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https://www.readbyqxmd.com/read/28109254/letm-presented-with-causalgia-and-ensued-by-sudden-death
#1
Rana Alnasser Alsukhni, Yasmin Aboras, Ziena Jriekh, Mahmoud Almalla, Ahmad Sheikh El-Kahwateya
BACKGROUND: Longitudinally Extensive Transverse Myelitis LETM is a specific pattern of myelitis wherein at least three continuous vertebral segments are involved. Characteristically, it is a defining feature of neuromyelitis optica NMO. However, it is described in many other etiologies. CASE PRESENTATION: We present a case of 60 year old male who presented with symptoms and signs of regional sympathetic dystrophy RSD followed by symptoms of myelitis. Spinal cord MRI revealed cervical LETM extending to the brainstem...
January 21, 2017: BMC Neurology
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/28080220/predictors-of-response-to-first-line-immunosuppressive-therapy-in-neuromyelitis-optica-spectrum-disorders
#5
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/28079804/aquaporin-4-positive-neuromyelitis-optica-spectrum-disorders-secondary-to-thrombopenic-purpura-a-case-report
#6
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/28068933/screening-for-onconeural-antibodies-in-neuromyelitis-optica-spectrum-disorders
#7
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
#8
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/28058965/clinical-characteristics-of-late-onset-neuromyelitis-optica-spectrum-disorder-a-multicenter-retrospective-study-in-korea
#9
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/28053819/thalamic-atrophy-contributes-to-low-slow-wave-sleep-in-neuromyelitis-optica-spectrum-disorder
#10
Lei Su, Yujuan Han, Rong Xue, Kristofer Wood, Fu-Dong Shi, Yaou Liu, Ying Fu
Slow wave sleep abnormality has been reported in neuromyelitis optica spectrum disorder (NMOSD), but mechanism for such abnormality is unknown. To determine the structural defects in the brain that account for the decrease of slow wave sleep in NMOSD patients. Thirty-three NMOSD patients and 18 matched healthy controls (HC) were enrolled. Polysomnography was used to monitor slow wave sleep and three-dimensional T1-weighted MRIs were obtained to assess the alterations of grey matter volume. The percentage of deep slow wave sleep decreased in 93% NMOSD patients...
December 2016: Aging and Disease
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
#11
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/28017203/pregnancy-in-neuromyelitis-optica-spectrum-disorder-a-multicenter-study-from-south-china
#12
Yanlu Huang, Yuge Wang, Yifan Zhou, Qiao Huang, Xiaobo Sun, Chen Chen, Ling Fang, Youming Long, Hui Yang, Honghao Wang, Caixia Li, Zhengqi Lu, Xueqiang Hu, Allan G Kermode, Wei Qiu
OBJECTIVE: This study aimed to assess the effect of pregnancy on the course of neuromyelitis optica spectrum disorder (NMOSD), and the effect of this disease on pregnancy outcomes. METHODS: Consecutive patients with NMOSD were recruited between September 2015 and April 2016 at an outpatient clinic from four referral institutes in South China. Demographic, clinical, and pregnancy data were retrieved by questionnaires to analyze the association between NMOSD and pregnancy, as well as the potential risk factors for relapse...
January 15, 2017: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/28017200/the-characteristics-of-spinal-imaging-in-different-types-of-demyelinating-diseases
#13
Chaisak Dumrikarnlert, Sasitorn Siritho, Pimwalai Chulapimphan, Chanon Ngamsombat, Chanjira Satukijchai, Naraporn Prayoonwiwat
BACKGROUND: Transverse myelitis is the common presentation in demyelinating conditions. OBJECTIVE: To determine the characteristics of spinal lesions among each type of demyelinating diseases. METHODS: Medical records and spinal imaging of patients who were [1] older than 18years, [2] had at least one attack of TM, [3] had available spinal MRI data and [4] were tested for aquaporin-4 antibody were included. RESULTS: One hundred and fifty-eight patients were eligible (27 clinically isolated syndrome [CIS], 38 MS, 55 seropositive neuromyelitis optica spectrum disorders [NMOSD], 9 seronegative NMOSD, and 29 idiopathic transverse myelitis [IDD-TM])...
January 15, 2017: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/28009104/deep-gray-matter-atrophy-in-neuromyelitis-optica-spectrum-disorder-and-multiple-sclerosis
#14
J-W Hyun, G Park, K Kwak, H-J Jo, A Joung, J-H Kim, S H Lee, S Kim, J-M Lee, S-H Kim, H J Kim
BACKGROUND AND PURPOSE: We investigated changes in deep gray matter (DGM) volume and its relationship to cognition and clinical factors in a large cohort of patients with neuromyelitis optica spectrum disorder (NMOSD) and compared them with results from multiple sclerosis (MS). METHODS: Brain magnetic resonance imaging (3 Tesla) and clinical data from 91 patients with NMOSD, 52 patients with MS and 44 healthy controls (HCs) were prospectively evaluated. Differences in DGM volumes were compared among groups...
February 2017: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/27994362/serological-markers-associated-with-neuromyelitis-optica-spectrum-disorders-in-south-india
#15
Lekha Pandit, Douglas Kazutoshi Sato, Sharik Mustafa, Toshiyuki Takahashi, Anitha D'Cunha, Chaithra Malli, Akshatha Sudhir, Kazuo Fujihara
BACKGROUND: Neuromyelitis optica spectrum disorders (NMOSDs) represent 20% of all demyelinating disorders in South India. No studies have determined the seroprevalence to both antibodies against aquaporin-4* and antimyelin oligodendrocyte glycoprotein antibody (anti-MOG+) in this population. OBJECTIVE: To identify and characterize seropositive patients for anti-aquaporin-4 antibody (anti-AQP4+) and anti-MOG+ in South India. MATERIALS AND METHODS: We included 125 consecutive patients (15 children) who were serologically characterized using live transfected cells to human M23-AQP4 or full-length MOG...
October 2016: Annals of Indian Academy of Neurology
https://www.readbyqxmd.com/read/27988051/the-clinical-characteristics-of-aqp4-antibody-positive-nmo-sd-in-a-large-cohort-of-chinese-han-patients
#16
Jingzi ZhangBao, Lei Zhou, Xiaoyang Li, Tongjia Cai, Jiahong Lu, Chuanzhen Lu, Chongbo Zhao, Chao Quan
We aim to summarize the clinical features of AQP4-ab-positive NMO/SD in a large Chinese Han cohort. The clinical data of 145 AQP4-ab-seropositive patients was retrospectively reviewed. 55.9% (81/145) of the patients were defined as NMO while 39.3% (57/145) were defined as NMOSD according to the criteria established in 2006 and 2007. The mean onset age was 34.4years and the female to male ratio was 8.7:1. The median disease duration was 57months. The median of "time to second attack" and "time to develop NMO" was 7 and 13months respectively...
January 15, 2017: Journal of Neuroimmunology
https://www.readbyqxmd.com/read/27951522/brain-lesion-distribution-criteria-distinguish-ms-from-aqp4-antibody-nmosd-and-mog-antibody-disease
#17
Maciej Juryńczyk, George Tackley, Yazhuo Kong, Ruth Geraldes, Lucy Matthews, Mark Woodhall, Patrick Waters, Wilhelm Kuker, Matthew Craner, Andrew Weir, Gabriele C DeLuca, Stephane Kremer, Maria Isabel Leite, Angela Vincent, Anu Jacob, Jérôme de Sèze, Jacqueline Palace
IMPORTANCE: Neuromyelitis optica spectrum disorders (NMOSD) can present with very similar clinical features to multiple sclerosis (MS), but the international diagnostic imaging criteria for MS are not necessarily helpful in distinguishing these two diseases. OBJECTIVE: This multicentre study tested previously reported criteria of '(1) at least 1 lesion adjacent to the body of the lateral ventricle and in the inferior temporal lobe; or (2) the presence of a subcortical U-fibre lesion or (3) a Dawson's finger-type lesion' in an independent cohort of relapsing-remitting multiple sclerosis (RRMS) and AQP4-ab NMOSD patients and also assessed their value in myelin oligodendrocyte glycoprotein (MOG)-ab positive and ab-negative NMOSD...
October 8, 2016: Journal of Neurology, Neurosurgery, and Psychiatry
https://www.readbyqxmd.com/read/27941536/lc-ms-ms-analysis-of-erythrocyte-thiopurine-nucleotides-and-their-association-with-genetic-variants-in-patients-with-neuromyelitis-optica-spectrum-disorders-taking-azathioprine
#18
Shenghui Mei, Xindi Li, Xiaoqing Gong, Xingang Li, Li Yang, Heng Zhou, Yonghong Liu, Anna Zhou, Leting Zhu, Xinghu Zhang, Zhigang Zhao
BACKGROUND: Azathioprine (AZA) is a first-line drug in treating neuromyelitis optica spectrum disorders (NMOSD). To exhibit its bioactivity, AZA needs to be converted to thiopurine nucleotides (TPNs) including 6-thioguanine nucleotides (6-TGNs) and 6-methylmercaptopurine nucleotides (6-MMPNs) that are affected by genetic polymorphisms. This study aims to develop an LC-MS/MS method for the analysis of erythrocyte concentrations of TPNs and to evaluate their associations with variants of various genes (MTHFR, TPMT, HLA, SLC29A1, SLC28A2, SLC28A3, ABCB1 and ABCC4) in patients with NMOSD...
November 29, 2016: Therapeutic Drug Monitoring
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
#19
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
#20
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
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