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acute disseminated encephalomyelitis

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https://www.readbyqxmd.com/read/29445493/acute-disseminated-encephalomyelitis-following-influenza-a-pneumonia
#1
Babikir Kheiri, Emad Abu Sitta, Anas Salih, Mohammed Al Qasmi, Ghassan Bachuwa
Acute disseminated encephalomyelitis (ADEM) is an autoimmune demyelinating disorder of the central nervous system and can present following influenza A infections as multifocal neurological deficits. ADEM remains a challenging diagnosis, and high clinical suspicious coupled with laboratory investigations and neuroimaging is required to exclude other primary and secondary demyelinating disorders.
February 2018: Clinical Case Reports
https://www.readbyqxmd.com/read/29443442/paediatric-adem-followed-by-optic-neuritis-disease-course-treatment-response-and-outcome
#2
Y Y M Wong, Y Hacohen, T Armangue, E Wassmer, H Verhelst, C Hemingway, E D van Pelt, C E Catsman-Berrevoets, R Q Hintzen, K Deiva, M J Lim, K Rostásy, R F Neuteboom
BACKGROUND: Acute disseminated encephalomyelitis, followed by optic neuritis (ADEM-ON) is a rare demyelinating syndrome different than MS and neuromyelitis optica spectrum disorder. We aim to describe the disease course, treatment response and outcome of these children. METHODS: children <18 years were identified from 6 countries of the EU Paediatric Demyelinating Disease Consortium. Patients fulfilled the diagnostic criteria for ADEM, followed by at least one ON...
February 14, 2018: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/29423614/mri-of-the-first-event-in-pediatric-acquired-demyelinating-syndromes-with-antibodies-to-myelin-oligodendrocyte-glycoprotein
#3
Matthias Baumann, Astrid Grams, Tanja Djurdjevic, Eva-Maria Wendel, Christian Lechner, Bettina Behring, Astrid Blaschek, Katharina Diepold, Astrid Eisenkölbl, Joel Fluss, Michael Karenfort, Johannes Koch, Bahadir Konuşkan, Steffen Leiz, Andreas Merkenschlager, Daniela Pohl, Mareike Schimmel, Charlotte Thiels, Barbara Kornek, Kathrin Schanda, Markus Reindl, Kevin Rostásy
Antibodies against the myelin oligodendrocyte glycoprotein (MOG-Ab) can be detected in various pediatric acquired demyelinating syndromes (ADS). Here, we analyze the spectrum of neuroradiologic findings in children with MOG-Ab and a first demyelinating event. The cerebral and spinal MRI of 69 children with different ADS was assessed in regard to the distribution and characteristics of lesions. Children with acute disseminated encephalomyelitis (n = 36) or neuromyelitis optica spectrum disorder (n = 5) presented an imaging pattern characterized predominantly by poorly demarcated lesions with a wide supra- and infratentorial distribution...
February 8, 2018: Journal of Neurology
https://www.readbyqxmd.com/read/29404583/differences-in-the-reponses-to-apheresis-therapy-of-patients-with-3-histopathologically-classified-immunopathological-patterns-of-multiple-sclerosis
#4
Lidia Stork, David Ellenberger, Tim Beißbarth, Tim Friede, Claudia F Lucchinetti, Wolfgang Brück, Imke Metz
Importance: Plasma exchange and immunoadsorption are second-line apheresis therapies for patients experiencing multiple sclerosis relapses. Early active multiple sclerosis lesions can be classified into different histopathological patterns of demyelination. Pattern 1 and 2 lesions show T-cell- and macrophage-associated demyelination, and pattern 2 is selectively associated with immunoglobulin and complement deposits, suggesting a humoral immune response. Pattern 3 lesions show signs of oligodendrocyte degeneration...
February 5, 2018: JAMA Neurology
https://www.readbyqxmd.com/read/29305608/disease-course-and-treatment-responses-in-children-with-relapsing-myelin-oligodendrocyte-glycoprotein-antibody-associated-disease
#5
Yael Hacohen, Yu Yi Wong, Christian Lechner, Maciej Jurynczyk, Sukhvir Wright, Bahadir Konuskan, Judith Kalser, Anne Lise Poulat, Helene Maurey, Esther Ganelin-Cohen, Evangeline Wassmer, Chery Hemingway, Rob Forsyth, Eva Maria Hennes, M Isabel Leite, Olga Ciccarelli, Banu Anlar, Rogier Hintzen, Romain Marignier, Jacqueline Palace, Matthias Baumann, Kevin Rostásy, Rinze Neuteboom, Kumaran Deiva, Ming Lim
Importance: Myelin oligodendrocyte glycoprotein antibodies (MOG-Abs) are consistently identified in a range of demyelinating disorders in adults and children. Current therapeutic strategies are largely center specific, and no treatments have been formally evaluated. Objective: To examine the clinical phenotypes, treatment responses, and outcomes of children with relapsing MOG-Ab-associated disease. Design, Setting, and Participants: This study prospectively collected demographic, clinical, and radiologic data from 102 patients from 8 countries of the EU Paediatric Demyelinating Disease Consortium from January 1, 2014, through December 31, 2016...
January 5, 2018: JAMA Neurology
https://www.readbyqxmd.com/read/29288492/-leukodystrophy-like-phenotype-in-children-with-myelin-oligodendrocyte-glycoprotein-antibody-associated-disease
#6
Yael Hacohen, Thomas Rossor, Kshitij Mankad, Wk 'Kling' Chong, Andrew Lux, Evangeline Wassmer, Ming Lim, Frederik Barkhof, Olga Ciccarelli, Cheryl Hemingway
AIM: To review the demographics and clinical and paraclinical parameters of children with myelin oligodendrocyte glycoprotein (MOG) antibody-associated relapsing disease. METHOD: In this UK-based, multicentre study, 31 children with MOG antibody-associated relapsing disease were studied retrospectively. RESULTS: Of the 31 children studied, 14 presented with acute disseminated encephalomyelitis (ADEM); they were younger (mean 4.1y) than the remainder (mean 8...
December 30, 2017: Developmental Medicine and Child Neurology
https://www.readbyqxmd.com/read/29288471/chemokines-cxcl10-cxcl11-and-cxcl13-in-acute-disseminated-encephalomyelitis-non-polio-enterovirus-aseptic-meningitis-and-neuroborreliosis-cxcl10-as-initial-discriminator-in-diagnostic-algorithm
#7
Lorna Stemberger Maric, Snjezana Zidovec Lepej, Lana Gorenec, Ivana Grgic, Vladimir Trkulja, Oktavija Dakovic Rode, Srdan Roglic, Tonci Grmoja, Nina Barisic, Goran Tesovic
We investigated potential diagnostic usefulness of serum and cerebrospinal fluid (CSF) concentrations of chemokines CXCL10, CXCL11, and CXCL13 in pediatric patients with acute disseminated encephalomyelitis (ADEM) (n = 23), non-polio enterovirus aseptic meningitis (NPEV AM) (n = 20), and neuroborreliosis (NB) (n = 21) and children with acute infectious diseases with neurological symptoms but with excluded neuroinfection/neuroinflammation (controls, n = 20). CSF levels of CXCL10 and CXCL11 were higher in patients with NPEV AM than those in other children, and CXCL10 levels showed a high discriminative potential (area under the receiver operating characteristic curve, ROC, 0...
December 29, 2017: Neurological Sciences
https://www.readbyqxmd.com/read/29260024/unihemispheric-central-nervous-system-vasculitis
#8
Sikawat Thanaviratananich, Bashar Katirji
Patients with primary central nervous system vasculitis (PCNSV) usually manifest with multiple enhancing bilateral hemispheric lesions. We presented an extremely rare clinical course and follow-up of a patient with PCNSV affecting only a single (right) hemisphere. A 33-year-old previously healthy man presented with a left hand clonic seizure followed by a secondary generalized tonic-clonic seizure and dysarthria. MRI brain revealed multiple hyperintense lesions confined to only the right hemisphere with contrast enhancement, involving both white and grey matters...
June 2017: ENeurologicalSci
https://www.readbyqxmd.com/read/29245364/x-linked-charcot-marie-tooth-disease-with-gjb1-mutation-presenting-as-acute-disseminated-encephalomyelitis-like-illness-a-case-report
#9
Jin Kyu Kim, Seung-A Han, Sun Jun Kim
RATIONALE: Charcot-Marie-Tooth disease (CMT) is typically an autosomal dominant, inherited neuropathy, although there is a rare male X-linked CMT. Such patients show central nervous system (CNS) involvement in addition to peripheral neuropathy. Recently, we encountered a patient who presented with acute disseminated encephalomyelitis (ADEM)-like symptoms, but was later diagnosed as having X-linked CMT (CMTX) due to a mutation. PATIENT CONCERNS: A previously healthy 11-year-old boy was admitted for a sudden transient weakness of his left side extremities...
December 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29222716/childhood-acute-disseminated-encephalomyelitis-an-egyptian-pilot-study
#10
Hamed Ahmed Elkhayat, Omnia Fathy El-Rashidy, Iman Ali Elagouza, Raghda Zaitoun, Yasser Abdel Azeem Abbas
Describing the variable clinical features, laboratory findings, neuroimaging findings, and treatments given to children who presented with ADEM and following them up both clinically and radiologically. 21 patients were recruited: 14 new cases, and 7 old ones presenting over the preceding 5 years (retrospective review of existing data). 11 males and 10 females, with a mean age of 4.4 years ± 2.7 SD, were included. All new patients were subject to full history, examination and a panel of investigations including MRI of the brain...
December 8, 2017: Acta Neurologica Belgica
https://www.readbyqxmd.com/read/29215409/imaging-of-acquired-demyelinating-syndrome-with-18f-fdg-pet-ct
#11
Caroline Malo-Pion, Raymond Lambert, Jean-Claude Décarie, Sophie Turpin
Acquired demyelinating syndromes include acute disseminated encephalomyelitis, transverse myelitis and may progress to multiple sclerosis (MS). Acute disseminated encephalomyelitis is characterized by impairment of level of consciousness and multifocal neurological deficits and transverse myelitis by back pain, weakness and sphincter dysfunction. Only a few cases of acquired demyelinating syndrome have been imaged with F-FDG PET/CT. We present two such cases.
December 5, 2017: Clinical Nuclear Medicine
https://www.readbyqxmd.com/read/29184769/diffuse-glioblastoma-resembling-acute-hemorrhagic-leukoencephalitis
#12
Carla Schettino, Ferdinando Caranci, Giacomo Lus, Elisabetta Signoriello, Marica Eoli, Elena Anghileri, Bianca Pollo, Mariarosa A B Melone, Giuseppe Di Iorio, Gaetano Finocchiaro, Lorenzo Ugga, Enrico Tedeschi
We report the case of a young man with sudden onset of diplopia after an upper respiratory tract infection. Based on the first radiological findings acute hemorrhagic leukoencephalitis, a variant of acute disseminated encephalomyelitis, was suspected and treatment with high dose intravenous dexamethasone was started but it was stopped for intolerance. The patient clinically worsened, developing gait instability, ataxia and ophthalmoplegia; brain MRI performed 20 days later showed severe progression of the disease with subependymal dissemination...
October 2017: Quantitative Imaging in Medicine and Surgery
https://www.readbyqxmd.com/read/29167056/diffusion-tensor-imaging-as-a-biomarker-to-differentiate-acute-disseminated-encephalomyelitis-from-multiple-sclerosis-at-first-demyelination
#13
Wint Yan Aung, Parinaz Massoumzadeh, Safa Najmi, Amber Salter, Jodi Heaps, Tammie L S Benzinger, Soe Mar
BACKGROUND: There are no clinical features or biomarkers that can reliably differentiate acute disseminated encephalomyelitis from multiple sclerosis at the first demyelination attack. Consequently, a final diagnosis is sometimes delayed by months and years of follow-up. Early treatment for multiple sclerosis is recommended to reduce long-term disability. Therefore, we intend to explore neuroimaging biomarkers that can reliably distinguish between the two diagnoses. METHODS: We reviewed prospectively collected clinical, standard MRI and diffusion tensor imaging data of 12 pediatric patients who presented with acute demyelination with and without encephalopathy...
September 28, 2017: Pediatric Neurology
https://www.readbyqxmd.com/read/29143589/biomarkers-in-the-evolution-of-multiple-sclerosis
#14
Thomas Berger
Nonimaging biomarkers can be applied in differential diagnosis, evaluation of disease progression and therapy monitoring of multiple sclerosis (MS). Presence of oligoclonal IgG bands in cerebrospinal fluid is a diagnostic element and a negative predictor of MS evolution. AQP4 antibodies are pathogenic and diagnostic for neuromyelitis optica spectrum disorder. Antibodies to myelin oligodendrocyte glycoprotein develop in about 50% of predominantly pediatric patients with acute disseminated encephalomyelitis, but their possible role in pathogenesis is unknown...
November 2017: Neurodegenerative Disease Management
https://www.readbyqxmd.com/read/29142145/clinical-course-therapeutic-responses-and-outcomes-in-relapsing-mog-antibody-associated-demyelination
#15
Sudarshini Ramanathan, Shekeeb Mohammad, Esther Tantsis, Tina Kim Nguyen, Vera Merheb, Victor S C Fung, Owen Bruce White, Simon Broadley, Jeannette Lechner-Scott, Steve Vucic, Andrew P D Henderson, Michael Harry Barnett, Stephen W Reddel, Fabienne Brilot, Russell C Dale
OBJECTIVE: We characterised the clinical course, treatment and outcomes in 59 patients with relapsing myelin oligodendrocyte glycoprotein (MOG) antibody-associated demyelination. METHODS: We evaluated clinical phenotypes, annualised relapse rates (ARR) prior and on immunotherapy and Expanded Disability Status Scale (EDSS), in 218 demyelinating episodes from 33 paediatric and 26 adult patients. RESULTS: The most common initial presentation in the cohort was optic neuritis (ON) in 54% (bilateral (BON) 32%, unilateral (UON) 22%), followed by acute disseminated encephalomyelitis (ADEM) (20%), which occurred exclusively in children...
February 2018: Journal of Neurology, Neurosurgery, and Psychiatry
https://www.readbyqxmd.com/read/29141794/short-term-outcome-of-adem-results-from-a-retrospective-cohort-study-from-south-india
#16
Mary Iype, P A Mohammed Kunju, Geetha Saradakutty, T S Anish, Mini Sreedharan, Shahanaz M Ahamed
INTRODUCTION: Acute disseminated encephalomyelitis (ADEM), an immune mediated inflammatory disease is common in children. The profile and immediate outcome of children hospitalized with ADEM is scarce in the available literature. OBJECTIVES: We aimed to study the clinical profile of children with ADEM and to look for prognostic factors for outcome at discharge from hospital METHODS: We chose a retrospective cohort study of all children diagnosed with ADEM at our institution between January 2006 and December 2015, and they were evaluated, after excluding other diagnoses when they were summoned for a follow up visit...
November 2017: Multiple Sclerosis and related Disorders
https://www.readbyqxmd.com/read/29140242/guillain-barr%C3%A3-syndrome-acute-disseminated-encephalomyelitis-and-encephalitis-associated-with-zika-virus-infection-in-brazil-detection-of-viral-rna-and-isolation-of-virus-during-late-infection
#17
Maria Lucia Brito Ferreira, Carlos Alexandre Antunes de Brito, Álvaro José Porto Moreira, Maria Íris de Morais Machado, Adélia Henriques-Souza, Marli Tenório Cordeiro, Ernesto Torres de Azevedo Marques, Lindomar José Pena
Zika virus (ZIKV) emerged in Brazil in 2015, which was followed by an increase of Guillain-Barre Syndrome (GBS) cases. We report the epidemiological, clinical, and laboratory findings of the first six neurological cases associated with ZIKV in Brazil seen in a reference neurology hospital in Pernambuco, Brazil. In all cases, ZIKV was detected in serum and/or cerebrospinal fluid (CSF) samples. In this case series, four cases were defined as GBS, one as acute disseminated encephalomyelitis (ADEM) and the other as encephalitis...
November 2017: American Journal of Tropical Medicine and Hygiene
https://www.readbyqxmd.com/read/29136091/clinical-presentation-and-prognosis-in-mog-antibody-disease-a-uk-study
#18
Maciej Jurynczyk, Silvia Messina, Mark R Woodhall, Naheed Raza, Rosie Everett, Adriana Roca-Fernandez, George Tackley, Shahd Hamid, Angela Sheard, Gavin Reynolds, Saleel Chandratre, Cheryl Hemingway, Anu Jacob, Angela Vincent, M Isabel Leite, Patrick Waters, Jacqueline Palace
A condition associated with an autoantibody against MOG has been recently recognized as a new inflammatory disease of the central nervous system, but the disease course and disability outcomes are largely unknown. In this study we investigated clinical characteristics of MOG-antibody disease on a large cohort of patients from the UK. We obtained demographic and clinical data on 252 UK patients positive for serum immunoglobulin G1 MOG antibodies as tested by the Autoimmune Neurology Group in Oxford. Disability outcomes and disease course were analysed in more detail in a cohort followed in the Neuromyelitis Optica Oxford Service (n = 75), and this included an incident cohort who were diagnosed at disease onset (n = 44)...
November 9, 2017: Brain: a Journal of Neurology
https://www.readbyqxmd.com/read/29136024/severe-oxidative-stress-in-an-acute-inflammatory-demyelinating-model-in-the-rhesus-monkey
#19
Jordon Dunham, Reinofke van de Vis, Jan Bauer, Jacqueline Wubben, Nikki van Driel, Jon D Laman, Bert A 't Hart, Yolanda S Kap
Oxidative stress is increasingly implicated as a co-factor of tissue injury in inflammatory/demyelinating disorders of the central nervous system (CNS), such as multiple sclerosis (MS). While rodent experimental autoimmune encephalomyelitis (EAE) models diverge from human demyelinating disorders with respect to limited oxidative injury, we observed that in a non-human primate (NHP) model for MS, namely EAE in the common marmoset, key pathological features of the disease were recapitulated, including oxidative tissue injury...
2017: PloS One
https://www.readbyqxmd.com/read/29108750/new-spectrum-of-the-neurologic-consequences-of-zika
#20
EDITORIAL
Marco T Medina, Marco Medina-Montoya
Zika virus infection represents a new neuropathological agent with association to a wide spectrum of neurological complications: a) Congenital Zika Syndrome by affecting the neural stem cells of the human fetal brain; b) Guillain-Barré Syndrome by an autoimmune response against peripheral myelin and/or axonal components or probable direct inflammatory reaction; c) Encephalitis/meningoencephalitis and myelitis by a direct viral inflammatory process on the central nervous system; d) Sensory neuropathy by infecting directly the peripheral neurons and causing substantial cell death and pathogenic transcriptional dysregulation; e) Acute Disseminated Encephalomyelitis and optic neuropathy; f) Seizures and Epilepsy and g) childhood arterial ischemic stroke by probable inflammatory reaction and endothelial injury...
December 15, 2017: Journal of the Neurological Sciences
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