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https://www.readbyqxmd.com/read/28340257/cd8-positive-t-cell-leukoencephalitis-with-astrocytopathy-clinically-presenting-as-neuromyelitis-optica
#1
Diana L Thomas, Jody Manners, Daniel Marker, Joseph Mettenburg, Geoffrey Murdoch, Bryan Stevens, Guoji Wang, Clayton Wiley
We describe a novel disease entity with the clinical and radiologic presentation of neuromyelitis optica (NMO) and widespread CD8-positive T-cell leukoencephalitis and astrocytopathy. The 59-year-old female patient had a complex 2-year neurological history that included early changes in cognition and memory, progressive lower extremity motor dysfunction, and multimodal sensory involvement. MRI of the spinal cord showed increased T2 signal in the central cord extending from C2 through T4. MRI of the brain showed symmetric radial enhancement in periventricular deep white matter without evidence of demyelinating lesions...
March 16, 2017: Journal of Neuropathology and Experimental Neurology
https://www.readbyqxmd.com/read/28331329/successful-treatment-for-psychomotor-agitation-in-neuromyelitis-optica-spectrum-disorder-with-trazodone-risperidone-combination-a-case-report
#2
Zui Narita, Harumasa Takano, Tomiki Sumiyoshi
Neuromyelitis optica (NMO) is a relapsing disease that typically affects the spinal cord and optic nerves. So far, a few studies have reported pharmacologic treatment for psychiatric symptoms in patients with NMO spectrum disorder (NMOSD). However, no literature has described psychomotor agitation associated with the disease and its treatment. We report an 84-year-old woman with NMOSD whose psychomotor agitation was effectively treated with a combination of trazodone and risperidone. Our observation suggests the ability of augmentation of antipsychotic drugs with antidepressants to ameliorate psychotic symptoms associated with NMOSD...
2017: Neuropsychiatric Disease and Treatment
https://www.readbyqxmd.com/read/28306572/neuromyelitis-optica-and-neuromyelitis-optica-spectrum-disorders
#3
Romain Marignier, Alvaro Cobo Calvo, Sandra Vukusic
PURPOSE OF REVIEW: The discovery of highly specific auto-antibodies directed against the water channel aquaporin 4 was a quantum leap in the definition, classification and management of neuromyelitis optica (NMO). Herein, we propose an update on epidemiological, clinical and therapeutic advances in the field, underlining unmet needs. RECENT FINDINGS: Large-scale epidemiological studies have recently provided a more precise evaluation of NMO prevalence and a better stratification regarding ethnicity and sex...
March 16, 2017: Current Opinion in Neurology
https://www.readbyqxmd.com/read/28293214/different-phenotypes-at-onset-in-neuromyelitis-optica-spectrum-disorder-patients-with-aquaporin-4-autoimmunity
#4
Youming Long, Junyan Liang, Linzhan Wu, Shaopeng Lin, Cong Gao, Xiaohui Chen, Wei Qiu, Yu Yang, Xueping Zheng, Ning Yang, Min Gao, Yaotang Chen, Zhanhang Wang, Quanxi Su
BACKGROUND: Although rare, brain abnormalities without optic neuritis (ON) or transverse myelitis (TM) diagnosed with neuromyelitis optica spectrum disorder (NMOSD) have been reported in patients positive for the aquaporin-4 (AQP4) antibody. OBJECTIVE: To analyze demographic and clinical differences among NMOSD patients without ON or TM, those with either ON or TM, and patients with simultaneous ON and TM at disease onset. METHODS: In this retrospective study, patients who were positive for the AQP4 antibody, as detected using a cell-based assay, at the Second Affiliated Hospital of Guangzhou Medical University in China were recruited...
2017: Frontiers in Neurology
https://www.readbyqxmd.com/read/28284341/mog-antibody-associated-demyelinating-disease-of-the-cns-a-clinical-and-pathological-study-in-chinese-han-patients
#5
Lei Zhou, Yongheng Huang, Haiqing Li, Jie Fan, Jingzi Zhangbao, Hai Yu, Yuxin Li, Jiahong Lu, Chongbo Zhao, Chuanzhen Lu, Min Wang, Chao Quan
We aim to evaluate the clinical relevance of MOG-ab in a cohort of Chinese Han adults with CNS inflammatory demyelinating diseases (IDDs). MOG-ab and AQP4-ab were examined through a fixed cell based indirect immune-fluorescence assay in 86 patients with CNS-IDDs. MOG-ab was positive in 12 patients, while AQP4-ab was positive in 31 patients; none double positives. Optic neuritis (ON) was the most frequent symptom at onset (75.0%) or during the whole disease course (83.3%) of MOG-ab associated IDDs (MOG-IDDs); 79...
April 15, 2017: Journal of Neuroimmunology
https://www.readbyqxmd.com/read/28283439/interleukin-6-analysis-of-572-consecutive-csf-samples-from-neurological-disorders-a-special-focus-on-neuromyelitis-optica
#6
Akiyuki Uzawa, Masahiro Mori, Hiroki Masuda, Ryohei Ohtani, Tomohiko Uchida, Setsu Sawai, Satoshi Kuwabara
BACKGROUND: Increases of cerebrospinal fluid (CSF) interleukin (IL)-6 has been reported in various neurological disorders but has never been systematically analyzed. We compared the CSF IL-6 concentrations among various neurological disorders and to evaluate the significance of CSF IL-6 measurements for the diagnosis of neuromyelitis optica (NMO). METHODS: We retrospectively investigated the IL-6 concentrations of 572 consecutive CSF samples in patients with various neurological disorders...
March 7, 2017: Clinica Chimica Acta; International Journal of Clinical Chemistry
https://www.readbyqxmd.com/read/28283104/comparing-epidemiology-and-baseline-characteristic-of-multiple-sclerosis-and-neuromyelitis-optica-a-case-control-study
#7
COMPARATIVE STUDY
Sharareh Eskandarieh, Saharnaz Nedjat, Ibrahim Abdollahpour, Abdorreza Naser Moghadasi, Amir Reza Azimi, Mohammad Ali Sahraian
BACKGROUND: Autoimmune syndromes such as Multiple Sclerosis (MS) and Neuromyelitis Optica (NMO) are chronic, demyelinating diseases of the central nervous system which usually affect young adults. Both environmental risk factors and genetic susceptibility have been proposed to explain the etiology of these diseases. The aim of this study was to compare epidemiology and possible risk factors in MS and NMO in Tehran, Iran. METHOD: A population-based incident case-control study was performed in Tehran based on a study conducted on1513 MS patients from Iranian MS Society registry center of Tehran, from 2014 to 2015, and 83 NMO patients diagnosed with NMO in 2015 at Sina hospital, a tertiary care referral center in Tehran...
February 2017: Multiple Sclerosis and related Disorders
https://www.readbyqxmd.com/read/28281110/headache-in-neuromyelitis-optica
#8
REVIEW
Alina Masters-Israilov, Matthew S Robbins
PURPOSE OF REVIEW: Neuromyelitis optica (NMO) classically features a clinical presentation that includes longitudinally extensive transverse myelitis and optic neuritis. However, many other pathognomonic phenomena have more recently been described in patients diagnosed with NMO, including intractable hiccups, vomiting, and painful tonic spasms, but less has been reported regarding the relationship between NMO and headache. Though headache is well established as both a symptom and comorbidity of multiple sclerosis (MS), it has been much less described thus far in the NMO literature and warrants more careful evaluation...
April 2017: Current Pain and Headache Reports
https://www.readbyqxmd.com/read/28274814/the-role-of-aquaporin-4-in-synaptic-plasticity-memory-and-disease
#9
REVIEW
Jacqueline A Hubbard, Jenny I Szu, Devin K Binder
Since the discovery of aquaporins, it has become clear that the various mammalian aquaporins play critical physiological roles in water and ion balance in multiple tissues. Aquaporin-4 (AQP4), the principal aquaporin expressed in the central nervous system (CNS, brain and spinal cord), has been shown to mediate CNS water homeostasis. In this review, we summarize new and exciting studies indicating that AQP4 also plays critical and unanticipated roles in synaptic plasticity and memory formation. Next, we consider the role of AQP4 in Alzheimer's disease (AD), amyotrophic lateral sclerosis (ALS), Parkinson's disease (PD), multiple sclerosis (MS), neuromyelitis optica (NMO), epilepsy, traumatic brain injury (TBI), and stroke...
March 6, 2017: Brain Research Bulletin
https://www.readbyqxmd.com/read/28243026/initial-pattern-of-optic-nerve-enhancement-in-korean-patients-with-unilateral-optic-neuritis
#10
COMPARATIVE STUDY
Dae Yong Son, Kyung-Ah Park, Su Sie Seok, Ju-Yeun Lee, Sei Yeul Oh
PURPOSE: The purpose of this study was to demonstrate whether the pattern of optic nerve enhancement in magnetic resonance imaging (MRI) can help to differentiate between idiopathic optic neuritis (ON), neuromyelitis optica (NMO), and multiple sclerosis (MS) in unilateral ON. METHODS: An MRI of the brain and orbits was obtained in patients with acute unilateral ON. Patients with ON were divided into three groups: NMO, MS, and idiopathic ON. The length and location of the abnormal optic nerve enhancement were compared for ON eyes with and without NMO or MS...
February 2017: Korean Journal of Ophthalmology: KJO
https://www.readbyqxmd.com/read/28226075/neuromyelitis-optica-phenotypic-characteristics-in-a-brazilian-case-series
#11
Maria Cristina Del Negro, Patricia Beatriz Christino Marinho, Regina Maria Papais-Alvarenga
Objective: To describe the characteristics of 34 Brazilian NMO patients. To evaluate the contribution of the 2015 criteria to the diagnosis of NMO spectrum disorders (NMOSD) in 40 patients with longitudinal extensive transverse myelitis (LEMT). Methods: This is a retrospective, descriptive and analytic study. Results: Among NMO patients, there was a predominance of women, with onset in the fourth decade of life, and AQP4-IgG seropositivity in 73...
February 2017: Arquivos de Neuro-psiquiatria
https://www.readbyqxmd.com/read/28212662/marked-central-nervous-system-pathology-in-cd59-knockout-rats-following-passive-transfer-of-neuromyelitis-optica-immunoglobulin-g
#12
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/28203460/the-prevalence-of-anti-aquaporin-4-antibody-in-patients-with-idiopathic-inflammatory-demyelinating-diseases-presented-to-a-tertiary-hospital-in-malaysia-presentation-and-prognosis
#13
S Abdullah, W F Wong, C T Tan
Background. There have been inconsistent reports on the prevalence and pathogenicity of anti-Aquaporin 4 (AQP4) in patients presented with idiopathic inflammatory demyelinating diseases (IIDDs). Objective. To estimate the prevalence of anti-AQP4 antibody in patients with IIDDs presented to University Malaya Medical Centre in terms of patients' clinical and radiological presentations and prognoses. Methods. Retrospective data review of IIDDs patients presented from 2005 to 2015. Patients were classified into classical multiple sclerosis (CMS), opticospinal (OS) presentation, optic neuritis (ON), transverse myelitis (TM), brainstem syndrome (BS), and tumefactive MS...
2017: Multiple Sclerosis International
https://www.readbyqxmd.com/read/28184993/pathogenic-implications-of-cerebrospinal-fluid-barrier-pathology-in-neuromyelitis-optica
#14
Yong Guo, Stephen D Weigand, Bogdan F Popescu, Vanda A Lennon, Joseph E Parisi, Sean J Pittock, Natalie E Parks, Stacey L Clardy, Charles L Howe, Claudia F Lucchinetti
Pathogenic autoantibodies associated with neuromyelitis optica (NMO) induce disease by targeting aquaporin-4 (AQP4) water channels enriched on astrocytic endfeet at blood-brain interfaces. AQP4 is also expressed at cerebrospinal fluid (CSF)-brain interfaces, such as the pial glia limitans and the ependyma and at the choroid plexus blood-CSF barrier. However, little is known regarding pathology at these sites in NMO. Therefore, we evaluated AQP4 expression, microglial reactivity, and complement deposition at pial and ependymal surfaces and in the fourth ventricle choroid plexus in 23 autopsy cases with clinically and/or pathologically confirmed NMO or NMO spectrum disorder...
April 2017: Acta Neuropathologica
https://www.readbyqxmd.com/read/28174515/upregulation-of-bcl-2-and-its-promoter-signals-in-cd4-t-cells-during-neuromyelitis-optica-remission
#15
Tao Yang, Su Wang, Xiao Yang, Qi Zheng, Lei Wang, Qian Li, Mingyan Wei, Zongpan Du, Yongping Fan
The homeostatic balance between production and elimination of CD4+ T cells in peripheral blood plays an important role in patients with neuromyelitis optica (NMO). The objective of the present study was to evaluate the anti-apoptosis genes Bcl-2 and its promoter signal (nuclear factor kappa-light-chain-enhancer of activated B cells, NFκB) in CD4+ T cells. Healthy subjects (HS, n = 25) and patients with multiple sclerosis (MS) (n = 25) and NMO (n = 30) in remission were consecutively enrolled in this prospective study between May and December 2015...
2017: Frontiers in Neuroscience
https://www.readbyqxmd.com/read/28173728/metabolic-changes-in-normal-appearing-white-matter-in-patients-with-neuromyelitis-optica-and-multiple-sclerosis-a-comparative-magnetic-resonance-spectroscopy-study
#16
Yunyun Duan, Zheng Liu, Yaou Liu, Jing Huang, Zhuoqiong Ren, Zheng Sun, Hai Chen, Huiqing Dong, Jing Ye, Kuncheng Li
Background Previous studies with a small sample size have not reported metabolic changes in neuromyelitis optica (NMO). Metabolic changes, such as decreased N-acetylaspartate (NAA), are well-established in patients with multiple sclerosis (MS). It remains unknown whether different patterns of metabolic changes occur in NMO and MS. Purpose To investigate the metabolic changes in normal-appearing white matter (NAWM) in NMO, compared with MS patients and healthy controls (HC), and correlate these changes with clinical disability...
January 1, 2017: Acta Radiologica
https://www.readbyqxmd.com/read/28159705/demyelinating-syndrome-in-sle-encompasses-different-subtypes-do-we-need-new-classification-criteria-pooled-results-from-systematic-literature-review-and-monocentric-cohort-analysis
#17
Matteo Piga, Elisabetta Chessa, Maria Teresa Peltz, Alberto Floris, Alessandro Mathieu, Alberto Cauli
OBJECTIVE: To describe features of demyelinating syndrome (DS) in systemic lupus erythematosus (SLE). METHODS: A systematic review using a combination of Mesh terms in PubMed and a retrospective analysis of 343 adult patients with SLE were carried out to identify patients with DS. Retrieved cases were classified as affected with DS according to 1999 ACR nomenclature and attributed to SLE by applying the 2015 algorithm. DS defined according to the clinical but not temporal 1999 ACR criteria was classified as clinically isolated syndrome (CIS)...
January 31, 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/28157198/subregional-structural-and-connectivity-damage-in-the-visual-cortex-in-neuromyelitis-optica
#18
Huanhuan Cai, Jiajia Zhu, Ningnannan Zhang, Qiuhui Wang, Chao Zhang, Chunsheng Yang, Jie Sun, Xianting Sun, Li Yang, Chunshui Yu
Patients with neuromyelitis optica (NMO) have shown structural and functional impairments in the visual cortex. We aimed to characterize subregional grey matter volume (GMV) and resting-state functional connectivity (rsFC) changes in the visual cortex in NMO. Thirty-seven NMO patients and forty-two controls underwent structural and functional MRI scans. The GMV and rsFC of each visual subregion were compared between the groups. Compared with controls, NMO patients had GMV reductions in the bilateral V1, V2, V3d, VP, and LO and in the left V3A...
February 3, 2017: Scientific Reports
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
#19
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/28131216/monitoring-cd27-memory-b-cells-in-neuromyelitis-optica-spectrum-disorders-patients-treated-with-rituximab-results-from-a-bicentric-study
#20
M Cohen, G Romero, J Bas, M Ticchioni, M Rosenthal, R Lacroix, C Brunet, A Rico, J Pelletier, B Audoin, C Lebrun
BACKGROUND: Rituximab (RTX) is increasingly used in the treatment of neuromyelitis optica spectrum disorder (NMO-SD). Administration regimen is not consensual as there is no reliable biomarker of RTX efficacy. In most cases, after induction, RTX is administered systematically every 6months. OBJECTIVE: To assess efficacy and safety of a maintenance regimen based on CD19+ CD27+ memory B-cell (mBc) detection. METHODS: We conducted a study in two French centers, including patients with NMO-SD who received an induction therapy with RTX...
February 15, 2017: Journal of the Neurological Sciences
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