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https://www.readbyqxmd.com/read/28806346/optic-pathway-gliomas-in-neurofibromatosis-type-1-an-update-surveillance-treatment-indications-and-biomarkers-of-vision
#1
Peter M K de Blank, Michael J Fisher, Grant T Liu, David H Gutmann, Robert Listernick, Rosalie E Ferner, Robert A Avery
Optic pathway gliomas (OPGs) occur in 15%-20% of children with neurofibromatosis type 1 (NF1), leading to visual deficits in fewer than half of these individuals. The goal of chemotherapy is to preserve vision, but vision loss in NF1-associated OPG can be unpredictable. Determining which child would benefit from chemotherapy and, equally important, which child is better observed without treatment can be difficult. Unfortunately, despite frequent imaging and ophthalmologic evaluations, some children experience progressive vision loss before treatment...
September 2017: Journal of Neuro-ophthalmology: the Official Journal of the North American Neuro-Ophthalmology Society
https://www.readbyqxmd.com/read/28796925/neurological-comorbidity-in-children-with-neurofibromatosis-type-1
#2
Keiko Hirabaru, Muneaki Matsuo
OBJECTIVE: To determine the frequency of central nervous system comorbidities in children with neurofibromatosis type 1 (NF1). METHODS: We performed a nationwide survey to investigate neurological comorbidities in 3-15-year-old children with NF1 in Japan by sending questionnaires to pediatricians and pediatric neurologists. A secondary questionnaire was sent to the parents of identified NF1 patients to assess neurological comorbidities including headache, attention deficit hyperactivity disorder (ADHD)-Rating Scale (RS), and the Social Responsiveness Scale -2...
August 10, 2017: Pediatrics International: Official Journal of the Japan Pediatric Society
https://www.readbyqxmd.com/read/28795843/intraoperative-imaging-techniques-for-glioma-surgery
#3
Tomas Garzon-Muvdi, Carmen Kut, Xingde Li, Kaisorn L Chaichana
Gliomas are CNS neoplasms that infiltrate the surrounding brain parenchyma, complicating their treatment. Tools that increase extent of resection while preventing neurological deficit are essential to improve prognosis of patients diagnosed with gliomas. Tools such as intraoperative MRI, ultrasound and fluorescence-guided microsurgery have been used in the surgical resection of CNS gliomas with the goal of maximizing extent of resection to improve patient outcomes. In addition, emerging experimental techniques, for example, optical coherence tomography and Raman spectroscopy are promising techniques which could 1 day add to the increasing armamentarium used in the surgical resection of CNS gliomas...
August 10, 2017: Future Oncology
https://www.readbyqxmd.com/read/28759636/ala-ppix-mediated-photodynamic-therapy-of-malignant-gliomas-augmented-by-hypothermia
#4
Carl J Fisher, Carolyn Niu, Warren Foltz, Yonghong Chen, Elena Sidorova-Darmos, James H Eubanks, Lothar Lilge
BACKGROUND: Malignant gliomas are highly invasive, difficult to treat, and account for 2% of cancer deaths worldwide. Glioblastoma Multiforme (GBM) comprises the most common and aggressive intracranial tumor. The study hypothesis is to investigate the modification of Photodynamic Therapy (PDT) efficacy by mild hypothermia leads to increased glioma cell kill while protecting normal neuronal structures. METHODS: Photosensitizer accumulation and PDT efficacy in vitro were quantified in various glioma cell lines, primary rat neurons, and astrocytes...
2017: PloS One
https://www.readbyqxmd.com/read/28738091/the-second-window-icg-technique-demonstrates-a-broad-plateau-period-for-near-infrared-fluorescence-tumor-contrast-in-glioblastoma
#5
Ryan Zeh, Saad Sheikh, Leilei Xia, John Pierce, Andrew Newton, Jarrod Predina, Steve Cho, MacLean Nasrallah, Sunil Singhal, Jay Dorsey, John Y K Lee
INTRODUCTION: Fluorescence-guided surgery has emerged as a powerful tool to detect, localize and resect tumors in the operative setting. Our laboratory has pioneered a novel way to administer an FDA-approved near-infrared (NIR) contrast agent to help surgeons with this task. This technique, coined Second Window ICG, exploits the natural permeability of tumor vasculature and its poor clearance to deliver high doses of indocyanine green (ICG) to tumors. This technique differs substantially from established ICG video angiography techniques that visualize ICG within minutes of injection...
2017: PloS One
https://www.readbyqxmd.com/read/28680735/pilocytic-astrocytoma-a-rare-presentation-as-intraventricular-tumor
#6
Sidra Sattar, Naveed Z Akhunzada, Gohar Javed, Zeeshan Uddin, Yasir A Khan
BACKGROUND: Pilocytic astrocytoma (PA) is the most prevalent central nervous system (CNS) tumor in pediatric population and accounts for an approximate of 5-6% of all gliomas. This neoplasm can occur at all levels of the neuraxis, with majority (67%) arising in the cerebellum and optic pathway. PAs are World Health Organization Grade I tumors and are the most benign of all astrocytomas characterized by an excellent prognosis. Other differentials include subependymal giant cell astrocytoma (SEGA), ependymoma, meningioma, and low-grade gliomas such as pilocytic or diffuse astrocytoma; calcification is more commonly regarded as a feature of benign or slow-growing tumors...
2017: Surgical Neurology International
https://www.readbyqxmd.com/read/28666648/morning-glory-disc-anomaly-with-an-ipsilateral-enlargement-of-the-optic-nerve-pathway
#7
D Thoma, I Nijs, P Demaerel, I Casteels
PURPOSE: To report three patients with an unilateral morning glory disc anomaly in association with an ipsilateral mild thickening of the optic nerve. METHODS: Three children with a morning glory disc anomaly underwent a magnetic resonance imaging (MRI) of the brain. Ophthalmological, genetic and MRI findings at follow-up are reported. A literature search on the association of morning glory anomaly in association with optic nerve glioma is reported.(1) RESULTS: Three children with an unilateral morning glory anomaly and ipsilateral poor visual acuity were found to have an ipsilateral mild optic nerve enlargement on brain MRI...
May 19, 2017: European Journal of Paediatric Neurology: EJPN
https://www.readbyqxmd.com/read/28665248/wavelength-specific-lighted-suction-instrument-for-5-aminolevulinic-acid-fluorescence-guided-resection-of-deep-seated-malignant-glioma-technical-note
#8
Ramin A Morshed, Seunggu J Han, Darryl Lau, Mitchel S Berger
Surgery guided by 5-aminolevulinic acid (ALA) fluorescence has become a valuable adjunct in the resection of malignant intracranial gliomas. Furthermore, the fluorescence intensity of biopsied areas of a resection cavity correlates with histological identification of tumor cells. However, in the case of lesions deep within a resection cavity, light penetration may be suboptimal, resulting in less excitation of 5-ALA metabolites, leading to decreased fluorescence emission. To address this obstacle, the authors report on the use of a 400-nm wavelength fiber-optic lighted suction instrument that can be used both during resection of a tumor and to provide direct light to deeper areas of a resection cavity...
June 30, 2017: Journal of Neurosurgery
https://www.readbyqxmd.com/read/28658822/recurrent-optic-nerve-pilocytic-astrocytoma-a-rare-case
#9
Sudha Girish Menon, Vuppalapati Nishant Janardhana Raju, Sulatha V Bhandary, Krishna Rao Addoor
Pilocytic astrocytoma is a low grade glioma that affects mostly children and young adults and can occur anywhere in the central nervous system. Pilocytic astrocytoma of the optic nerve is an equally indolent subtype that is often associated with Neurofibromatosis Type I (NFI). A 40-year-old male presented with left sided axial proptosis and exposure keratopathy. MRI revealed a mass in left proximal orbit, extending posteriorly abutting the chiasma and the right optic nerve on MRI. Enucleation of the left eye along with near total excision of intracranial part of the mass was performed...
May 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28645590/endoscopic-supracerebellar-transtentorial-approach-to-the-atrium-of-the-lateral-ventricle-preliminary-surgical-and-optical-considerations
#10
Tao Xie, Linjun Zhou, Xiaobiao Zhang, Wei Sun, Hailin Ding, Tengfei Liu, Ye Gu, Chongjing Sun, Fan Hu, Wei Zhu
OBJECTIVE: To report the operative techniques of the endoscopic supracerebellar transtentorial approach (ESTA) to the atrium of the lateral ventricle, especially focusing on the role of the endoscope and analyzing optically related issues. METHODS: A retrospective data review was performed on five patients with lesions in the atrium of the lateral ventricle undergoing the ESTA. The patients were positioned in the three-quarters prone position, and a paramidline linear incision was used...
June 20, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/28640700/cns-tumors-in-neurofibromatosis
#11
Jian Campian, David H Gutmann
Neurofibromatosis (NF) encompasses a group of distinct genetic disorders in which affected children and adults are prone to the development of benign and malignant tumors of the nervous system. The purpose of this review is to discuss the spectrum of CNS tumors arising in individuals with NF type 1 (NF1) and NF type 2 (NF2), their pathogenic etiologies, and the rational treatment options for people with these neoplasms. This article is a review of preclinical and clinical data focused on the treatment of the most common CNS tumors encountered in children and adults with NF1 and NF2...
July 20, 2017: Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology
https://www.readbyqxmd.com/read/28633092/epilepsy-in-neurofibromatosis-type-1
#12
Anthony Pecoraro, Eric Arehart, William Gallentine, Rodney Radtke, Edward Smith, Carolyn Pizoli, Sujay Kansagra, Elie Abdelnour, Roger McLendon, Mohamad A Mikati
OBJECTIVES: To describe the characteristics of epilepsy in patients with Neurofibromatosis type 1 (NF1). METHODS: Analysis of a cohort of consecutive NF1 patients seen in our NF1 clinic during a three-year period. RESULTS: Of the 184 NF1 patients seen during that period, 26 had epilepsy and three had febrile seizures. Of the 26, 17 (65%) had localization-related epilepsy, seven of whom (41%) were drug resistant. Six (23%) had apparently primary generalized epilepsy (0/6 drug resistant), two (8%) Lennox-Gastaut syndrome, and one (4%) West syndrome (all three were drug-resistant)...
June 17, 2017: Epilepsy & Behavior: E&B
https://www.readbyqxmd.com/read/28631895/growth-hormone-excess-in-children-with-neurofibromatosis-type-1-and-optic-glioma
#13
Paola Cambiaso, Stefania Galassi, Melania Palmiero, Angela Mastronuzzi, Francesca Del Bufalo, Rossella Capolino, Antonella Cacchione, Paola S Buonuomo, Michaela V Gonfiantini, Andrea Bartuli, Marco Cappa, Marina Macchiaiolo
In children with neurofibromatosis type 1 (NF1) and optic pathways glioma (OPG), growth hormone (GH) excess has been rarely reported and mainly associated to central precocious puberty. The aim of our study is to evaluate the prevalence of GH excess, the association with central precocious puberty, the relation with tumor site and the evolution over time in a large cohort of children with NF1 and OPG. Sixty-four NF1 children with OPG were evaluated. Patients with stature and/or height velocity >2 SD for age were studied for GH secretion...
June 20, 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28620004/cancer-and-central-nervous-system-tumor-surveillance-in-pediatric-neurofibromatosis-1
#14
REVIEW
D Gareth R Evans, Hector Salvador, Vivian Y Chang, Ayelet Erez, Stephan D Voss, Kami Wolfe Schneider, Hamish S Scott, Sharon E Plon, Uri Tabori
Although the neurofibromatoses consist of at least three autosomal dominantly inherited disorders, neurofibromatosis 1 (NF1), neurofibromatosis 2 (NF2), and schwannomatosis, NF1 represents a multisystem pleiotropic condition very different from the other two. NF1 is a genetic syndrome first manifesting in childhood; affecting multiple organs, childhood development, and neurocognitive status; and presenting the clinician with often complex management decisions that require a multidisciplinary approach. Molecular genetic testing (see article for detailed discussion) is recommended to confirm NF1, particularly in children fulfilling only pigmentary features of the diagnostic criteria...
June 15, 2017: Clinical Cancer Research: An Official Journal of the American Association for Cancer Research
https://www.readbyqxmd.com/read/28606795/endothelium-independent-primitive-myxoid-vascularization-creates-invertebrate-like-channels-to-maintain-blood-supply-in-optic-gliomas
#15
Matija Snuderl, Guoan Zhang, Pamela Wu, Tara S Jennings, Seema Shroff, Valerio Ortenzi, Rajan Jain, Benjamin Cohen, Jason J Reidy, Mitchell S Dushay, Jeffrey H Wisoff, David H Harter, Matthias A Karajannis, David Fenyo, Thomas A Neubert, David Zagzag
Optic gliomas are brain tumors characterized by slow growth, progressive loss of vision, and limited therapeutic options. Optic gliomas contain various amounts of myxoid matrix, which can represent most of the tumor mass. We sought to investigate biological function and protein structure of the myxoid matrix in optic gliomas to identify novel therapeutic targets. We reviewed histological features and clinical imaging properties, analyzed vasculature by immunohistochemistry and electron microscopy, and performed liquid chromatography-mass spectrometry on optic gliomas, which varied in the amount of myxoid matrix...
August 2017: American Journal of Pathology
https://www.readbyqxmd.com/read/28593402/clinical-presentation-and-prognostic-indicators-in-100-adults-and-children-with-neurofibromatosis-1-associated-non-optic-pathway-brain-gliomas
#16
Susan Byrne, Steve Connor, Karine Lascelles, Ata Siddiqui, Darren Hargrave, Rosalie E Ferner
Type 1 Neurofibromatosis (NF1) is a common autosomal dominant condition, with a major impact on the nervous system, eye, bone, and skin, and a predisposition to malignancy. At present it is not possible to predict clinically or on imaging, whether a brain tumour will remain indolent or undergo high-grade change. There are no consensus guidelines on the follow-up of non-optic pathway glioma (non-OPG) tumours in NF1. One hundred patients from the National NF1 Service with generalised NF1 and a diagnosis of non-OPG glioma were followed up for a median time of 63 months after glioma detection...
July 2017: Journal of Neuro-oncology
https://www.readbyqxmd.com/read/28587881/mr-imaging-findings-in-children-with-spasmus-nutans
#17
Meredith Bowen, Jason Peragallo, Stephen F Kralik, Andrea Poretti, Thierry A G M Huisman, Bruno P Soares
BACKGROUND: Spasmus Nutans (SN) is a rare pediatric ophthalmologic syndrome of nystagmus, head bobbing and abnormal head positioning. Historically, SN has been associated with underlying optic pathway gliomas (OPG); however, evidence of this is association is based primarily on a small number of isolated case reports. Prior retrospective analyses have found the rate of OPG to be less than 2%, but only intermittently utilized neuroimaging with CT, which has limited sensitivity for detection of small lesions in the optic pathway...
June 3, 2017: Journal of AAPOS: the Official Publication of the American Association for Pediatric Ophthalmology and Strabismus
https://www.readbyqxmd.com/read/28587872/validation-of-an-automated-tractography-method-for-the-optic-radiations-as-a-biomarker-of-visual-acuity-in-neurofibromatosis-associated-optic-pathway-glioma
#18
REVIEW
Peter de Blank, Michael J Fisher, Haley Gittleman, Jill S Barnholtz-Sloan, Chaitra Badve, Jeffrey I Berman
INTRODUCTION: Fractional anisotropy (FA) of the optic radiations has been associated with vision deficit in multiple intrinsic brain pathologies including NF1 associated optic pathway glioma, but hand-drawn regions of interest used in previous tractography methods limit consistency of this potential biomarker. We created an automated method to identify white matter tracts in the optic radiations and compared this method to previously reported hand-drawn tractography. METHOD: Automated tractography of the optic radiation using probabilistic streamline fiber tracking between the lateral geniculate nucleus of the thalamus and the occipital cortex was compared to the hand-drawn method between regions of interest posterior to Meyer's loop and anterior to tract branching near the calcarine cortex...
June 3, 2017: Experimental Neurology
https://www.readbyqxmd.com/read/28577031/natural-history-of-optic-pathway-gliomas-in-a-cohort-of-unselected-patients-affected-by-neurofibromatosis-1
#19
Eva Trevisson, Matteo Cassina, Enrico Opocher, Virginia Vicenzi, Marta Lucchetta, Raffaele Parrozzani, Giacomo Miglionico, Rodica Mardari, Elisabetta Viscardi, Edoardo Midena, Maurizio Clementi
Optic pathway glioma (OPG) represents the most common central nervous system tumor in children with Neurofibromatosis type-1 (NF1). Although overall survival is usually good, no clear prognostic factors have been identified so far. We assessed the natural history of OPG in a cohort of unselected patients affected by NF1. We retrospectively evaluated 414 consecutive patients affected by NF1 and referred to our NF1 clinic before age 6. Average follow-up was 11.9 years: 52 out of 414 patients had OPG with a total cumulative incidence of 15...
June 2, 2017: Journal of Neuro-oncology
https://www.readbyqxmd.com/read/28575296/letter-intraoperative-near-infrared-optical-imaging-can-localize-gadolinium-enhancing-gliomas-during-surgery
#20
Marc Zanello, Alexandre Roux, Johan Pallud
No abstract text is available yet for this article.
June 1, 2017: Neurosurgery
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