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Optic neuromyelitis

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https://www.readbyqxmd.com/read/28212662/marked-central-nervous-system-pathology-in-cd59-knockout-rats-following-passive-transfer-of-neuromyelitis-optica-immunoglobulin-g
#1
Xiaoming Yao, Alan S Verkman
Neuromyelitis optica spectrum disorders (herein called NMO) is an inflammatory demyelinating disease of the central nervous system in which pathogenesis involves complement-dependent cytotoxicity (CDC) produced by immunoglobulin G autoantibodies targeting aquaporin-4 (AQP4-IgG) on astrocytes. We reported evidence previously, using CD59(-/-) mice, that the membrane-associated complement inhibitor CD59 modulates CDC in NMO (Zhang and Verkman, J. Autoimmun. 53:67-77, 2014). Motivated by the observation that rats, unlike mice, have human-like complement activity, here we generated CD59(-/-) rats to investigate the role of CD59 in NMO and to create NMO pathology by passive transfer of AQP4-IgG under conditions in which minimal pathology is produced in normal rats...
February 17, 2017: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/28199381/impact-of-the-anti-aquaporin-4-autoantibody-on-inner-retinal-structure-function-and-structure-function-associations-in-japanese-patients-with-optic-neuritis
#2
Yoshiko Matsumoto, Sotaro Mori, Kaori Ueda, Takuji Kurimoto, Akiyasu Kanamori, Yuko Yamada, Ichiro Nakashima, Makoto Nakamura
PURPOSE: An autoantibody against aquaporin-4 (AQP4 Ab) is highly specific for neuromyelitis optica spectrum disorder and plays a pathogenic role in this disease. The purpose of this study was to investigate the impact of AQP4 Ab on inner retinal structure, function, and the structure-function relationships in eyes with optic neuritis. METHODS: Thirty five eyes from 25 cases who had received visual function tests and RTVue optical coherence tomography (OCT) measurement at least six months after the latest episode of optic neuritis were enrolled...
2017: PloS One
https://www.readbyqxmd.com/read/28149102/magnetic-resonance-imaging-brain-findings-in-a-case-of-aquaporin-4-antibody-positive-neuromyelitis-optica-spectrum-disorder-presenting-with-intractable-vomiting-and-hiccups
#3
Prerna Garg, Muthusubramanian Rajasekaran, Salil Pandey, Gnanashanmugam Gurusamy, Devanand Balalakshmoji, Rajakumar Rathinasamy
Neuromyelitisoptica (NMO) and multiple sclerosis (MS) were once considered to be differing manifestation of same auto immune disease, NMO predominantly involving the optic nerve and cord. Now with discovery of NMO antibody the concept has changed and a spectrum of disorders with lesions in brain has been identified. Occasionally, brain may be the first or the only site of involvement in these disorders hence it is essential to be aware of this spectrum. The brain lesions in NMO/NMOSD may be located in characteristic regions and present with symptoms mimicking non neurological disease...
January 2017: Journal of Neurosciences in Rural Practice
https://www.readbyqxmd.com/read/28132975/a-case-of-neuromyelitis-optica-spectrum-disorder-nmosd-with-sj%C3%A3-gren-s-syndrome-manifested-only-brain-involvement-by-preceding-parotitis
#4
Takahiro Furukawa, Naoko Matsui, Keiko Tanaka, Yuishin Izumi, Ryuji Kaji
A 33 year-old woman presented with intentional incontinence, motor aphasia, supranuclear gaze palsy, and spasticity after parotitis. Brain magnetic resonance images (MRI) showed abnormal signaling in long corticospinal tract involving internal capsules and cerebral peduncles, middle cerebellar peduncle, and frontal subcortical white matter lesions. She had a long history of dry eye and mouth. Immunoserological study showed that she was positive for anti-SS-A, aquaporin 4 (AQP4), and AQP5 antibodies. She clinically showed not only Sjögren's syndrome but also neuromyelitis optica spectrum disorder (NMOSD) without optic neuritis or myelitis...
January 28, 2017: Rinshō Shinkeigaku, Clinical Neurology
https://www.readbyqxmd.com/read/28131170/longitudinally-extensive-transverse-myelitis-immune-mediated-in-aquaporin-4-antibody-negative-patients-disease-heterogeneity
#5
Edgar Carnero Contentti, Javier Pablo Hryb, Sergio Morales, Alejandra Gomez, Edson Chiganer, José Luis Di Pace, Carmen Lessa, Mónica Perassolo
BACKGROUND: Longitudinally extensive transverse myelitis (LETM) is a frequent manifestation of neuromyelitis optica spectrum disorder (NMOSD). However, it can also occur in other immune-mediated diseases of the central nervous system (CNS). Positive aquoporin-4 antibodies (AQP4-ab) predict higher relapse rate after LETM. OBJECTIVE: To assess clinical and brain/spinal cord magnetic resonance imaging (MRI) features of LETM immune-mediated at onset and to compare AQP4-ab negative (N-LETM) with AQP4-ab positive (P-LETM) patients...
February 15, 2017: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/28104256/severe-structural-and-functional-visual-system-damage-leads-to-profound-loss-of-vision-related-quality-of-life-in-patients-with-neuromyelitis-optica-spectrum-disorders
#6
Felix Schmidt, Hanna Zimmermann, Janine Mikolajczak, Frederike C Oertel, Florence Pache, Maria Weinhold, Johann Schinzel, Judith Bellmann-Strobl, Klemens Ruprecht, Friedemann Paul, Alexander U Brandt
BACKGROUND: Neuromyelitis optica spectrum disorders (NMOSD) are characterized by devastating optic neuritis attacks causing more structural damage and visual impairment than in multiple sclerosis (MS). The objective of this study was to compare vision-related quality of life in NMOSD and MS patients and correlate it to structural retinal damage and visual function. METHODS: Thirty-one NMOSD and 31 matched MS patients were included. Vision-related quality of life was assessed with the 39-item National Eye Institute Visual Function Questionnaire (NEI-VFQ)...
January 2017: Multiple Sclerosis and related Disorders
https://www.readbyqxmd.com/read/28071581/role-of-aqp4-antibody-serostatus-and-its-prediction-of-visual-outcome-in-neuromyelitis-optica-a-systematic-review-and-meta-analysis
#7
Nan Lin, Qing Liu, Xiaoyu Wang, Jianmei Ma, Yuyuan Li
: Backgroud: Neuromyelitis optica (NMO) is an autoimmune inflammatory disorder, which is characterized by severe attacks of optic neuritis and myelitis. Antibodies (Ab) to aquaporin-4 (AQP4) (or NMO-IgG) as a serological biomarker of NMO have been widespread used. Nevertheless, some NMO patients remain seronegative for AQP4-Ab and/or have no detected optic nerve involvement. In addition, no consensus exists on the association between AQP4-Ab serostatus and visual outcome in NMO. To drive a more precise estimate of this postulated relationship, a meta-analysis was performed based on existing relevant studies...
January 10, 2017: Protein and Peptide Letters
https://www.readbyqxmd.com/read/28068933/screening-for-onconeural-antibodies-in-neuromyelitis-optica-spectrum-disorders
#8
Benjamin Berger, Tilman Hottenrott, Sebastian Rauer, Oliver Stich
BACKGROUND: Some so-called "non-classical" paraneoplastic neurological syndromes (PNS), namely optic neuritis and myelitis, clinically overlap with neuromyelitis optica spectrum disorders (NMOSD), and conversely, in cancer-associated NMOSD, a paraneoplastic etiology has been suggested in rare cases. Therefore, we retrospectively investigated the prevalence of onconeural antibodies, which are highly predictive for a paraneoplastic etiology, and the prevalence of malignancies in NMOSD patients...
January 10, 2017: BMC Neurology
https://www.readbyqxmd.com/read/28060988/-neuromyelitis-optica-presenting-concomitantly-with-systemic-lupus-erythematosus-report-of-one-case
#9
Felipe Suárez H, Daniela Urrutia E, Felipe Canales P, Camila Gutiérrez O
Neuromyelitis optica (NMO) is a severe demyelinating disease of the central nervous system, which preferentially attacks the optic nerve and spinal cord. It is associated with antibodies against aquaporin 4. Morbidity and mortality are higher than in multiple sclerosis and its treatment focuses on immunosuppressive drugs. Immunomodulators are contraindicated. We report a previously healthy 35-year-old man, presenting with NMO concomitantly with systemic lupus erythematosus. His evolution was torpid with three outbreaks in the 10 months after the diagnosis, requiring a first-line therapy with methylprednisolone and cyclophosphamide and then a second-line therapy with rituximab...
September 2016: Revista Médica de Chile
https://www.readbyqxmd.com/read/28058965/clinical-characteristics-of-late-onset-neuromyelitis-optica-spectrum-disorder-a-multicenter-retrospective-study-in-korea
#10
Jin Myoung Seok, Hye-Jin Cho, Suk-Won Ahn, Eun Bin Cho, Min Su Park, In-Soo Joo, Ha Young Shin, Sun-Young Kim, Byung-Jo Kim, Jong Kuk Kim, Joong-Yang Cho, So-Young Huh, Ohyun Kwon, Kwang-Ho Lee, Byoung Joon Kim, Ju-Hong Min
BACKGROUND: There are currently few studies regarding late-onset neuromyelitis optica spectrum disorder (LO-NMOSD). OBJECTIVE: We aimed to describe the characteristic features of patients with LO-NMOSD in Korea. METHODS: Anti-aquaporin-4 antibody-positive patients with neuromyelitis optica spectrum disorder (NMOSD) from nine tertiary hospitals were reviewed retrospectively. The patients were divided into two groups based on age of onset: LO-NMOSD (⩾50 years of age at onset) versus early-onset neuromyelitis optica spectrum disorder (EO-NMOSD) (<50 years of age at onset)...
January 1, 2017: Multiple Sclerosis: Clinical and Laboratory Research
https://www.readbyqxmd.com/read/28057643/retinal-segmented-layers-with-strong-aquaporin-4-expression-suffered-more-injuries-in-neuromyelitis-optica-spectrum-disorders-compared-with-optic-neuritis-with-aquaporin-4-antibody-seronegativity-detected-by-optical-coherence-tomography
#11
Chun Xia Peng, Hong Yang Li, Wei Wang, Jun Qing Wang, Lei Wang, Quan Gang Xu, Shan Shan Cao, Huan Fen Zhou, Shuo Zhao, Shi Hui Wei
PURPOSE: To evaluate retinal segmented layer alterations in optic neuritis (ON) in an AQP4-Ab seropositive (AQP4-Ab+/ON) cohort and in neuromyelitis optica (NMO) with ON eyes (NMO-ON) compared with an AQP4-Ab seronegative ON (AQP4-Ab-/ON) cohort using optical coherence tomography (OCT). METHODS: We recruited 109 patients with ON (161 eyes) and 47 healthy controls. All patients with ON were subdivided into three subcohorts: 37 patients (54 eyes) with AQP4-Ab+/ON, 45 patients (65 eyes) with AQP4-Ab-/ON and 27 patients (42 eyes) with NMO-ON...
January 5, 2017: British Journal of Ophthalmology
https://www.readbyqxmd.com/read/28017256/treatment-of-neuromyelitis-optica-and-neuromyelitis-optica-spectrum-disorders-with-rituximab-using-a-maintenance-treatment-regimen-and-close-cd19-b-cell-monitoring-a-six-year-follow-up
#12
M E Evangelopoulos, E Andreadou, G Koutsis, V Koutoulidis, M Anagnostouli, P Katsika, D S Evangelopoulos, I Evdokimidis, C Kilidireas
Neuromyelitis optinca (NMO) represents a serious demyelinating disease of the central nervous system selectively attacking the spinal cord and optic nerve. Early differential diagnosis from multiple sclerosis is of vital importance, as NMO mandates immunosuppressive and not immunomodulatory treatment. Rituximab has been recently introduced as a treatment option for NMO. However, optimal surrogate measures and treatment intervals are still unclear. Five patients (females, mean age 54±10.21years) with NMO and NMO spectrum disorders (NMOSD) were evaluated with respect to disability and relapse rate...
January 15, 2017: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/27990369/macular-changes-of-neuromyelitis-optica-through-spectral-domain-optical-coherence-tomography
#13
Lu Cheng, Jing Wang, Xu He, Xun Xu, Zhen-Fen Ling
AIM: To evaluate the thickness of the retinal layers in the macula using spectral-domain optical coherence tomography (SD-OCT) in patients with neuromyelitis optica (NMO). METHODS: Spectralis SD-OCT, utilizing automated macular layer segmentation, was performed in 26 NMO patients and 26 healthy controls. Visual function including visual field tests and pattern visual evoked potential were recorded in study subjects. RESULTS: Forty-one eyes from 26 NMO patients and 52 eyes from 26 age- and sex-matched healthy controls were included...
2016: International Journal of Ophthalmology
https://www.readbyqxmd.com/read/27940915/tolerance-checkpoint-bypass-permits-emergence-of-pathogenic-t-cells-to-neuromyelitis-optica-autoantigen-aquaporin-4
#14
Sharon A Sagan, Ryan C Winger, Andrés Cruz-Herranz, Patricia A Nelson, Sarah Hagberg, Corey N Miller, Collin M Spencer, Peggy P Ho, Jeffrey L Bennett, Michael Levy, Marc H Levin, Alan S Verkman, Lawrence Steinman, Ari J Green, Mark S Anderson, Raymond A Sobel, Scott S Zamvil
Aquaporin-4 (AQP4)-specific T cells are expanded in neuromyelitis optica (NMO) patients and exhibit Th17 polarization. However, their pathogenic role in CNS autoimmune inflammatory disease is unclear. Although multiple AQP4 T-cell epitopes have been identified in WT C57BL/6 mice, we observed that neither immunization with those determinants nor transfer of donor T cells targeting them caused CNS autoimmune disease in recipient mice. In contrast, robust proliferation was observed following immunization of AQP4-deficient (AQP4(-/-)) mice with AQP4 peptide (p) 135-153 or p201-220, peptides predicted to contain I-A(b)-restricted T-cell epitopes but not identified in WT mice...
December 20, 2016: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/27936193/occurrence-of-asymptomatic-acute-neuromyelitis-optica-spectrum-disorder-typical-brain-lesions-during-an-attack-of-optic-neuritis-or-myelitis
#15
Su-Hyun Kim, Jae-Won Hyun, AeRan Joung, Sang Hyun Lee, Ho Jin Kim
We aimed to investigate the frequency of asymptomatic acute brain MRI abnormalities accompanying optic neuritis (ON) or myelitis in neuromyelitis optica spectrum disorder (NMOSD) patients with aquaporin-4 antibodies (AQP4-Ab). We reviewed 324 brain MRI scans that were obtained during acute attacks of ON or myelitis, in 165 NMOSD patients with AQP4-Ab. We observed that acute asymptomatic NMOSD-typical brain lesions accompanied 27 (8%) acute attacks of ON or myelitis in 24 (15%) patients. The most common asymptomatic brain abnormalities included edematous corpus callosum lesions (n = 17), followed by lesions on the internal capsule and/or cerebral peduncle lesions (n = 9), periependymal surfaces of the fourth ventricle (n = 5), large deep white matter lesions (n = 4), periependymal cerebral lesions surrounding the lateral ventricles (n = 3), and hypothalamic lesions (n = 1)...
2016: PloS One
https://www.readbyqxmd.com/read/27936154/retinal-morphology-and-sensitivity-are-primarily-impaired-in-eyes-with-neuromyelitis-optica-spectrum-disorder-nmosd
#16
Ryutaro Akiba, Hirotaka Yokouchi, Masahiro Mori, Toshiyuki Oshitari, Takayuki Baba, Setsu Sawai, Satoshi Kuwabara, Shuichi Yamamoto
BACKGROUND: Previous studies of neuromyelitis optica spectrum disorder (NMOSD) using spectral domain optical coherence tomography (SD-OCT) showed that the outer nuclear layer (ONL) in eyes without a history of optic neuritis (ON) was thinner than that of healthy controls. It remains unclear whether the ONL thinning is caused by a direct attack on the retina by an autoantibody or a retrograde degeneration. OBJECTIVE: To determine the mechanisms involved in the retinal damage in eyes with NMOSD without ON...
2016: PloS One
https://www.readbyqxmd.com/read/27919493/eleven-episodes-of-recurrent-optic-neuritis-of-the-same-eye-for-22-years-eventually-diagnosed-as-neuromyelitis-optica-spectrum-disorder
#17
Yih Chian Yew, Jyh Yung Hor, Thien Thien Lim, Ruban Kanesalingam, Yee Ming Ching, Masita Arip, P E Samuel Easaw, Gaik Bee Eow
It is difficult to predict whether a particular attack of neuromyelitis optica spectrum disorder (NMOSD) will affect the optic nerve [optic neuritis (ON): unilateral or bilateral], spinal cord (myelitis), brain or brainstem, or a combination of the above. We report an interesting case of recurrent ON of the same eye for a total of 11 episodes in a Chinese woman. Over a period of 22 years, the attacks only involved the left eye, and never the right eye and also no myelitis. For a prolonged duration, she was diagnosed as recurrent idiopathic ON...
November 2016: Multiple Sclerosis and related Disorders
https://www.readbyqxmd.com/read/27919188/clinical-analysis-of-neuromyelitis-optica-presenting-as-intractable-nausea-vomiting-and-hiccups
#18
Xuehong Jin, Shaofang Pei, Yi Liu, Xia Li
Vomiting and hiccups can be the manifestations of numerous systemic and neurological illnesses. Intractable nausea, vomiting and hiccups (INH) are reported as possible initial manifestations of neuromyelitis optica (NMO), but not correctly identified. Awareness of these atypical presentations is conducive to NMO early diagnosis and proper treatment to prevent further disability. In this paper, 12 NMO were reported, whose intractable vomiting and hiccups were the sole manifestations of the first attack and other attacks involving spinal cord and optic nerves developed later...
December 16, 2016: International Journal of Neuroscience
https://www.readbyqxmd.com/read/27859858/diffusion-weighted-imaging-helps-differentiate-multiple-sclerosis-and-neuromyelitis-optica-related-acute-optic-neuritis
#19
Hailin Wan, Huijin He, Fang Zhang, Yan Sha, Guohong Tian
PURPOSE: To evaluate the apparent diffusion coefficient (ADC) values between multiple sclerosis (MS) and neuromyelitis optica (NMO)-related acute optic neuritis (ON) patients and predict their optic nerve atrophy of optic coherence tomography (OCT) parameters. MATERIALS AND METHODS: Nineteen MS and 15 NMO-related acute ON patients who underwent a diffusion-weighted imaging sequence in 3.0 Tesla MR scanner and a follow-up OCT examination after 6 months were included...
November 9, 2016: Journal of Magnetic Resonance Imaging: JMRI
https://www.readbyqxmd.com/read/27854301/optical-coherence-tomography-and-magnetic-resonance-imaging-in-multiple-sclerosis-and-neuromyelitis-optica-spectrum-disorder
#20
REVIEW
Praveena Manogaran, James V M Hanson, Elisabeth D Olbert, Christine Egger, Carla Wicki, Christina Gerth-Kahlert, Klara Landau, Sven Schippling
Irreversible disability in multiple sclerosis (MS) and neuromyelitis optica spectrum disorder (NMOSD) is largely attributed to neuronal and axonal degeneration, which, along with inflammation, is one of the major pathological hallmarks of these diseases. Optical coherence tomography (OCT) is a non-invasive imaging tool that has been used in MS, NMOSD, and other diseases to quantify damage to the retina, including the ganglion cells and their axons. The fact that these are the only unmyelinated axons within the central nervous system (CNS) renders the afferent visual pathway an ideal model for studying axonal and neuronal degeneration in neurodegenerative diseases...
November 15, 2016: International Journal of Molecular Sciences
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