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Neuromyelitis

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https://www.readbyqxmd.com/read/28106565/neuromyelitis-optica-in-a-patient-from-family-with-both-myotonic-dystrophy-type%C3%A2-1-and-2
#1
V Rakocevic-Stojanovic, S Peric, I Dujmovic, J Drulovic, J Pesovic, D Savic-Pavicevic
INTRODUCTION: The aim of this study was to present a family co-segregating myotonic dystrophy type 1 (DM1) and 2 (DM2), and one member affected with neuromyelitis optica (NMO). CASE REPORT: Index case underwent cataract surgery at age 39. Although she had no muscle symptoms, genetic testing revealed a DM2 mutation and a DM1 protomutation. The patient noticed difficulties in climbing stairs at age 47. Clinical examination showed mild muscle weakness, calf hypertrophy, mild myotonia and several multisystem signs...
January 20, 2017: Journal of Neuromuscular Diseases
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
#2
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/28104255/nmosd-triggered-by-yellow-fever-vaccination-an-unusual-clinical-presentation-with-segmental-painful-erythema
#3
F Schöberl, E Csanadi, O Eren, M Dieterich, T Kümpfel
Neuromyelitis Optica Spectrum Disorder (NMOSD) is an immune-mediated disease of the central nervous system with the presence of aquaporin 4-antibodies (AQP4-abs) in most cases. We describe a patient who developed NMOSD after a yellow fever vaccination. He presented to us with an unusual painful erythema Th7-9 triggered by touch in the respective skin area due to a cervical spinal cord lesion affecting the dorsolateral parts of C6/7. To our knowledge, this is the first case of NMOSD with such a clinical presentation expanding the clinical spectrum of NMOSD...
January 2017: Multiple Sclerosis and related Disorders
https://www.readbyqxmd.com/read/28100408/increased-cerebrospinal-fluid-progranulin-correlates-with-interleukin-6-in-the-acute-phase-of-neuromyelitis-optica-spectrum-disorder
#4
Akio Kimura, Masao Takemura, Kuniaki Saito, Ginette Serrero, Nobuaki Yoshikura, Yuichi Hayashi, Takashi Inuzuka
We examined progranulin (PGRN) levels in cerebrospinal fluid (CSF) samples during the acute phase in 15 patients with neuromyelitis optica spectrum disorders (NMOSD) and compared the results with those from 17 patients with multiple sclerosis (MS), 30 patients with other inflammatory neurological diseases (OIND), and 20 non-inflammatory controls (NIC). CSF PGRN levels of NMOSD patients were significantly higher than those of MS patients and NICs. These levels correlated with CSF interleukin-6 levels, CSF cell counts, CSF protein levels, improvements in the Expanded Disability Status Scale score, and affected total spinal cord lesion length in the NMOSD patients...
January 12, 2017: Journal of Neuroimmunology
https://www.readbyqxmd.com/read/28097302/association-of-extension-of-cervical-cord-lesion-and-area-postrema-syndrome-with-neuromyelitis-optica-spectrum-disorder
#5
Divyanshu Dubey, Sean J Pittock, Karl N Krecke, Eoin P Flanagan
No abstract text is available yet for this article.
January 9, 2017: JAMA Neurology
https://www.readbyqxmd.com/read/28092989/therapeutic-complement-inhibition-a-promising-approach-for-treatment-of-neuroimmunological-diseases
#6
Kjara S Pilch, Peter Spaeth, Yuki Nobuhiro, Benjamin R Wakerley
Autoimmunity is an important cause of disease both in the central and peripheral nervous systems. Aetiologies and clinical manifestations are complex and heterogeneous. Inappropriate control of complement activation at inappropriate sites has been recognized as a major determinant in several neurological conditions, including Guillain-Barré syndrome and neuromyelitis optica. In each case pathogenesis is thought to be associated with generation of autoantibodies which upon binding guide activation of the complement system to self-tissue...
January 17, 2017: Expert Review of Neurotherapeutics
https://www.readbyqxmd.com/read/28080220/predictors-of-response-to-first-line-immunosuppressive-therapy-in-neuromyelitis-optica-spectrum-disorders
#7
Su-Hyun Kim, Jae-Won Hyun, AeRan Joung, Eun Young Park, Jungnam Joo, Ho Jin Kim
BACKGROUND: Azathioprine (AZA) and mycophenolate mofetil (MMF) are the most commonly used first-line therapies for patients with neuromyelitis optica spectrum disorders (NMOSD). However, some patients experience a relapse following AZA or MMF treatment. OBJECTIVES: To identify factors that predict a response to AZA or MMF in NMOSD. METHODS: We retrospectively evaluated medical records from 116 patients who were initially treated with AZA or MMF for at least 6 months...
January 1, 2017: Multiple Sclerosis: Clinical and Laboratory Research
https://www.readbyqxmd.com/read/28080211/neuromyelitis-optica-spectrum-disorder-diagnostic-criteria-sensitivity-and-specificity-are-both-important
#8
Dean M Wingerchuk, Brian G Weinshenker
No abstract text is available yet for this article.
January 1, 2017: Multiple Sclerosis: Clinical and Laboratory Research
https://www.readbyqxmd.com/read/28079804/aquaporin-4-positive-neuromyelitis-optica-spectrum-disorders-secondary-to-thrombopenic-purpura-a-case-report
#9
Ying Wang, Qiaoyun Gong, Mingqin Zhu, Chao Lu, Li Sun, Jiachun Feng, Hongliang Zhang
RATIONALE: Neuromyelitis optica spectrum disorders (NMOSD) is considered as an immune-mediated disorder in the central nervous system (CNS). Numerous autoimmune diseases are frequently complicated with NMOSD and distinct clinical characteristics are noted in NMOSD patients with other autoimmune diseases. However, to our best knowledge, co-occurrence of NMOSD and thrombopenic purpura is rarely identified. PATIENT CONCERNS: We presented a rare case of a 72-year-old female with 6-year history of thrombopenic purpura, and 1-month history of blurred vision as well as chest zonethesia...
January 2017: Medicine (Baltimore)
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
#10
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/28069981/child-neurology-neuromyelitis-optica-spectrum-disorders
#11
Michael J Bradshaw, NgocHanh Vu, Tracy E Hunley, Tanuja Chitnis
No abstract text is available yet for this article.
January 10, 2017: Neurology
https://www.readbyqxmd.com/read/28069755/influence-of-the-gut-microbiome-on-autoimmunity-in-the-central-nervous-system
#12
REVIEW
Sara L Colpitts, Lloyd H Kasper
Autoimmune disorders of the CNS have complex pathogeneses that are not well understood. In multiple sclerosis and neuromyelitis optica spectrum disorders, T cells destroy CNS tissue, resulting in severe disabilities. Mounting evidence suggests that reducing inflammation in the CNS may start with modulation of the gut microbiome. The lymphoid tissues of the gut are specialized for the induction of regulatory cells, which are directly responsible for the suppression of CNS-damaging autoreactive T cells. Whether cause or effect, the onset of dysbiosis in the gut of patients with multiple sclerosis and neuromyelitis optica provides evidence of communication along the gut-brain axis...
January 15, 2017: Journal of Immunology: Official Journal of the American Association of Immunologists
https://www.readbyqxmd.com/read/28068933/screening-for-onconeural-antibodies-in-neuromyelitis-optica-spectrum-disorders
#13
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/28067584/short-segment-myelitis-as-a-first-manifestation-of-neuromyelitis-optica-spectrum-disorders
#14
So-Young Huh, Su-Hyun Kim, Jae-Won Hyun, In Hye Jeong, Min Su Park, Sang-Hyun Lee, Ho Jin Kim
BACKGROUND: Some patients with neuromyelitis optica spectrum disorders (NMOSD) present with spinal cord lesions extending fewer than three vertebral segments (short transverse myelitis, STM), hindering an early diagnosis. OBJECTIVE: We investigated the frequency and imaging characteristics of STM lesions in patients presenting with myelitis as an initial manifestation of NMOSD. METHODS: Patients seen at three referral hospitals in Korea between June 2005 and March 2015 who met the following inclusion criteria were recruited for review: seropositivity for aquaporin-4 antibody, initial presentation with myelitis and spinal cord magnetic resonance imaging (MRI) performed within 1 month of initial myelitis onset...
January 1, 2017: Multiple Sclerosis: Clinical and Laboratory Research
https://www.readbyqxmd.com/read/28060988/-neuromyelitis-optica-presenting-concomitantly-with-systemic-lupus-erythematosus-report-of-one-case
#15
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
#16
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/28058717/deletional-tolerance-prevents-aqp4-directed-autoimmunity-in-mice
#17
Anna-Lena Vogel, Benjamin Knier, Katja Lammens, Sudhakar Reddy Kalluri, Tanja Kuhlmann, Jeffrey L Bennett, Thomas Korn
Neuromyelitis optica (NMO) is an autoimmune disorder of the central nervous system (CNS) mediated by antibodies to water channel protein AQP4 expressed in astrocytes. The contribution of AQP4-specific T cells to the class switch recombination of pathogenic AQP4-specific antibodies and to the inflammation of the blood-brain-barrier is incompletely understood, as immunogenic naturally processed T-cell epitopes of AQP4 are unknown. By immunizing Aqp4(-/-) mice with full length murine AQP4 protein followed by recall with overlapping peptides, we here identify AQP4(201-220) as the major immunogenic IA(b) -restricted epitope of AQP4...
January 5, 2017: European Journal of Immunology
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
#18
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/28056870/anti-n-methyl-d-aspartate-receptor-nmdar-antibody-encephalitis-presents-in-atypical-types-and-coexists-with-neuromyelitis-optica-spectrum-disorder-or-neurosyphilis
#19
Kaiyu Qin, Wenqing Wu, Yuming Huang, Dongmei Xu, Lei Zhang, Bowen Zheng, Meijuan Jiang, Cheng Kou, Junhua Gao, Wurong Li, Jinglin Zhang, Sumei Wang, Yanfei Luan, Chaoling Yan, Dan Xu, Xinmei Zheng
BACKGROUND: Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a clinically heterogeneous disorder characterized by epileptic seizures, psychosis, dyskinesia, consciousness impairments, and autonomic instability. Symptoms are always various. Sometimes it presents in milder or incomplete forms. We report 4 cases of anti-NMDAR encephalitis with incomplete forms, 3 cases of which were accompanied by neuromyelitis optica spectrum disorder or neurosyphilis respectively. CASE PRESENTATION: A 33-year-old man presented with dysarthria, movement disorder and occasional seizures...
January 5, 2017: BMC Neurology
https://www.readbyqxmd.com/read/28054001/aquaporin-4-antibody-titration-in-nmo-patients-treated-with-rituximab-a-retrospective-study
#20
Paola Valentino, Fabiana Marnetto, Letizia Granieri, Marco Capobianco, Antonio Bertolotto
OBJECTIVE: We undertook an observational retrospective study to investigate the usefulness of aquaporin-4 (AQP4) antibodies (Ab) titration in the management of patients with neuromyelitis optica (NMO) treated with rituximab (RTX) by studying (1) the correlation between AQP4-Ab titer and disease activity, (2) the influence of RTX on antibody levels, and (3) the association between AQP4-Ab levels and responsiveness to RTX. METHODS: A cell-based assay was used for AQP4-Ab titration in 322 serum samples from 7 patients with NMO treated with RTX (median follow-up 65 months), according to a treatment-to-target approach...
March 2017: Neurology® Neuroimmunology & Neuroinflammation
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