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Neuromyelitis Optica (NMO) Spectrum Disorders

Nadja Borisow, Ingo Kleiter, Anna Gahlen, Katrin Fischer, Klaus-Dieter Wernecke, Florence Pache, Klemens Ruprecht, Joachim Havla, Markus Krumbholz, Tania Kümpfel, Orhan Aktas, Marius Ringelstein, Christian Geis, Christoph Kleinschnitz, Achim Berthele, Bernhard Hemmer, Klemens Angstwurm, Robert Weissert, Jan-Patrick Stellmann, Simon Schuster, Martin Stangel, Florian Lauda, Hayrettin Tumani, Christoph Mayer, Lena Zeltner, Ulf Ziemann, Ralf A Linker, Matthias Schwab, Martin Marziniak, Florian Then Bergh, Ulrich Hofstadt-van Oy, Oliver Neuhaus, Alexander Winkelmann, Wael Marouf, Lioba Rückriem, Jürgen Faiss, Brigitte Wildemann, Friedemann Paul, Sven Jarius, Corinna Trebst, Kerstin Hellwig
BACKGROUND: Gender and age at onset are important epidemiological factors influencing prevalence, clinical presentation, and treatment response in autoimmune diseases. OBJECTIVE: To evaluate the impact of female sex and fertile age on aquaporin-4-antibody (AQP4-ab) status, attack localization, and response to attack treatment in patients with neuromyelitis optica (NMO) and its spectrum disorders (neuromyelitis optica spectrum disorder (NMOSD)). METHODS: Female-to-male ratios, diagnosis at last visit (NMO vs NMOSD), attack localization, attack treatment, and outcome were compared according to sex and age at disease or attack onset...
October 6, 2016: Multiple Sclerosis: Clinical and Laboratory Research
Konstantin Balashov
PURPOSE OF REVIEW: This article focuses on neuroimaging in multiple sclerosis (MS), the most common central nervous system (CNS) demyelinating disorder encountered by practicing neurologists. Less common adult demyelinating disorders and incidental subclinical white matter abnormalities that are often considered in the differential diagnosis of MS are also reviewed. RECENT FINDINGS: Advancements in neuroimaging techniques, eg, the application of ultrahigh-field MRI, are rapidly expanding the use of neuroimaging in CNS demyelinating disorders...
October 2016: Continuum: Lifelong Learning in Neurology
Amit Bar-Or, Larry Steinman, Jacinta M Behne, Daniel Benitez-Ribas, Peter S Chin, Michael Clare-Salzler, Donald Healey, James I Kim, David M Kranz, Andreas Lutterotti, Roland Martin, Sven Schippling, Pablo Villoslada, Cheng-Hong Wei, Howard L Weiner, Scott S Zamvil, Terry J Smith, Michael R Yeaman
Neuromyelitis optica spectrum disorder (NMO/SD) and its clinical variants have at their core the loss of immune tolerance to aquaporin-4 and perhaps other autoantigens. The characteristic phenotype is disruption of astrocyte function and demyelination of spinal cord, optic nerves, and particular brain regions. In this second of a 2-part article, we present further perspectives regarding the pathogenesis of NMO/SD and how this disease might be amenable to emerging technologies aimed at restoring immune tolerance to disease-implicated self-antigens...
October 2016: Neurology® Neuroimmunology & Neuroinflammation
Larry Steinman, Amit Bar-Or, Jacinta M Behne, Daniel Benitez-Ribas, Peter S Chin, Michael Clare-Salzler, Donald Healey, James I Kim, David M Kranz, Andreas Lutterotti, Roland Martin, Sven Schippling, Pablo Villoslada, Cheng-Hong Wei, Howard L Weiner, Scott S Zamvil, Michael R Yeaman, Terry J Smith
Neuromyelitis optica (NMO) and spectrum disorder (NMO/SD) represent a vexing process and its clinical variants appear to have at their pathogenic core the loss of immune tolerance to the aquaporin-4 water channel protein. This process results in a characteristic pattern of astrocyte dysfunction, loss, and demyelination that predominantly affects the spinal cord and optic nerves. Although several empirical therapies are currently used in the treatment of NMO/SD, none has been proven effective in prospective, adequately powered, randomized trials...
October 2016: Neurology® Neuroimmunology & Neuroinflammation
R Patejdl, M Wittstock, U K Zettl, K Jost, A Grossmann, J Prudlo
INTRODUCTION: Recently defined consensus criteria for the diagnosis of neuromyelitis optica spectrum disorders (NMOSD) allow establishing the diagnosis in patients without elevated AQP4-Ab and optic nerve involvement. According to the new extended definition, NMOSD is closely associated with extensive spinal cord inflammation occurring in the course of systemic autoimmune diseases as sarcoidosis or lupus erythematodes. NMOSD occurring in the course of hematological disease have not yet been reported in the literature...
September 2016: Multiple Sclerosis and related Disorders
Wenshu Qian, Koon Ho Chan, Edward S Hui, Chi Yan Lee, Yong Hu, Henry Ka-Fung Mak
Multiple sclerosis (MS) and neuromyelitis optica (NMO) are two common types of inflammatory demyelinating disease of the central nervous system. Early distinction of NMO from MS is crucial but quite challenging. In this study, 13 NMO spectrum disorder patients (Expanded Disability Status Scale (EDSS) of 3.0 ± 1.7, ranging from 2 to 6.5; disease duration of 5.3 ± 4.7 years), 17 relapsing-remitting MS patients (EDSS of 2.6 ± 1.4, ranging from 1 to 5.5; disease duration of 7.9 ± 7.8 years) and 18 healthy volunteers were recruited...
November 2016: NMR in Biomedicine
Silvia Tenembaum, Tanuja Chitnis, Ichiro Nakashima, Nicolas Collongues, Andrew McKeon, Michael Levy, Kevin Rostasy
Neuromyelitis optica (NMO) is a severe autoimmune disease of the CNS characterized by recurrent inflammatory events primarily involving the optic nerves and spinal cord. NMO is infrequent in children, but early recognition is important to start adequate treatment. In this article, we review the evolving diagnostic criteria of NMO and provide an update on the clinical and neuroimaging spectrum of the disorder in pediatric patients, including current knowledge on immunopathogenesis and treatment recommendations for children with NMO...
August 30, 2016: Neurology
Benedikt Grünewald, Jeffrey L Bennett, Klaus V Toyka, Claudia Sommer, Christian Geis
Neuromyelitis Optica Spectrum Disorders (NMOSD) are associated with autoantibodies (ABs) targeting the astrocytic aquaporin-4 water channels (AQP4-ABs). These ABs have a direct pathogenic role by initiating a variety of immunological and inflammatory processes in the course of disease. In a recently-established animal model, chronic intrathecal passive-transfer of immunoglobulin G from NMOSD patients (NMO-IgG), or of recombinant human AQP4-ABs (rAB-AQP4), provided evidence for complementary and immune-cell independent effects of AQP4-ABs...
2016: International Journal of Molecular Sciences
Shahd Hm Hamid, Liene Elsone, Kerry Mutch, Tom Solomon, Anu Jacob
BACKGROUND: The international panel for neuromyelitis optica (NMO) diagnosis has proposed diagnostic criteria for neuromyelitis optica spectrum disorders (NMOSD). OBJECTIVES: We assessed the impact of these criteria on diagnostic rates in a large cohort of patients. METHODS: We identified and applied the 2006 and 2015 criteria to all patients (n = 176) seen in the NMO and non-multiple sclerosis central nervous system demyelination clinic (part of the UK NMO service) from January 2013 to May 2015...
August 23, 2016: Multiple Sclerosis: Clinical and Laboratory Research
Jin Myoung Seok, Eun Bin Cho, Hye Lim Lee, Hye-Jin Cho, Ju-Hong Min, Kwang Ho Lee, Byoung Joon Kim
BACKGROUND: Individual attacks of neuromyelitis optica (NMO) are generally severe enough to cause disability even after the onset attack. We aimed to elucidate the clinical characteristics of disabling attacks at the onset of NMO. METHODS: We investigated the clinical characteristics at onset and at first relapse in patients with NMO or NMO spectrum disorder with seropositive for the anti-aquaporin-4 antibody. A disabling attack at onset (DAO) was defined as an onset attack in which, at best recovery (allowing up to one year), patients were unable to walk without assistance or were left functionally blind in at least one affected eye...
September 15, 2016: Journal of the Neurological Sciences
Jeffrey L Bennett
Neuromyelitis optica (NMO) is an autoimmune demyelinating disorder of the central nervous system (CNS) with predilection for the optic nerves and spinal cord. Since its emergence in the medical literature in the late 1800's, the diagnostic criteria for NMO has slowly evolved from the simultaneous presentation of neurologic and ophthalmic signs to a relapsing or monophasic CNS disorder defined by clinical, neuroimaging, and laboratory criteria. Due to the identification of a specific autoantibody response against the astrocyte water channel aquaporin-4 (AQP4) in the vast majority of affected individuals, the clinical spectrum of NMO has greatly expanded necessitating the development of new international criteria for the diagnosis of NMO spectrum disorder (NMOSD)...
September 2016: Journal of Neuro-ophthalmology: the Official Journal of the North American Neuro-Ophthalmology Society
Bleranda Zeka, Maria Hastermann, Nathalie Kaufmann, Kathrin Schanda, Marko Pende, Tatsuro Misu, Paulus Rommer, Kazuo Fujihara, Ichiro Nakashima, Charlotte Dahle, Fritz Leutmezer, Markus Reindl, Hans Lassmann, Monika Bradl
Neuromyelitis optica/spectrum disorder (NMO/SD) is a severe, inflammatory disease of the central nervous system (CNS). In the majority of patients, it is associated with the presence of pathogenic serum autoantibodies (the so-called NMO-IgGs) directed against the water channel aquaporin 4 (AQP4), and with the formation of large, astrocyte-destructive lesions in spinal cord and optic nerves. A large number of recent studies using optical coherence tomography (OCT) demonstrated that damage to optic nerves in NMO/SD is also associated with retinal injury, as evidenced by retinal nerve fiber layer (RNFL) thinning and microcystic inner nuclear layer abnormalities...
2016: Acta Neuropathologica Communications
Osamu Higuchi, Shunya Nakane, Waka Sakai, Yasuhiro Maeda, Masaaki Niino, Toshiyuki Takahashi, Toshiyuki Fukazawa, Seiji Kikuchi, Kazuo Fujihara, Hidenori Matsuo
OBJECTIVES: To examine anti-KIR4.1 antibodies by 2 different assays in Japanese patients with multiple sclerosis (MS) or neuromyelitis optica (NMO). METHODS: One hundred sixty serum samples from 57 patients with MS, 40 patients with NMO/NMO spectrum disorder (NMOSD), and 50 healthy controls (all were Japanese) were tested with ELISA using a synthetic peptide of the first extracellular portion of human KIR4.1. In addition, we attempted to detect anti-KIR4.1 immunoglobulin G in the serum by the luciferase immunoprecipitation systems (LIPS) with the full length of human KIR4...
October 2016: Neurology® Neuroimmunology & Neuroinflammation
Jennifer Massey, Michael E Buckland, Yael Barnett, Ian Sutton
Neuromyelitis optica (NMO) spectrum disorder typically associates with a pathognomonic antibody response directed against aquaporin 4 and a unique immunopathological signature characterised by loss of aquaporin 4 expression. We describe a tumefactive presentation of NMO in which a biopsy specimen demonstrated active demyelination and reactive astrocytes with preserved surface aquaporin 4 immunoreactivity and dystrophic processes. While the astrocytic pathology is more typical of classical multiple sclerosis, a positive NMO-IgG (1:80) and subsequent clinical presentation with an area postrema syndrome is consistent with a diagnosis of NMO spectrum disorder...
2016: BMJ Case Reports
Ismael Sánchez Gomar, María Díaz Sánchez, Antonio José Uclés Sánchez, José Luis Casado Chocán, Nela Suárez-Luna, Reposo Ramírez-Lorca, Javier Villadiego, Juan José Toledo-Aral, Miriam Echevarría
Detection of IgG anti-Aquaporin-4 (AQP4) in serum of patients with Neuromyelitis optica syndrome disorders (NMOSD) has improved diagnosis of these processes and differentiation from Multiple sclerosis (MS). Recent findings also claim that a subgroup of patients with NMOSD, serum negative for IgG-anti-AQP4, present antibodies anti-AQP1 instead. Explore the presence of IgG-anti-AQP1 using a previously developed cell-based assay (CBA) highly sensitive to IgG-anti-AQP4. Serum of 205 patients diagnosed as NMOSD (8), multiple sclerosis (94), optic neuritis (39), idiopathic myelitis (29), other idiopathic demyelinating disorders of the central nervous system (9), other neurological diseases (18) and healthy controls (8), were used in a CBA over fixed HEK cells transfected with hAQP1-EGFP or hM23-AQP4-EGFP, treated with Triton X-100 and untreated...
2016: International Journal of Molecular Sciences
Teresa M Crout, Laura P Parks, Vikas Majithia
Neuromyelitis optica (NMO) and neuromyelitis optica spectrum disorders (NMOSD), previously known as Devic's syndrome, are a group of inflammatory disorders of the central nervous system (CNS) characterized by severe, immune-mediated demyelination and axonal damage, predominantly targeting optic nerves and the spinal cord typically associated with a disease-specific serum NMO-IgG antibody that selectively binds aquaporin-4 (AQP4). The classic and best-defined features of NMOSD include acute attacks of bilateral or rapidly sequential optic neuritis (leading to visual loss) or transverse myelitis (often causing limb weakness and bladder dysfunction) or both with a typically relapsing course...
August 2016: Current Rheumatology Reports
Azusa Oshiro, Sadao Nakamura, Kunihito Tamashiro, Kazuo Fujihara
A 10-year-old boy developed bilateral optic neuritis and myelitis after a suspected viral infection and appendicitis. Magnetic resonance imaging (MRI) showed multiple lesions in both optic nerves, the optic chiasm, and the spinal cord. Several small lesions were also observed in the cerebellum and cerebral white matter. The serum tested negative for anti-aquaporin (AQP)-4 antibody and positive for anti-myelin oligodendrocyte glycoprotein (MOG) antibody. The diagnosis was neuromyelitis optica spectrum disorder (NMOSD)...
May 2016: No to Hattatsu. Brain and Development
Ameneh Zare-Shahabadi, Hamidreza Ghalyanchi Langroodi, Amir Reza Azimi, Mohammad Ali Sahraian, Mohammad Hossein Harirchian, Seyed Mohammad Baghbanian
Neuromyelitis optica (NMO) and the associated NMO spectrum disorders are demyelinating disorders affecting the spinal cord and optic nerves. It has prominent female predominance and many of these patients are in their childbearing years. As pregnancy seems to have a major impact on this disease course, in this review, recent studies with a focus on this disease and pregnancy and safety of available treatment options during this period are discussed.
June 15, 2016: Acta Neurologica Belgica
Eiji Matsusue, Yoshio Fujihara, Yutaka Suto, Shotaro Takahashi, Kenichiro Tanaka, Hiroyuki Nakayasu, Kazuhiko Nakamura, Toshihide Ogawa
Neuromyelitis optica (NMO) is characterized by attacks of optic neuritis and longitudinally extensive transverse myelitis. Cases positive for aquaporin 4 antibodies are classified to NMO spectrum disorder (NMOSD) which includes cases with optic neuritis, transverse myelitis, or with brain lesions typical of NMO. Our three cases with NMO/NMOSD revealed five imaging features: (i) extensive transverse cord lesions, extending more than three vertebral segments, partially persisting as cavitation; (ii) periependymal lesions; (iii) lesions of the corticospinal tracts; (iv) extensive and confluent hemispheric white matter lesions reflecting vasogenic edema and partially involving the cerebral cortices and basal ganglia; and (v) two patterns of serial hemispheric white matter lesions: one is cavitation and another is partial regression or disappearance...
May 2016: Acta Radiologica Open
Sona Narula, Brenda Banwell
PURPOSE OF REVIEW: This review summarizes a general approach to pediatric demyelination as well as specific features of each of the acquired demyelinating syndromes to help clinicians in their evaluation of children with these disorders. Case studies are included to illustrate the expanding phenotype of many of these syndromes. RECENT FINDINGS: With the creation of consensus definitions for the pediatric acquired demyelinating syndromes, recognition of demyelination in children has increased, as has understanding of the clinical and radiologic features, prognosis, and response to treatment...
August 2016: Continuum: Lifelong Learning in Neurology
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