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Leptomeningeal disease

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https://www.readbyqxmd.com/read/29785266/atypical-teratoid-rhabdoid-tumor-in-a-65-year-old-man-presenting-with-disseminated-leptomeningeal-disease-a-case-report-and-review-of-the-literature
#1
Marc-Alain Babi, Peter Fecci, Matthew Luedke, Olinda Pineda, Yasmin Ali O'Keefe
Central nervous system atypical teratoid rhabdoid tumors are very rare aggressive tumor of childhood, primarily occurring at age of less than 3 years old. The prognosis of these tumors is very poor, with a reported median survival of 6-12 months in most cases. Treatment typically consists of aggressive chemotherapy and radiotherapy. We present the case of a 65-year-old man who presented with progressive encephalopathy and change in personality over 3 months period. The patient had further accelerated decline over 3 weeks...
2018: SAGE Open Medical Case Reports
https://www.readbyqxmd.com/read/29775413/human-cerebral-collateral-arteriole-function-in-normal-cognition-mild-cognitive-impairment-and-dementia-subjects
#2
Raymond Q Migrino, Seth Truran, Nina Karamanova, Geidy Serrano, Calvin Madrigal, Hannah A Davies, Jillian Madine, Peter Reaven, Thomas G Beach
Clinical and preclinical studies suggest a link between cardiovascular disease and dementia disorders but the role of collateral brain circulation in cognitive dysfunction remains unknown. We aim to test the hypothesis that leptomeningeal arteriole (LMA) function and response to metabolic stressors differ among dementia, mild cognitive impairment (MCI) and normal cognition (CN) subjects. Following rapid autopsy, LMA were isolated from CN (N=10), MCI (N=12) or dementia subjects (N=42, Alzheimer's disease [AD], vascular dementia [VaD], or Other Dementia) and endothelial and smooth muscle-dependent function were measured at baseline and following exposure to β-amyloid (Aβ42 2 μM), palmitic acid (PA 150 μM) or medin (5μM) and compared...
May 18, 2018: American Journal of Physiology. Heart and Circulatory Physiology
https://www.readbyqxmd.com/read/29764408/congenital-rhabdomyosarcoma-a-different-clinical-presentation-in-two-cases
#3
Ida Russo, Virginia Di Paolo, Carmelo Gurnari, Angela Mastronuzzi, Francesca Del Bufalo, Pier Luigi Di Paolo, Angela Di Giannatale, Renata Boldrini, Giuseppe Maria Milano
BACKGROUND: Rhabdomyosarcoma (RMS), one of the most common soft tissue sarcomas of childhood, is very rare in the neonatal period (0.4-2% of cases). In order to gain a deeper understanding of this disease at such age, patient and tumor features, as well as treatment modality and outcome need to be reported. CASE PRESENTATION: We describe two cases with congenital RMS treated at Bambino Gesù Children's Hospital between 2000 and 2016. They represent only 2.24% of all RMS patients diagnosed during that period in our Institution; this data is in agreement with the incidence reported in the literature...
May 15, 2018: BMC Pediatrics
https://www.readbyqxmd.com/read/29752148/malignant-primary-diffuse-leptomeningeal-gliomatosis-with-histone-h3-3-k27m-mutation
#4
C Champeaux, A Drier, B Devaux, A Tauziède-Espariat
INTRODUCTION: Malignant primary diffuse leptomeningeal gliomatosis (MPDLG) are rare central nervous system neoplasms associated with a poor outcome. CASE REPORT: We report the case of a 40-year-old woman who presented with unusual worsening of bilateral sciatica, headaches, diplopia and a left proptosis. MRI of the head and spine showed multiple leptomeningeal lesions with no intra parenchymal involvement. The search for a primary tumor was negative. An open surgical biopsy of the prominent intradural lumbar tumor was performed within a week...
May 8, 2018: Neuro-Chirurgie
https://www.readbyqxmd.com/read/29749569/postoperative-hypofractionated-stereotactic-brain-radiation-hsrt-for-resected-brain-metastases-improved-local-control-with-higher-bed-10
#5
Aryavarta M S Kumar, Jonathan Miller, Seth A Hoffer, David B Mansur, Michael Coffey, Simon S Lo, Andrew E Sloan, Mitchell Machtay
INTRODUCTION: HSRT directed to large surgical beds in patients with resected brain metastases improves local control while sparing patients the toxicity associated with whole brain radiation. We review our institutional series to determine factors predictive of local failure. METHODS: In a total of 39 consecutive patients with brain metastases treated from August 2011 to August 2016, 43 surgical beds were treated with HSRT in three or five fractions. All treatments were completed on a robotic radiosurgery platform using the 6D Skull tracking system...
May 10, 2018: Journal of Neuro-oncology
https://www.readbyqxmd.com/read/29737389/4d-asl-based-mr-angiography-for-visualization-of-distal-arteries-and-leptomeningeal-collateral-vessels-in-moyamoya-disease-a-comparison-of-techniques
#6
Osamu Togao, Akio Hiwatashi, Makoto Obara, Koji Yamashita, Daichi Momosaka, Ataru Nishimura, Koichi Arimura, Nobuhiro Hata, Koji Yoshimoto, Koji Iihara, Marc Van Cauteren, Hiroshi Honda
OBJECTIVES: To evaluate the performance of four-dimensional pseudo-continuous arterial spin labeling (4D-pCASL)-based angiography using CENTRA-keyhole and view sharing (4D-PACK) in the visualization of flow dynamics in distal cerebral arteries and leptomeningeal anastomosis (LMA) collaterals in moyamoya disease in comparison with contrast inherent inflow-enhanced multiphase angiography (CINEMA), with reference to digital subtraction angiography (DSA). METHODS: Thirty-two cerebral hemispheres from 19 patients with moyamoya disease (mean age, 29...
May 8, 2018: European Radiology
https://www.readbyqxmd.com/read/29736618/leptomeningeal-form-of-immunoglobulin-g4-related-hypertrophic-meningitis-with-perivascular-spread-a-case-report-and-review-of-the-literature
#7
Jasmina Boban, Selin Ardalı, Majda M Thurnher
PURPOSE: Immunoglobulin G4 (IgG4)-related disease represents a spectrum of fibro-inflammatory disorders that affects various organ systems, including the central nervous system. METHODS: Here we present the case of lgG4-related hypertrophic meningitis with exclusively leptomeningeal involvement and spread via perivascular spaces. RESULTS: A 58-year-old male patient presented with complex partial seizures. Initial computed tomography examination showed left frontal sulcal hyperdensity...
May 7, 2018: Neuroradiology
https://www.readbyqxmd.com/read/29735452/-characteristics-of-collateral-circulation-in-adult-moyamoya-disease-based-on-modified-suzuki-staging
#8
Qing-Shun Zhao, Gang Wang, Hao-Jiang Xiao, Wen-Feng Feng, Guo-Zhong Zhang, Ming-Zhou Li, Yong-Hong Liao, Yun-Yu Wen, Song-Tao Qi
OBJECTIVE: To investigate the characteristics of collateral circulation in adult moyamoya disease (MMD). METHODS: The clinical data were collected from all adult patients with MMD undergoing digital subtractive angiography (DSA) in our department from 2006 to 2016. Based on the imaging findings, the patients were divided into ischemia group and bleeding group. A double-blind analysis was conducted of the CT or magnetic resonance imaging findings and the severity of the disease was graded using the modified Suzuki score (mSS)...
April 20, 2018: Nan Fang Yi Ke da Xue Xue Bao, Journal of Southern Medical University
https://www.readbyqxmd.com/read/29725622/somatic-gnaq-mutation-in-the-forme-fruste-of-sturge-weber-syndrome
#9
Michael S Hildebrand, A Simon Harvey, Stephen Malone, John A Damiano, Hongdo Do, Zimeng Ye, Lara McQuillan, Wirginia Maixner, Renate Kalnins, Bernadette Nolan, Martin Wood, Ezgi Ozturk, Nigel C Jones, Greta Gillies, Kate Pope, Paul J Lockhart, Alexander Dobrovic, Richard J Leventer, Ingrid E Scheffer, Samuel F Berkovic
Objective: To determine whether the GNAQ R183Q mutation is present in the forme fruste cases of Sturge-Weber syndrome (SWS) to establish a definitive molecular diagnosis. Methods: We used sensitive droplet digital PCR (ddPCR) to detect and quantify the GNAQ mutation in tissues from epilepsy surgery in 4 patients with leptomeningeal angiomatosis; none had ocular or cutaneous manifestations. Results: Low levels of the GNAQ mutation were detected in the brain tissue of all 4 cases-ranging from 0...
June 2018: Neurology. Genetics
https://www.readbyqxmd.com/read/29725432/a-nomogram-for-predicting-survival-in-patients-with-breast-cancer-brain-metastasis
#10
Zhou Huang, Bing Sun, Shikai Wu, Xiangying Meng, Yang Cong, Ge Shen, Santai Song
Brain metastasis (BM) is common in patients with breast cancer. Predicting patient survival is critical for the clinical management of breast cancer brain metastasis (BCBM). The present study was designed to develop and evaluate a prognostic model for patients with newly diagnosed BCBM. Based on the clinical data of patients with BCBM treated in the Affiliated Hospital of Academy of Military Medical Sciences (Beijing, China) between 2002 and 2014, a nomogram was developed to predict survival using proportional hazards regression analysis...
May 2018: Oncology Letters
https://www.readbyqxmd.com/read/29723263/borrelia-burgdorferi-adhere-to-blood-vessels-in-the-dura-mater-and-are-associated-with-increased-meningeal-t-cells-during-murine-disseminated-borreliosis
#11
Ali Divan, Timothy Casselli, S Anand Narayanan, Sanjib Mukherjee, David C Zawieja, John A Watt, Catherine A Brissette, M Karen Newell-Rogers
Borrelia burgdorferi, the causative agent of Lyme disease, is a vector-borne bacterial infection that is transmitted through the bite of an infected tick. If not treated with antibiotics during the early stages of infection, disseminated infection can spread to the central nervous system (CNS). In non-human primates (NHPs) it has been demonstrated that the leptomeninges are among the tissues colonized by B. burgdorferi spirochetes. Although the NHP model parallels aspects of human borreliosis, a small rodent model would be ideal to study the trafficking of spirochetes and immune cells into the CNS...
2018: PloS One
https://www.readbyqxmd.com/read/29723241/intra-operative-cerebrospinal-fluid-sampling-versus-post-operative-lumbar-puncture-for-detection-of-leptomeningeal-disease-in-malignant-paediatric-brain-tumours
#12
Sharon Y Y Low, Chen Min Wei, Kenneth T E Chang, Chan Yiong Huak, Ng Lee Ping, Seow Wan Tew, David C Y Low
INTRODUCTION: Leptomeningeal disease is a feared sequelae of malignant paediatric brain tumours. Current methods for its detection is the combined use of cranio-spinal MRI, and CSF cytology from a post-operative lumbar puncture. In this study, the authors hypothesize that CSF taken at the start of surgery, either from an external ventricular drain or neuroendoscope will have equal sensitivity for positive tumour cells, in comparison to lumbar puncture. Secondary hypotheses include positive correlation between CSF cytology and MRI findings of LMD...
2018: PloS One
https://www.readbyqxmd.com/read/29722740/rheumatoid-meningitis-a-case-review
#13
Angela M Parsons, Leslie A Zuniga, Joseph M Hoxworth, Mark Lyons, Fawad Aslam, Brent P Goodman
INTRODUCTION: Rheumatoid meningitis (RM) is a rare complication of rheumatoid arthritis (RA) and has a high mortality rate. It can present as a first diagnosis of RA, in long-standing disease, or in active or well-controlled disease. Neurological manifestations vary widely. CASE REPORT: A patient with a 30-year history of RA, well controlled with methotrexate therapy, presented with new-onset seizures. Magnetic resonance imaging showed leptomeningeal and pachymeningeal enhancement...
May 2018: Neurologist
https://www.readbyqxmd.com/read/29706150/neurosyphilis-mighty-imitator-forays-with-benign-presentation-and-unique-neuroimaging-findings
#14
Harmanpreet Tiwana, Aiesha Ahmed
Background: Common causes of temporal lobe hyper intensities are central nervous system infections like herpes simplex encephalitis, Lyme disease, limbic encephalitis and vascular pathology like Cerebral Autosomal Dominant Arteriopathy with Subcortical infarcts and Leukoencephalopathy. Methods: Personal assessment, laboratory data analysis and neuroimaging for the patient who was admitted to a central Pennsylvania tertiary care referral centre were conducted. Results: A 52-year-old male presented with a 1-year history of diffuse dysesthesia in upper and lower extremities with associated intermittent headaches and neck stiffness...
April 30, 2018: Sexual Health
https://www.readbyqxmd.com/read/29700687/medical-treatment-options-for-patients-with-epidermal-growth-factor-receptor-mutation-positive-non-small-cell-lung-cancer-suffering-from-brain-metastases-and-or-leptomeningeal-disease
#15
Maximilian Hochmair
Brain metastases and/or leptomeningeal disease (LMD) with associated central nervous system (CNS) metastases are known complications of advanced epidermal growth factor receptor (EGFR) mutation-positive non-small cell lung cancer (NSCLC). It is important, therefore, to assess the activity of EGFR tyrosine kinase inhibitors (TKIs) versus such CNS complications. This review explores the literature reporting the intracranial activity of EGFR TKIs, and finds that there is evidence for varying efficacy of the approved agents, erlotinib, gefitinib, afatinib, and osimertinib in patients with CNS metastases...
April 27, 2018: Targeted Oncology
https://www.readbyqxmd.com/read/29695592/clinical-spectrum-and-prognostic-value-of-cns-mog-autoimmunity-in-adults-the-mogador-study
#16
Alvaro Cobo-Calvo, Anne Ruiz, Elisabeth Maillart, Bertrand Audoin, Helene Zephir, Bertrand Bourre, Jonathan Ciron, Nicolas Collongues, David Brassat, Francois Cotton, Caroline Papeix, Francoise Durand-Dubief, David Laplaud, Romain Deschamps, Mikaël Cohen, Damien Biotti, Xavier Ayrignac, Caroline Tilikete, Eric Thouvenot, Bruno Brochet, Cecile Dulau, Thibault Moreau, Ayman Tourbah, Pierre Lebranchu, Laure Michel, Christine Lebrun-Frenay, Alexis Montcuquet, Guillaume Mathey, Marc Debouverie, Jean Pelletier, Pierre Labauge, Nathalie Derache, Marc Coustans, Fabien Rollot, Jérôme De Seze, Sandra Vukusic, Romain Marignier
OBJECTIVE: To describe clinical and radiologic features associated with myelin oligodendrocyte glycoprotein antibodies (MOG-Ab) in a large French nationwide adult cohort, to assess baseline prognostic features of MOG-Ab-associated diseases after a first acute demyelinating syndrome, and to evaluate the clinical value of MOG-Ab longitudinal analysis. METHODS: Clinical data were obtained from 197 MOG-Ab-positive patients ≥18 years of age. Complete imaging data were available in 108, and 54 serum samples were eligible for longitudinal evaluation...
April 25, 2018: Neurology
https://www.readbyqxmd.com/read/29663721/alveolar-rhabdomyosarcoma-in-the-cerebrospinal-fluid
#17
Janice S Ahn, Melina Flanagan
Alveolar rhabdomyosarcoma (RMS) is an aggressive soft tissue mass demonstrating rapid growth, dissemination, and leptomeningeal spread. Primary diagnosis is usually established by core biopsy. In rare cases, cytopathologic evaluation is indicated to identify recurrent or metastatic disease. We present a case of a 24-year-old man with a previously diagnosed alveolar RMS of the foot who presented to our institution with back pain. A lumbar puncture was performed and the cerebrospinal fluid (CSF) showed atypical cells demonstrating nuclear enlargement, eccentricity, binucleation, and frequent karyorrhexis...
April 16, 2018: Diagnostic Cytopathology
https://www.readbyqxmd.com/read/29652210/idiopathic-central-nervous-system-inflammatory-disease-in-the-setting-of-hla-b27-uveitis
#18
Eric L Crowell, Margaret L Pfeiffer, Ankur A Kamdar, Mary K Koenig, Susan E Wittenberg, Emilio P Supsupin, Ore-Ofe O Adesina
PURPOSE: The purpose of the article is to describe a novel case of idiopathic central nervous system inflammatory disease with bilateral human leukocyte antigen (HLA)-B27-positive anterior uveitis. METHODS/RESULTS: A 15-year-old African American boy with bilateral HLA-B27-positive anterior uveitis controlled with topical and oral steroids for 8 months acutely developed headaches, left eyelid ptosis, and binocular diplopia. Imaging showed lesions in the right midbrain, superior colliculus, cerebellar peduncles, and cerebellar vermis and leptomeningeal enhancement along the vermian foliae...
April 13, 2018: Ocular Immunology and Inflammation
https://www.readbyqxmd.com/read/29651369/unusual-relapse-of-chronic-lymphocytic-leukemia-after-remission
#19
Wajeeha Rizvi, Quoc Truong
Chronic lymphocytic leukemia (CLL) is the most prevalent leukemia with over 20,000 estimated cases in 2017. Leukemic involvement of the nervous system from CLL causing neurologic symptoms is reported in only about one percent of patients. Unfortunately, there is no current standard therapy for the treatment of CLL leptomeningeal disease. In this case, we discuss an unusual presentation of CLL leptomeningeal disease misdiagnosed as chronic rebound headache. A 61-year-old female was diagnosed with Rai stage I CLL in 2002...
February 9, 2018: Curēus
https://www.readbyqxmd.com/read/29650781/anatomic-and-angiographic-analyses-of-ophthalmic-artery-collaterals-in-moyamoya-disease
#20
T Robert, G Cicciò, P Sylvestre, A Chiappini, A G Weil, S Smajda, C Chaalala, R Blanc, M Reinert, M Piotin, M W Bojanowski
BACKGROUND AND PURPOSE: Moyamoya disease is a progressive neurovascular pathology defined by steno-occlusive disease of the distal internal carotid artery and associated with the development of compensatory vascular collaterals. The etiology and exact anatomy of vascular collaterals have not been extensively studied. The aim of this study was to describe the anatomy of collaterals developed between the ophthalmic artery and the anterior cerebral artery in a Moyamoya population. MATERIALS AND METHODS: All patients treated for Moyamoya disease from 2004 to 2016 in 4 neurosurgical centers with available cerebral digital subtraction angiography were included...
April 12, 2018: AJNR. American Journal of Neuroradiology
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