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Optic neuromyelitis

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https://www.readbyqxmd.com/read/28932609/neuromyelitis-optica-in-a-nepalese-man
#1
Yogesh Subedi, Utsav Joshi, Sanjeeb Sudarshan Bhandari, Ashbina Pokharel, Ashbita Pokharel
BACKGROUND: Neuromyelitis optica is a severely disabling inflammatory disorder of the central nervous system of autoimmune etiology that mainly affects the optic nerves and spinal cord. Here, we present a case report detailing a patient with tingling and weakness of right upper and lower limbs who was neuromyelitis optica immunoglobulin G-positive. CASE PRESENTATION: A 46-year-old Nepalese man presented to the hospital with a history of tingling and weakness of right upper and lower limbs that developed over a period of two months...
2017: Case Reports in Neurological Medicine
https://www.readbyqxmd.com/read/28926419/peduncular-hallucinosis-and-autonomic-dysfunction-in-anti-aquaporin-4-antibody-syndrome
#2
Renee Berry, Peter K Panegyres
Neuromyelitis optica is a rare, severe inflammatory demyelinating disease of the central nervous system, previously described as affecting only the optic nerve and spinal cord. Since the discovery of a highly specific autoantibody, anti-aquaporin-4, lesions are now recognized outside these regions. We report a man with severe, debilitating symptoms resulting from a symptomatic lesion within the diencephalon, manifesting with abnormal circadian rhythms, autonomic dysfunction, behavioral disturbance, and complex visual hallucinations...
September 2017: Cognitive and Behavioral Neurology: Official Journal of the Society for Behavioral and Cognitive Neurology
https://www.readbyqxmd.com/read/28904454/rituximab-in-neuromyelitis-optica-spectrum-disorders-our-experience
#3
Jui Dilip Jade, Srishti Bansi, Bhim Singhal
BACKGROUND: Neuromyelitis optica (NMO) is an inflammatory demyelinating central nervous system disease, with recurrent attacks of severe bilateral optic neuritis and longitudinally extensive transverse myelitis. Aggressive immunosuppression is essential to prevent clinical relapses and permanent disability. Rituximab, a monoclonal antibody to CD20, has been found effective in several reports and small uncontrolled studies. There is a paucity of data regarding its use in Indian patients...
July 2017: Annals of Indian Academy of Neurology
https://www.readbyqxmd.com/read/28903961/paediatric-optic-neuritis-factors-leading-to-unfavourable-outcome-and-relapses
#4
Delphine Averseng-Peaureaux, Marie Mizzi, Helene Colineaux, Laurence Mahieu, Maria Carmela Pera, David Brassat, Yves Chaix, Emilie Berard, Kumaran Deiva, Emmanuel Cheuret
OBJECTIVES: To identify prognostic factors associated with poor visual recovery and chronic relapsing diseases, for example, multiple sclerosis (MS), in children with optic neuritis (ON) at onset. METHODS: This multicentre retrospective study included 102 children with a first ON episode between 1990 and 2012. The primary criterion was poor visual recovery determined by visual acuity, and the secondary was relapses following ON. RESULTS: Median age was 11 years, 66% were girls and mean follow-up was 24 months...
September 13, 2017: British Journal of Ophthalmology
https://www.readbyqxmd.com/read/28879451/prognostic-factors-and-disease-course-in-aquaporin-4-antibody-positive-chinese-patients-with-acute-optic-neuritis
#5
Hao Kang, Tingjun Chen, Hongyang Li, Quangang Xu, Shanshan Cao, Shihui Wei
The objective is to assess the frequency of AQP4-Ab in Chinese patients with optic neuritis (ON) and to investigate the prognostic implications of AQP4-Ab seropositivity in such patients. The presence of aquaporin-4 (AQP4) antibodies in human AQP4-transfected cells was determined by indirect immunofluorescence, and the diagnostic and prognostic relevance of AQP4 antibodies in 215 Chinese patients with optic neuritis was evaluated. The patients were enrolled from the Chinese People's Liberation Army General Hospital (PLAGH) and followed up to 1-228 months...
September 6, 2017: Journal of Neurology
https://www.readbyqxmd.com/read/28872108/induction-of-paralysis-and-visual-system-injury-in-mice-by-t-cells-specific-for-neuromyelitis-optica-autoantigen-aquaporin-4
#6
Sharon A Sagan, Andrés Cruz-Herranz, Collin M Spencer, Peggy P Ho, Lawrence Steinman, Ari J Green, Raymond A Sobel, Scott S Zamvil
While it is recognized that aquaporin-4 (AQP4)-specific T cells and antibodies participate in the pathogenesis of neuromyelitis optica (NMO), a human central nervous system (CNS) autoimmune demyelinating disease, creation of an AQP4-targeted model with both clinical and histologic manifestations of CNS autoimmunity has proven challenging. Immunization of wild-type (WT) mice with AQP4 peptides elicited T cell proliferation, although those T cells could not transfer disease to naïve recipient mice. Recently, two novel AQP4 T cell epitopes, peptide (p) 135-153 and p201-220, were identified when studying immune responses to AQP4 in AQP4-deficient (AQP4(-/-)) mice, suggesting T cell reactivity to these epitopes is normally controlled by thymic negative selection...
August 21, 2017: Journal of Visualized Experiments: JoVE
https://www.readbyqxmd.com/read/28863861/neuromyelitis-optica-atypical-hemophagocytic-lymphohistiocytosis-and-heterozygous-perforin-a91v-mutation
#7
Boaz Palterer, Francesca Brugnolo, Elena Sieni, Alessandro Barilaro, Paola Parronchi
Neuromyelitis optica is an autoimmune demyelinating inflammatory disease characterized by optic neuritis and myelitis with anti-aquaporin 4 antibodies. Hemophagocytic lymphohistiocytosis is a severe systemic inflammatory syndrome that can present in a genetic primary form or secondarily to infective, neoplastic or autoimmune diseases. Our case discusses the first reported case of atypical late-onset hemophagocytic lymphohistiocytosis in a patient with neuromyelitis optica, with multiple triggering factors and carrying the common A91V hypomorphic perforin mutation, that blurs the distinction between primary and secondary forms...
October 15, 2017: Journal of Neuroimmunology
https://www.readbyqxmd.com/read/28843390/a-mathematical-model-of-cellular-swelling-in-neuromyelitis-optica
#8
Simão Laranjeira, Mkael Symmonds, Jacqueline Palace, Stephen J Payne, Piotr Orlowski
Neuromyelitis Optica (NMO) is a severe neuro-inflammatory disease of the central nervous system characterized by predominant damage to the optic nerve and of the spinal cord. The pathogenic antibody found in the majority of patients targets the AQP4 channels on astrocytic endfeet and causes the cells to swell. Although, the pathophysiology of the disease is broadly known, there are no specific targeted treatments for this process clinically available nor accurate prognostic markers both during attacks and for predicting long term neuronal damage...
November 21, 2017: Journal of Theoretical Biology
https://www.readbyqxmd.com/read/28834867/cortical-blindness-and-not-optic-neuritis-as-a-cause-of-vision-loss-in-a-sj%C3%A3-gren-s-syndrome-ss-patient-with-the-neuromyelitis-optica-spectrum-disorder-nmosd-challenges-of-ascribing-demyelinating-syndromes-to-ss-a-case-report
#9
Aliya Lalji, Izlem Izbudak, Julius Birnbaum
RATIONALE: The conception that multiple sclerosis may be challenging to distinguish from demyelinating manifestations of Sjögren's syndrome (SS) was introduced more than 30 years ago. However, it is now recognized that the neuromyelitis optica spectrum disorder (NMOSD) may occur more frequently in SS as opposed to multiple sclerosis. Characteristic NMOSD features can include severe attacks of optic neuritis, myelitis which is frequently longitudinally-extensive (spanning at least three vertebral segments on magnetic resonance imaging [MRI]), and an association with anti-aquaporin-4 antibodies...
August 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28806345/pediatric-optic-neuritis-what-is-new
#10
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
https://www.readbyqxmd.com/read/28770374/mog-antibody-related-disorders-common-features-and-uncommon-presentations
#11
Álvaro Cobo-Calvo, Anne Ruiz, Hyacintha D'Indy, Anne-Lise Poulat, Maryline Carneiro, Nicolas Philippe, Françoise Durand-Dubief, Kumaran Deiva, Sandra Vukusic, Vincent Desportes, Romain Marignier
Myelin oligodendrocyte glycoprotein antibodies (MOG-Ab) have been reported in acute demyelinating encephalomyelitis (ADEM), optic neuritis (ON), and neuromyelitis optica spectrum disorders (NMOSD) in adults and pediatrics. We aimed to delineate the common features of MOG-Ab-related disorders in children and adults, and report uncommon presentations. Twenty-seven consecutive pediatric and adult patients testing positive for MOG-Ab, with a minimum follow-up of 6 months, were included. Comprehensive epidemiological, clinical, radiological, and laboratory data were retrospectively analyzed...
August 2, 2017: Journal of Neurology
https://www.readbyqxmd.com/read/28750658/complement-regulator-cd59-prevents-peripheral-organ-injury-in-rats-made-seropositive-for-neuromyelitis-optica-immunoglobulin-g
#12
Xiaoming Yao, Alan S Verkman
Pathogenesis in aquaporin-4 immunoglobulin G (AQP4-IgG) seropositive neuromyelitis optica spectrum disorders (herein called NMO) involves complement-dependent cytotoxicity initiated by AQP4-IgG binding to astrocyte AQP4. We recently reported that rats lacking complement inhibitor protein CD59 were highly susceptible to development of NMO pathology in brain and spinal cord following direct AQP4-IgG administration (Yao and Verkman, Acta Neuropath Commun 2017, 5:15). Here, we report evidence that CD59 is responsible for protection of peripheral, AQP4-expressing tissues in seropositive NMO...
July 27, 2017: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/28716289/devic-s-disease-before-devic-on-the-contribution-of-friedrich-albin-schanz-1863-1923
#13
S Jarius, B Wildemann
Neuromyelitis optica (NMO, Devic's disease) is an autoimmune disorder of the CNS that mainly affects the optic nerves and spinal cord and, if not treated, can leave patients blind and wheelchair-bound. NMO was long considered a clinical variant of multiple sclerosis (MS). While the history of MS has been studied extensively, relatively little is known about the early history of NMO. A dissertation written by Fernand Gault (1873-1936), a doctoral student to Eugène Devic (1858-1930), a Lyon-based neurologist after whom the disease would later be named, is considered by many the first review of NMO...
August 15, 2017: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/28684532/longitudinal-analysis-of-myelin-oligodendrocyte-glycoprotein-antibodies-in-cns-inflammatory-diseases
#14
Jae-Won Hyun, Mark R Woodhall, Su-Hyun Kim, In Hye Jeong, Byungsoo Kong, Gayoung Kim, Yeseul Kim, Min Su Park, Sarosh R Irani, Patrick Waters, Ho Jin Kim
BACKGROUND: We evaluated the seroprevalence of myelin oligodendrocyte glycoprotein immunoglobulin G1 (MOG-IgG) and associated clinical features of patients from a large adult-dominant unselected cohort with mainly relapsing central nervous system (CNS) inflammatory diseases. We also investigate the clinical relevance of MOG-IgG through a longitudinal analysis of serological status over a 2-year follow-up period. METHODS: Serum samples from 505 patients with CNS inflammatory diseases at the National Cancer Center were analysed using cell-based assays for MOG-IgG and aquaporin-4 immunoglobulin G (AQP4-IgG)...
July 6, 2017: Journal of Neurology, Neurosurgery, and Psychiatry
https://www.readbyqxmd.com/read/28674367/neuromyelitis-optica-spectrum-disorder-with-recurrent-intracranial-hemorrhage
#15
Hiroaki Yaguchi, Yasunori Mito, Ikkei Ohashi, Taichi Nomura, Ichiro Yabe, Yasutaka Tajima
The patient was a woman without hypertension who had previously experienced intracranial hemorrhage twice at 48 and 56 years of age. At 59 years of age, she was diagnosed with neuromyelitis optica spectrum disorder (NMOSD) based on the presence of a brain stem lesion and the detection of anti-aquaporin 4 (AQP4) antibodies. After 5 months of continuous treatment with prednisolone (15 mg/day), she presented with optic neuritis and intracranial bleeding. A recurrent attack of NMOSD and intracranial hemorrhage were concurrently diagnosed...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28670343/differential-diagnosis-of-neuromyelitis-optica-spectrum-disorders
#16
REVIEW
Sung-Min Kim, Seong-Joon Kim, Haeng Jin Lee, Hiroshi Kuroda, Jacqueline Palace, Kazuo Fujihara
Neuromyelitis optica spectrum disorder (NMOSD) is an inflammatory disorder of the central nervous system (CNS) mostly manifesting as optic neuritis and/or myelitis, which are frequently recurrent/bilateral or longitudinally extensive, respectively. As the autoantibody to aquaporin-4 (AQP4-Ab) can mediate the pathogenesis of NMOSD, testing for the AQP4-Ab in serum of patients can play a crucial role in diagnosing NMOSD. Nevertheless, the differential diagnosis of NMOSD in clinical practice is often challenging despite the phenotypical and serological characteristics of the disease because: (1) diverse diseases with autoimmune, vascular, infectious, or neoplastic etiologies can mimic these phenotypes of NMOSD; (2) patients with NMOSD may only have limited clinical manifestations, especially in their early disease stages; (3) test results for AQP4-Ab can be affected by several factors such as assay methods, serologic status, disease stages, or types of treatment; (4) some patients with NMOSD do not have AQP4-Ab; and (5) test results for the AQP4-Ab may not be readily available for the acute management of patients...
July 2017: Therapeutic Advances in Neurological Disorders
https://www.readbyqxmd.com/read/28638708/citation-classics-in-central-nervous-system-inflammatory-demyelinating-disease
#17
Jee-Eun Kim, Kang M Park, Yerim Kim, Dae Y Yoon, Jong S Bae
OBJECTIVES: To identify and analyze the characteristics of the most influential articles about central nervous system (CNS) inflammatory demyelinating disease. MATERIALS AND METHODS: The Institute for Scientific Information (ISI) Web of Science database and the 2014 Journal Citation Reports Science Edition were used to retrieve the top 100 cited articles on CNS inflammatory demyelinating disease. The citation numbers, journals, years of publication, authorships, article types, subjects and main issues were analyzed...
June 2017: Brain and Behavior
https://www.readbyqxmd.com/read/28637379/neuropsychological-outcomes-of-pediatric-demyelinating-diseases-a-review
#18
Alexander Tan, Cole Hague, Benjamin M Greenberg, Lana Harder
Immune-mediated central nervous system (CNS) demyelinating diseases impact various areas of the brain, optic nerves, and/or spinal cord and can result in a wide range of neurologic symptoms including adverse cognitive outcomes. Neuropsychological outcomes in adult multiple sclerosis (MS) are well documented, while literature on such outcomes in pediatric cohorts is more limited. Furthermore, literature on neuropsychological outcomes in pediatric acute disseminated encephalomyelitis (ADEM), neuromyelitis optica (NMO), and transverse myelitis (TM) is even more limited...
June 21, 2017: Child Neuropsychology: a Journal on Normal and Abnormal Development in Childhood and Adolescence
https://www.readbyqxmd.com/read/28632021/neuromyelitis-optica-application-of-computer-diagnostics-to-historical-case-reports
#19
Pablo Garcia Reitboeck, Peter Garrard, Timothy Peters
The retrospective diagnosis of illnesses by medical historians can often be difficult and prone to bias, although knowledge of the medical disorders of historical figures is key to the understanding of their behavior and reactions. The recent application of computer diagnostics to historical figures allows an objective differential diagnosis to be accomplished. Taking an example from clinical neurology, we analyzed the earliest reported cases of Devic's disease (neuromyelitis optica) that commonly affects the optic nerve and spinal cord and was previously often confused with multiple sclerosis...
October 2017: Journal of the History of the Neurosciences
https://www.readbyqxmd.com/read/28619428/neuromyelitis-optica-presenting-with-horner-syndrome-a-case-report-and-review-of-literature
#20
İrem Fatma Uludağ, Alp Sarıteke, Levent Öcek, Yaşar Zorlu, Ufuk Şener, Figen Tokuçoğlu, Burhanettin Uludağ
Neuromyelitis optica (NMO) is a demyelinating disease of the central nervous system that predominantly affects the spinal cord and optic nerves. We describe a 19 years old woman with left Horner syndrome (HS), who was diagnosed as NMO with characteristic longitudinally extensive myelitis and positive serum aquaporin-4 immunoglobulin G antibodies (AQP4-IgG). Our report describes one of the very rare ocular motor symptoms in NMO patients.
May 2017: Multiple Sclerosis and related Disorders
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