden in neuroimmunological diseases. In neuromyelitis optica spectrum disorder (NMOSD), a severe autoimmune central nervous system (CNS) inflammatory disease with autoantibodies reactive to aquaporin-4, there are few reports about fatigue and quality of life (QOL). We aimed to evaluate the severity of fatigue and its relationship with QOL in patients with NMOSD. We prospectively studied patients with NMOSD who were in remission and seropositive for anti-aquaporin-4 antibody, and they were divided into 2 groups based on the presence of fatigue assessed using the Functional Assessment of Chronic Illness Therapy-fatigue score...
2017: PloS One
https://www.readbyqxmd.com/read/28542521/regeneration-of-myelin-sheaths-of-normal-length-and-thickness-in-the-zebrafish-cns-correlates-with-growth-of-axons-in-caliber
#6
Marja J Karttunen, Tim Czopka, Marieke Goedhart, Jason J Early, David A Lyons
Demyelination is observed in numerous diseases of the central nervous system, including multiple sclerosis (MS). However, the endogenous regenerative process of remyelination can replace myelin lost in disease, and in various animal models. Unfortunately, the process of remyelination often fails, particularly with ageing. Even when remyelination occurs, it is characterised by the regeneration of myelin sheaths that are abnormally thin and short. This imperfect remyelination is likely to have implications for the restoration of normal circuit function and possibly the optimal metabolic support of axons...
2017: PloS One
https://www.readbyqxmd.com/read/28540600/microglial-interferon-signaling-and-white-matter
#7
Ashley McDonough, Richard V Lee, Jonathan R Weinstein
Microglia, the resident immune cells of the CNS, are primary regulators of the neuroimmune response to injury. Type I interferons (IFNs), including the IFNαs and IFNβ, are key cytokines in the innate immune system. Their activity is implicated in the regulation of microglial function both during development and in response to neuroinflammation, ischemia, and neurodegeneration. Data from numerous studies in multiple sclerosis (MS) and stroke suggest that type I IFNs can modulate the microglial phenotype, influence the overall neuroimmune milieu, regulate phagocytosis, and affect blood-brain barrier integrity...
May 25, 2017: Neurochemical Research
https://www.readbyqxmd.com/read/28539075/fatigue-in-multiple-sclerosis-associations-with-clinical-mri-and-csf-parameters
#8
Viola Biberacher, Paul Schmidt, Rebecca C Selter, Verena Pernpeinter, Markus C Kowarik, Benjamin Knier, Dorothea Buck, Muna-Miriam Hoshi, Thomas Korn, Achim Berthele, Jan S Kirschke, Claus Zimmer, Bernhard Hemmer, Mark Mühlau
BACKGROUND: Damage of different brain structures has been related to fatigue. Alternatively, functional alterations of central nervous system (CNS) cells by the inflammatory milieu within the CNS may be responsible for the development of fatigue. AIM: To investigate the effect of structural brain damage and inflammatory cerebrospinal fluid (CSF) changes on fatigue in multiple sclerosis (MS). METHODS: We determined the association of different clinical, CSF and magnetic resonance imaging (MRI) parameters with prevalence and severity of fatigue, as measured by the Fatigue Scale for Motor and Cognitive Functions in 68 early MS patients (discovery cohort)...
May 1, 2017: Multiple Sclerosis: Clinical and Laboratory Research
https://www.readbyqxmd.com/read/28538697/mammalian-metallothionein-3-new-functional-and-structural-insights
#9
REVIEW
Milan Vašák, Gabriele Meloni
Metallothionein-3 (MT-3), a member of the mammalian metallothionein (MT) family, is mainly expressed in the central nervous system (CNS). MT-3 possesses a unique neuronal growth inhibitory activity, and the levels of this intra- and extracellularly occurring metalloprotein are markedly diminished in the brain of patients affected by a number of metal-linked neurodegenerative disorders, including Alzheimer's disease (AD). In these pathologies, the redox cycling of copper, accompanied by the production of reactive oxygen species (ROS), plays a key role in the neuronal toxicity...
May 24, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/28538508/central-nervous-system-disease-education-and-race-impact-radiation-refusal-in-pediatric-cancer-patients
#10
Chirayu G Patel, Mark Stavas, Stephanie Perkins, Eric T Shinohara
To investigate the determinants of radiation therapy refusal in pediatric cancer, we used the Surveillance, Epidemiology, and End Results registry to identify 24,421 patients who met the eligibility criteria, diagnosed between 1974 and 2012. Patients had any stage of cancer, were aged 0 to 19, and received radiation therapy or refused radiation therapy when it was recommended. One hundred twenty-eight patients (0.52%) refused radiation therapy when it was recommended. Thirty-two percent of patients who refused radiation therapy ultimately died from their cancer, at a median of 7 months after diagnosis (95% confidence interval, 3-11 mo), as compared with 29...
May 22, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28537651/the-burden-of-hiv-associated-neurocognitive-disorder-hand-in-post-haart-era-a-multidisciplinary-review-of-the-literature
#11
G Caruana, G Vidili, P A Serra, P Bagella, A Spanu, V Fiore, D F Calvisi, R Manetti, G Rocchitta, S Nuvoli, S Babudieri, M M Simile, G Madeddu
OBJECTIVE: The purpose of the present multidisciplinary review is to give an updated insight into the most recent findings regarding the pathophysiology, diagnosis and therapeutics of HIV-associated neurocognitive disorder (HAND). MATERIALS AND METHODS: We performed a comprehensive search, through electronic databases (Pubmed - MEDLINE) and search engines (Google Scholar), of peer-reviewed publications (articles and reviews) and conferences proceedings on HAND pathophysiology, diagnosis, and therapy, from 1999 to 2016...
May 2017: European Review for Medical and Pharmacological Sciences
https://www.readbyqxmd.com/read/28536263/axodendritic-sorting-and-pathological-missorting-of-tau-is-isoform-specific-and-determined-by-axon-initial-segment-architecture
#12
Hans Zempel, Frank Dennissen, Yatender Kumar, Julia Luedtke, Jacek Biernat, Eva-Maria Mandelkow, Eckhard Mandelkow
Subcellular mislocalization of the microtubule-associated protein Tau is a hallmark of Alzheimer disease (AD) and other tauopathies. Six Tau isoforms, differentiated by the presence or absence of a second repeat or of N-terminal inserts, exist in the human CNS but their physiological and pathological differences have long remained remain elusive. Here, we investigated the properties and distributions of human and rodent Tau isoforms in primary forebrain rodent neurons. We found that the Tau-Diffusion-Barrier (TDB), located within the Axon-Initial-Segment (AIS), controls retrograde (axon-to-soma) and anterograde (soma-to-axon) traffic of Tau...
May 23, 2017: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/28535095/managing-cns-disease-in-adults-with-acute-lymphoblastic-leukemia
#13
Richard A Larson
The central nervous system (CNS) is an important site of involvement by acute lymphoblastic leukemia (ALL) in adults. The prevalence is sufficiently high that prophylactic treatment is routinely given to this sanctuary site in order to eradicate occult disease that might otherwise lead to a relapse. A lumbar puncture should be routinely performed in all newly diagnosed patients with ALL. The risks of CNS leukemia vary by phenotype and genotype. Preventive treatment of the CNS during post-remission therapy has become an integral part of all current ALL treatment protocols...
May 23, 2017: Leukemia & Lymphoma
https://www.readbyqxmd.com/read/28535084/impact-of-initial-csf-findings-on-outcome-among-patients-with-national-cancer-institute-standard-and-high-risk-b-cell-acute-lymphoblastic-leukemia-a-report-from-the-children-s-oncology-group
#14
Naomi Winick, Meenakshi Devidas, Si Chen, Kelly Maloney, Eric Larsen, Leonard Mattano, Michael J Borowitz, Andrew Carroll, Julie M Gastier-Foster, Nyla A Heerema, Cheryl Willman, Brent Wood, Mignon L Loh, Elizabeth Raetz, Stephen P Hunger, William L Carroll
Purpose To determine the prognostic significance of blasts, and of white and red blood cells, in CSF samples at diagnosis of acute lymphoblastic leukemia (ALL), a uniform CSF and risk group classification schema was incorporated into Children's Oncology Group B-cell ALL (B-ALL) clinical trials. Methods CSF status was designated as follows: CNS1, no blasts; CNS2a to 2c, < 5 WBCs/μL and blasts with/without ≥ 10 RBCs/μL or ≥ 5 WBCs/μL plus blasts, with WBCs ≥ 5 times the number of RBCs; CNS3a to 3c, ≥ 5 WBCs/μL plus blasts with/without ≥ 10 RBCs/μL or clinical signs of CNS disease...
May 23, 2017: Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology
https://www.readbyqxmd.com/read/28534251/sequencing-of-alk-inhibitors-in-alk-non-small-cell-lung-cancer
#15
REVIEW
Shirish M Gadgeel
Major therapeutic advances have occurred over the last several years in the management of advanced ALK+ NSCLC patients. Crizotinib was the first agent approved for the management of ALK+ NSCLC patients after it demonstrated significantly greater clinical benefit compared to chemotherapy. Several next generation ALK inhibitors have demonstrated clinical benefit in patients with crizotinib refractory NSCLC patients including in the CNS. Based on available data, therapy with a next generation ALK inhibitor can be initiated following therapy with crizotinib without any assessment of the molecular mechanisms of resistance...
June 2017: Current Treatment Options in Oncology
https://www.readbyqxmd.com/read/28534174/the-il-1%C3%AE-phenomena-in-neuroinflammatory-diseases
#16
REVIEW
Andrew S Mendiola, Astrid E Cardona
It is becoming increasingly clear that neuroinflammation has a causal role in the pathogenesis of central nervous system (CNS)-related diseases, and therefore therapeutic strategies targeting the regulation or availability of inflammatory mediators can be used to prevent or mitigate pathology. Interestingly, the proinflammatory cytokine, interleukin-1 beta (IL-1β), has been implicated in perpetuating immune responses and contributing to disease severity in a variety of CNS diseases ranging from multiple sclerosis, neurodegenerative diseases, traumatic brain injury, and diabetic retinopathy...
May 22, 2017: Journal of Neural Transmission
https://www.readbyqxmd.com/read/28533776/reactivity-to-novel-autoantigens-in-patients-with-coexisting-central-nervous-system-demyelinating-disease-and-autoimmune-thyroid-disease
#17
Judith M Greer, Simon Broadley, Michael P Pender
Several lines of evidence suggest a definite and unique link between CNS demyelinating diseases and autoimmune thyroid disease (AITD). The aim of the current study was to systematically compare the clinical and laboratory features of patients with coexistent AITD and CNS demyelinating disease with those of patients with just CNS demyelinating disease. Forty-four patients with coexisting CNS demyelinating disease and AITD were identified and their clinical and radiological features were recorded. Blood and DNA were collected and tested for HLA type and for the response of T cells and antibodies to a variety of antigens...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/28533655/-18-f-fdg-pet-ct-in-localizing-additional-cns-lesion-in-a-case-of-langerhans-cell-histiocytosis-determining-accurate-extent-of-the-disease
#18
Shamim Ahmed Shamim, Sarthak Tripathy, Anirban Mukherjee, Chandrasekhar Bal, Madhavi Tripathi
Central nervous system involvement is a rare manifestation of Langerhans cell histiocytosis (LCH), with bone and skin lesions being more frequent. MR remains the investigation of choice for localizing brain lesions. However, due to poor sensitivity of MRI in detecting osseous and pulmonary lesions, it is not used routinely in staging purposes until and unless indicated. We hereby discuss a case of 6-year-old boy of LCH who was referred for 18-F-FDG PET/CT for staging and knowing the extent of the disease, but a lesion in hypothalamus was picked up incidentally on FDG PET-CT study that was confirmed by MRI...
April 2017: Indian Journal of Nuclear Medicine: IJNM: the Official Journal of the Society of Nuclear Medicine, India
https://www.readbyqxmd.com/read/28532447/clinical-characteristics-of-disseminated-cryptococcosis-in-previously-healthy-children-in-china
#19
Li-Wei Gao, An-Xia Jiao, Xi-Rong Wu, Shun-Ying Zhao, Yun Ma, Gang Liu, Ju Yin, Bao-Ping Xu, Kun-Ling Shen
BACKGROUND: Disseminated cryptococcosis is a rare and fatal disease, and limited data exist regarding it in children. This study aimed to investigate the clinical characteristics of disseminated cryptococcosis in previously healthy children in China. METHODS: Hospitalized patients with disseminated cryptococcosis were enrolled during January 1996 to December 2015 in Beijing Children's Hospital, Capital Medical University, China. Data on clinical manifestations, laboratory tests, treatment, and prognosis were evaluated...
May 22, 2017: BMC Infectious Diseases
https://www.readbyqxmd.com/read/28532172/cranio-spinal-rosai-dorfman-disease-case-series-and-literature-review
#20
Shashank S Joshi, Shilpa Joshi, Girish Muzumdar, Keki E Turel, Rajan M Shah, Indoo Ammbulkar, Muhammad Masood Hussain, Kishor A Choudhari
Rosai-Dorfman disease (RDD) is a rare condition similar to lymphomas, presenting with cervical lymphadenopathy in young adults. Extra-nodal involvement is relatively common but involvement of the central nervous system (CNS) is rare. Cranial RDD presents with symptoms of raised intracranial pressure, focal or generalised seizures, while spinal RDD presents with pain, peripheral neurological deficits and radiculopathy. In contrast to other similar neoplastic or degenerative conditions affecting the CNS, RDD is a benign, non-infective, granulomatous disorder...
May 22, 2017: British Journal of Neurosurgery
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