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Neuromyelitis Optica (NMO) Spectrum Disorders

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https://www.readbyqxmd.com/read/28780848/-clinical-characteristics-of-neuromyelitis-optica-spectrum-disorders-associated-with-syringomyelia
#1
Y G Wang, Y Q Wang, W Qiu, X Q Hu, Z Z Lu
Objective: To analyze the clinical characteristics of neuromyelitis optica spectrum disorders (NMOSD) associated with syringomyelia (SML). Methods: The clinical manifestations, laboratory parameters and imaging findings of 7 patients with NMOSD associated with SML during June 2008 to August 2016 from The Third Affiliated Hospital of Sun Yat-sen University were retrospectively analyzed. Results: Six patients were female and 1 was male, with ages ranging from 27-67 years, the course of the disease was 3-12 years, recurrence was 2-7 times, all the patients were cerebrospinal fluid oligoclonal bands (CSF OCB) negative, 5 patients were positive for aquaporin-4-antibody/NMO-IgG...
August 1, 2017: Zhonghua Yi Xue za Zhi [Chinese medical journal]
https://www.readbyqxmd.com/read/28750658/complement-regulator-cd59-prevents-peripheral-organ-injury-in-rats-made-seropositive-for-neuromyelitis-optica-immunoglobulin-g
#2
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/28748851/insights-into-initial-demyelinating-episodes-of-central-nervous-system-during-puerperium
#3
Qian Wu, Bo Chen, Na Liu, Yang Hu, Chao Pan, Ping Zhang, Zhou-Ping Tang, Bi-Tao Bu
BACKGROUND: Inflammatory demyelinating disease of central nervous system (CNS) is an inflammatory disease characterized by a high childbearing female predominance. Labor-related alterations for postpartum demyelinating attacks are not entirely clear. This study aimed to summarize clinical features of female patients of reproductive age with initial CNS inflammatory demyelinating attacks during puerperium. METHODS: Fourteen female patients with initial demyelinating events during puerperium between January 2013 and December 2016 were retrospectively studied...
August 5, 2017: Chinese Medical Journal
https://www.readbyqxmd.com/read/28748449/first-report-of-persistent-dengue-1-associated-autoimmune-neurological-disturbance-neuromyelitis-optica-spectrum-disorder
#4
Marzia Puccioni-Sohler, Alice M M Ornelas, Andrea S de Souza, Mauro Jorge Cabral-Castro, Jessyca T M A Ramos, Carolina Rosadas, Maria Cecilia F Salgado, Alexandre A Castiglione, Fernando Ferry, Jose Mauro Peralta, Carolina Moreira Voloch, Amilcar Tanuri, Fernanda Tovar-Moll, Renato Santana Aguiar
Dengue virus (DENV) causes immune-mediated diseases. Neurological involvement represents a severe condition that is rarely observed in DENV-1 infection. Neuromyelitis optica (NMO)/NMO spectrum disorders (NMOSD) are idiopathic immune-mediated demyelinating syndromes of the central nervous system. We report a 17-year-old female with oligosymptomatic DENV-1 viremia, diagnosed as NMOSD. Magnetic resonance imaging showed spinal cord and brainstem lesions. Antibody for aquaporin 4 was negative. DENV-1 RNA infection was detected by serial RT-PCR and confirmed by phylogenetic analysis in serum...
July 26, 2017: Journal of Neurovirology
https://www.readbyqxmd.com/read/28642888/aquaporin-4-antibodies-in-patients-treated-with-natalizumab-for-suspected-ms
#5
Anna Gahlen, Anne-Kathrin Trampe, Steffen Haupeltshofer, Marius Ringelstein, Orhan Aktas, Achim Berthele, Brigitte Wildemann, Ralf Gold, Sven Jarius, Ingo Kleiter
OBJECTIVE: To evaluate (1) the frequency of aquaporin-4 antibody (AQP4-ab)-seropositive cases among patients treated with natalizumab (NAT) and previously diagnosed with MS (MS(NAT)) in a nationwide cohort, (2) the clinical course of NAT-treated AQP4-ab-seropositive neuromyelitis optica spectrum disorder (NMOSD) patients (NMO(NAT)), (3) AQP4-ab titers in NMO(NAT) and AQP4-ab-seropositive NMOSD treated with other immunotherapies (NMO(IT)), and (4) immune mechanisms influencing disease activity in NMO(NAT)...
July 2017: Neurology® Neuroimmunology & Neuroinflammation
https://www.readbyqxmd.com/read/28640199/the-role-of-peripheral-cns-directed-antibodies-in-promoting-inflammatory-cns-demyelination
#6
REVIEW
Silke Kinzel, Martin S Weber
In central nervous system (CNS) demyelinating disorders, such as multiple sclerosis (MS), neuromyelitis optica (NMO) and related NMO-spectrum disorders (NMO-SD), a pathogenic role for antibodies is primarily projected into enhancing ongoing CNS inflammation by directly binding to target antigens within the CNS. This scenario is supported at least in part, by antibodies in conjunction with complement activation in the majority of MS lesions and by deposition of anti-aquaporin-4 (AQP-4) antibodies in areas of astrocyte loss in patients with classical NMO...
June 22, 2017: Brain Sciences
https://www.readbyqxmd.com/read/28608315/effectiveness-of-low-dose-of-rituximab-compared-with-azathioprine-in-chinese-patients-with-neuromyelitis-optica-an-over-2-year-follow-up-study
#7
Meini Zhang, Chuntao Zhang, Peng Bai, Huiru Xue, Guilian Wang
Neuromyelitis optical (NMO) and neuromyelitis optical spectrum disorder (NMOSD) are inflammatory autoimmune demyelination diseases affecting the central nervous system. We investigated the efficiency of low-dose rituximab treatment in 31 Chinese patients with NMO/NMOSD across a median period of 2.29 ± 0.97 years and azathioprine combined with corticosteroid treatment in 34 Chinese patients with NMO/NMOSD across a median period of 2.61 ± 0.94 years. Among the rituximab-treated patients, the mean Expanded Disability Status Scale (EDSS) was 5...
June 12, 2017: Acta Neurologica Belgica
https://www.readbyqxmd.com/read/28601281/association-of-cd58-gene-polymorphisms-with-nmo-spectrum-disorders-in-a-han-chinese-population
#8
Ju Liu, Ziyan Shi, Zhiyun Lian, Hongxi Chen, Qin Zhang, Huiru Feng, Xiaohui Miao, Qin Du, Hongyu Zhou
This study aimed to perform a comprehensive assessment of the association between CD58 polymorphisms and the risk of neuromyelitis optica spectrum disorders (NMOSD) in a Han Chinese population. Nine single-nucleotide polymorphisms (SNPs) were genotyped in 230 NMOSD patients and 487 healthy controls. Five SNPs were significantly associated with an increased risk of NMOSD (rs2300747, rs1335532, rs56302466, rs1016140, and rs12044852). The haplotype TAGCCCAA significantly increased the risk of NMOSD, while TATTACGG reduced the risk...
August 15, 2017: Journal of Neuroimmunology
https://www.readbyqxmd.com/read/28601277/late-onset-neuromyelitis-optica-mimicking-an-acute-stroke-in-an-elderly-patient
#9
Kushak Suchdev, Sara Razmjou, Praveen Venkatachalam, Omar A Khan, Wazim Mohamed, Mohammed S Ibrahim
Neuromyelitis optica spectrum disorder (NMOSD) was recently proposed as a unifying term for Neuromyelitis optica (NMO) and related syndromes to incorporate patients with NMO antibody but without the full clinical spectrum. NMO is a rare, demyelinating condition which predominantly affects females with a peak incidence in the third and fourth decade of life. We report a case of NMOSD in an elderly patient with extremely late onset (>80years) of disease. The patient presented with findings of sudden onset unilateral symptoms, which is extremely unusual for NMOSD...
August 15, 2017: Journal of Neuroimmunology
https://www.readbyqxmd.com/read/28571193/recurrent-longitudinally-extensive-myelitis-and-aquaporin-4-seronegativity-the-expanding-spectrum-of-neuromyelitis-optica
#10
Ravi Anadure, Coimbatore Sivasubramanian Narayanan, Govindaraj Varadraj
Neuromyelitis Optica Spectrum Disorder (NMOSD) is a recently proposed unifying term for Neuromyelitis Optica (NMO), also known as Devic's disease and related syndromes. It is a relapsing inflammatory demyelinating disease that most commonly affects optic nerves and the spinal cord, leading to sudden vision loss or weakness in one or both eyes, and loss of sensation and bladder function. Though inflammation may also involve the brain, the lesions seen in NMO are different from Multiple Sclerosis (MS) which has a similar clinical course...
April 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28567523/bystander-mechanism-for-complement-initiated-early-oligodendrocyte-injury-in-neuromyelitis-optica
#11
Lukmanee Tradtrantip, Xiaoming Yao, Tao Su, Alex J Smith, Alan S Verkman
Neuromyelitis optica spectrum disorder (herein called NMO) is an autoimmune inflammatory disease of the central nervous system in which immunoglobulin G antibodies against astrocyte water channel aquaporin-4 (AQP4-IgG) cause demyelination and neurological deficit. Injury to oligodendrocytes, which do not express AQP4, links the initiating pathogenic event of AQP4-IgG binding to astrocyte AQP4 to demyelination. Here, we report evidence for a complement 'bystander mechanism' to account for early oligodendrocyte injury in NMO in which activated, soluble complement proteins following AQP4-IgG binding to astrocyte AQP4 result in deposition of the complement membrane attack complex (MAC) on nearby oligodendrocytes...
July 2017: Acta Neuropathologica
https://www.readbyqxmd.com/read/28465700/neuromyelitis-optica-spectrum-disease-characteristics-in-isfahan-iran-a-cross-sectional-study
#12
Fereshte Ashtari, Ali Safaei, Vahid Shaygannejad, Mohammad Amin Najafi, Sahar Vesal
BACKGROUND: Neuromyelitis optica spectrum disease (NMOSD) is a severe autoimmune demyelinating disorder of the central nervous system that throughout epidemiological data, it has not been completely determined. The aim of this study was to assess characteristics of NMOSD patients in Isfahan as one of the most prevalent cities for multiple sclerosis in Iran. MATERIALS AND METHODS: Forty-five patients diagnosed as neuromyelitis optica (NMO) disease through 5 years enrolled in this study...
2017: Journal of Research in Medical Sciences: the Official Journal of Isfahan University of Medical Sciences
https://www.readbyqxmd.com/read/28427704/comparison-of-brain-and-spinal-cord-magnetic-resonance-imaging-features-in-neuromyelitis-optica-spectrum-disorders-patients-with-or-without-aquaporin-4-antibody
#13
Moli Fan, Ying Fu, Lei Su, Yi Shen, Kristofer Wood, Li Yang, Yaou Liu, Fu-Dong Shi
BACKGROUND: The spinal cord and brain measurements are rarely investigated in neuromyelitis optica (NMO) patients with and without antibodies to aquaporin-4 (AQP4), directly compared to multiple sclerosis (MS) patients. OBJECTIVES: To investigate magnetic resonance imaging (MRI) features of both brain and spinal cord in NMO patients with and without antibodies to AQP4, compared with MS patients and healthy controls (HC). METHODS: We recruited 55 NMO including 30 AQP4 (+) and 25 AQP4 (-), 25 MS and 25 HC...
April 2017: Multiple Sclerosis and related Disorders
https://www.readbyqxmd.com/read/28393003/syndrome-of-inappropriate-antidiuretic-hormone-secretion-associated-with-seronegative-neuromyelitis-optica-spectrum-disorder
#14
Shin Ju Oh, Chun Gyoo Ihm, Tae Won Lee, Jin Sug Kim, Da Rae Kim, Eun Ji Park, Su Woong Jung, Ji-Hoon Lee, Sung Hyuk Heo, Kyung Hwan Jeong
The syndrome of inappropriate antidiuretic hormone secretion (SIADH) is a potential cause of hyponatremia of the central nervous system (CNS). Although SIADH has been reported to be associated with many other central nervous disorders, its association with neuromyelitis optica (NMO) or NMO spectrum disorders are rare. NMO is a demyelinating disorder characterized by optic neuritis and transverse myelitis. Aquaporin-4 (AQP4), which is the target antigen for a NMO autoantibody, is the predominant CNS water channel...
March 2017: Kidney Research and Clinical Practice
https://www.readbyqxmd.com/read/28391784/neuromyelitis-optica-spectrum-disorders
#15
REVIEW
Tetsuya Akaishi, Ichiro Nakashima, Douglas Kazutoshi Sato, Toshiyuki Takahashi, Kazuo Fujihara
Neuromyelitis optica (NMO) is clinically characterized by severe optic neuritis and transverse myelitis, but recent studies with anti-aquaporin-4-antibody specific to NMO have revealed that the clinical spectrum is wider than previously thought. International consensus diagnostic criteria propose NMO spectrum disorders (NMOSD) as the term to define the entire spectrum including typical NMO, optic neuritis, acute myelitis, brain syndrome, and their combinations. NMOSD is now divided into anti-aquaporin-4-antibody-seropositive NMOSD and -seronegative NMOSD (or unknown serostatus)...
May 2017: Neuroimaging Clinics of North America
https://www.readbyqxmd.com/read/28331329/successful-treatment-for-psychomotor-agitation-in-neuromyelitis-optica-spectrum-disorder-with-trazodone-risperidone-combination-a-case-report
#16
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
#17
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...
June 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
#18
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/28226075/neuromyelitis-optica-phenotypic-characteristics-in-a-brazilian-case-series
#19
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
#20
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
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