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Acute disseminating encephalomyelitis

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https://www.readbyqxmd.com/read/29143589/biomarkers-in-the-evolution-of-multiple-sclerosis
#1
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
#2
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...
November 15, 2017: 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
#3
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
#4
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
#5
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
#6
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
#7
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...
November 1, 2017: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/29103426/the-spectrum-of-inflammatory-acquired-demyelinating-syndromes-in-children
#8
Rinze Neuteboom, Colin Wilbur, Danielle Van Pelt, Moses Rodriguez, Ann Yeh
Acquired demyelinating syndromes in childhood comprise a spectrum of monophasic and recurrent inflammatory conditions of the central nervous system. Examples of monophasic conditions include, clinically isolated syndromes such as optic neuritis and transverse myelitis, as well as acute disseminated encephalomyelitis, whereas recurrent disorders include entities such as multiple sclerosis and neuromyelitis optica spectrum disorder. Knowledge about these disorders has expanded due to rigorously evaluated diagnostic criteria, magnetic resonance imaging features, outcomes, and serum biomarkers in these disorders...
August 2017: Seminars in Pediatric Neurology
https://www.readbyqxmd.com/read/29093388/an-adult-case-of-recurrent-guillain-barr%C3%A3-syndrome-with-anti-galactocerebroside-antibodies
#9
Hisashi Takahashi, Tadashi Kimura, Natsuko Yuki, Akira Yoshioka
A 79-year-old woman with a history of Guillain-Barré syndrome (GBS) developed somnolence and tetraparesis after pneumonia. Based on clinical and laboratory findings, she was diagnosed with complications of acute inflammatory demyelinating polyneuropathy (AIDP) and acute disseminated encephalomyelitis (ADEM). Anti-galactocerebroside (Gal-C) IgG antibodies were detected in her serum. Cases of recurrent GBS in patients who are positive for this antibody are extremely rare. The anti-Gal-C IgG antibodies likely played an important role in the pathogenesis of the AIDP and ADEM...
November 1, 2017: Internal Medicine
https://www.readbyqxmd.com/read/29070756/a-case-of-anti-mog-antibody-positive-multiphasic-disseminated-encephalomyelitis-co-occurring-with-unilateral-cerebral-cortical-encephalitis
#10
Naoya Fukushima, Miki Suzuki, Ryo Ogawa, Kitami Hayashi, Jun-Ichi Takanashi, Takashi Ohashi
A 20-year-old woman first developed acute disseminated encephalomyelitis (ADEM) at 11 years of age. At 17 years of age, she was hospitalized due to generalized seizure and diagnosed with encephalitis. Brain MRI revealed a FLAIR-hyperintense lesion in the unilateral cerebral cortex. At 18 years of age, serum anti-myelin oligodendrocyte glycoprotein (MOG) antibody was detected. At 20 years of age, she was admitted to our hospital, diagnosed with multifocal disseminated encephalomyelitis (MDEM). MDEM has been observed in patients that are seropositive for the anti-MOG antibody...
October 26, 2017: Rinshō Shinkeigaku, Clinical Neurology
https://www.readbyqxmd.com/read/29070051/human-antibodies-against-the-myelin-oligodendrocyte-glycoprotein-can-cause-complement-dependent-demyelination
#11
Patrick Peschl, Kathrin Schanda, Bleranda Zeka, Katherine Given, Denise Böhm, Klemens Ruprecht, Albert Saiz, Andreas Lutterotti, Kevin Rostásy, Romana Höftberger, Thomas Berger, Wendy Macklin, Hans Lassmann, Monika Bradl, Jeffrey L Bennett, Markus Reindl
BACKGROUND: Antibodies to the myelin oligodendrocyte glycoprotein (MOG) are associated with a subset of inflammatory demyelinating diseases of the central nervous system such as acute disseminated encephalomyelitis and neuromyelitis optica spectrum disorders. However, whether human MOG antibodies are pathogenic or an epiphenomenon is still not completely clear. Although MOG is highly conserved within mammals, previous findings showed that not all human MOG antibodies bind to rodent MOG...
October 25, 2017: Journal of Neuroinflammation
https://www.readbyqxmd.com/read/29049805/health-related-quality-of-life-in-pediatric-patients-with-demyelinating-diseases-relevance-of-disability-relapsing-presentation-and-fatigue
#12
Mariella M Self, Aaron Fobian, Katherine Cutitta, Arianne Wallace, Timothy E Lotze
Decreased health-related quality of life (HRQOL) in pediatric patients with multiple sclerosis is established, but little research has examined HRQOL in the broader pediatric demyelinating disease population, and predictors of reduced HRQOL are largely unexplored. We sought to (1) compare generic HRQOL and fatigue of pediatric patients with relapsing (i.e., multiple sclerosis and neuromyelitis optica) versus monophasic demyelinating diseases (i.e., acute disseminated encephalomyelitis, optic neuritis, transverse myelitis, clinically isolated syndrome) and (2) examine the extent to which disability, relapsing disease, and fatigue predict HRQOL...
July 21, 2017: Journal of Pediatric Psychology
https://www.readbyqxmd.com/read/29033632/review-of-viral-encephalitis-cases-seen-at-a-tertiary-care-center-in-turkey-focus-on-herpes-simplex-type-1
#13
Beyza Çiftçi Kavaklioğlu, Eda Çoban, Aysu Şen, Elif Söylemezoğlu, Mehmet Ali Aldan, Dilek Atakli, Aysun Soysal
INTRODUCTION: The objective of this study was to determine the incidence of herpes simplex encephalitis (HSE), known as the most common, potentially mortal, and treatable cause of sporadic encephalitis, in a sample Turkish population. METHODS: The demographic, clinical, laboratory, imaging, electrophysiology, and polymerase chain reaction (PCR) DNA results of patients examined with a pre-diagnosis of encephalitis were retrospectively examined. RESULTS: A total of 68 patients were included in the study...
September 2017: Noro Psikiyatri Arsivi
https://www.readbyqxmd.com/read/29025172/-anti-myelin-oligodendrocyte-glycoprotein-antibodies-in-paediatric-patients-with-optic-neuritis
#14
Helmut Tegetmeyer, Andreas Merkenschlager
Background Myelin oligodendrocyte glycoprotein (MOG) is located on the surface of oligodendrocytes and myelin in the central nervous system. MOG-IgG is associated with acute disseminated encephalomyelitis (ADEM), relapsing and bilateral optic neuritis (NNO), and transverse myelitis (TM) in both paediatric and adult patients. The combination of NNO and TM or other inflammatory brain lesions is a typical feature of neuromyelitis optica spectrum disorders (NMO-SD) which are associated with specific pathogenic autoantibodies against the water channel aquaporin-4 (AQP4-IgG)...
October 2017: Klinische Monatsblätter Für Augenheilkunde
https://www.readbyqxmd.com/read/28987175/inflammatory-demyelinating-diseases-of-the-central-nervous-system
#15
Romana Höftberger, Hans Lassmann
Inflammatory demyelinating diseases are a heterogeneous group of disorders, which occur against the background of an acute or chronic inflammatory process. The pathologic hallmark of multiple sclerosis (MS) is the presence of focal demyelinated lesions with partial axonal preservation and reactive astrogliosis. Demyelinated plaques are present in the white as well as gray matter, such as the cerebral or cerebellar cortex and brainstem nuclei. Activity of the disease process is reflected by the presence of lesions with ongoing myelin destruction...
2017: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/28941527/pediatric-optic-neuritis
#16
Melinda Y Chang, Stacy L Pineles
Optic neuritis is rare in children in comparison to adults, but accounts for approximately 25% of pediatric acute demyelinating syndromes. Features of pediatric optic neuritis that differ from adults include a higher rate of bilaterality, poor visual acuity on presentation, and papillitis. Diagnostic work-up includes brain magnetic resonance imaging, lumbar puncture, and blood tests to exclude infectious and inflammatory disorders. Pediatric optic neuritis may occur following infection or vaccination, or in association with a systemic demyelinating process such as acute disseminated encephalomyelitis, neuromyelitis optica, or multiple sclerosis...
May 2017: Seminars in Pediatric Neurology
https://www.readbyqxmd.com/read/28936092/multifocal-balo-s-concentric-sclerosis-in-children-report-of-a-case-and-review-of-literature
#17
Sanjeev Kumar Bhoi, Suprava Naik, Jayantee Kalita, U K Misra
Balo's concentric sclerosis (BCS) is a rare demyelinating lesion considered to be a variant of multiple sclerosis (MS). On magnetic resonance imaging (MRI) Balo's concentric sclerosis shows the typical concentric pattern. We report a case of 10 year old child with BCS who presented as post infectious acute disseminated encephalomyelitis (ADEM). He is asymptomatic and had no relapse after 6 years of follow-up.
August 2017: Journal of Neurosciences in Rural Practice
https://www.readbyqxmd.com/read/28931785/ventricular-fibrillation-due-to-early-repolarization-syndrome-in-the-wake-of-hypothermia-due-to-fulminant-acute-disseminated-encephalomyelitis
#18
Kyoichiro Yazaki, Yoichi Ajiro, Kunihiko Shimizu, Fumiaki Mori, Nobue Yagihara, Akinori Sato, Tatsuya Takahashi, Kazunori Iwade
No abstract text is available yet for this article.
September 20, 2017: Circulation Journal: Official Journal of the Japanese Circulation Society
https://www.readbyqxmd.com/read/28887792/liver-failure-as-the-presentation-of-ornithine-transcarbamylase-deficiency-in-a-13-month-old-female
#19
Farrah Rajabi, Lance H Rodan, Maureen M Jonas, Janet S Soul, Nicole J Ullrich, Ann Wessel, Susan E Waisbren, Wen-Hann Tan, Gerard T Berry
Ornithine transcarbamylase deficiency (OTCD) is an X-linked urea cycle disorder with variable expressivity in heterozygous females. While liver function testing is often abnormal in patients with OTCD, liver failure is uncommon on presentation. A 13-month-old female with no significant past medical history presented with irritability, right arm weakness, and decreased appetite. Initial workup revealed hepatic dysfunction with an INR of 3.4, ammonia level of 75 μmol/L, and abnormal brain MRI with gyral edema with restricted diffusion, and patchy signal abnormality in basal ganglia...
September 9, 2017: JIMD Reports
https://www.readbyqxmd.com/read/28806345/pediatric-optic-neuritis-what-is-new
#20
REVIEW
Mark Borchert, Grant T Liu, Stacy Pineles, Amy T Waldman
Few diseases blur the margins between their childhood and adult-onset varieties as much as optic neuritis. This report will review our state of knowledge of pediatric optic neuritis, as well as its relationship to the latest consensus definitions of neuroinflammatory disease. Current diagnostic and treatment options will be explored, as well as our potential to uncover an understanding of pediatric optic neuritis through systematic prospective studies. The risk of evolving multiple sclerosis is probably less than in adults, but pediatric optic neuritis is more likely to be an initial manifestation of acute disseminated encephalomyelitis...
September 2017: Journal of Neuro-ophthalmology: the Official Journal of the North American Neuro-Ophthalmology Society
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