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Optic neuromyelitis

Edgar Carnero Contentti, Mariana De Virgiliis, Javier Pablo Hryb, Felisa Leguizamon, Julia Celso, José Luis Di Pace, Mónica Perassolo
BACKGROUND: Antibodies against aquaporin-4 (AQP4-ab) have diagnostic and prognostic value. However, little is known to date about their utility in the first event of optic neuritis (ON). OBJECTIVE: To evaluate the utility of systematic AQP4-ab determination in a retrospective cohort of patients with a first onset of ON. PATIENTS AND METHODS: All patients (n = 42) were tested for AQP4-ab in the following context: typical ON (TON) and atypical ON (AON)...
October 13, 2016: European Neurology
Sithara Ramdas, Danny Morrison, Michael Absoud, Ming Lim
Optic neuritis (ON) is an acquired disorder of the optic nerve due to inflammation, demyelination or degeneration. We report a child who presented with acute onset bilateral visual loss who, following a diagnosis of ON, was treated and had excellent visual recovery. Paediatric ON is considered to be different clinical entity to adult ON. Although in children ON is usually parainfectious or postinfectious, it can be the first presenting feature of multiple sclerosis or neuromyelitis optica spectrum disease. In this paper, we discuss the literature on treatment of ON and prediction of risk of recurrence...
October 4, 2016: BMJ Case Reports
Valentina Damato, Amelia Evoli, Raffaele Iorio
Importance: Neuromyelitis optica spectrum disorders (NMOSDs) are autoimmune astrocytopathies characterized by predominant involvement of the optic nerves and spinal cord. In most patients, an IgG autoantibody binding to astrocytic aquaporin 4, the principal water channel of the central nervous system, is detected. Rituximab, a chimeric monoclonal antibody specific for the CD20 B-lymphocyte surface antigen, has been increasingly adopted as a first-line off-label treatment for patients with NMOSDs...
September 26, 2016: JAMA 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
Tanawat Tengsirikomol, Sasitorn Siritho, Naraporn Prayoonwiwat
BACKGROUND AND PURPOSE: A few reports studied the time use to diagnose patient with neuromyelitis optica (NMO). The Aim of the study is to evaluate the interval from disease onset to the time when patients fulfilled the NMO diagnostic criteria 2006 in Thai. METHOD: A retrospective study of the NMO patients visiting the MS clinic and related disorders at Siriraj hospital was reviewed. RESULTS: There were 42 definite NMO. All were female. The most common first presentations were optic neuritis (ON) (45...
September 2016: Multiple Sclerosis and related Disorders
Xin Yang, Bruce R Ransom, Jian-Fang Ma
Neuromyelitis optica (NMO) is a recurrent inflammatory disease that preferentially targets the optic nerves and spinal cord. The presence of antibodies to the water channel protein aquaporin-4 (AQP4), expressed almost exclusively in astrocytes in the central nervous system (CNS), is a reliable biomarker for NMO. These antibodies, NMO-IgG, may be responsible for the sequential cascade of immune events, including IgG/IgM deposition, infiltration of granulocytes and complement-mediated cytotoxicity (i.e. astrocyte loss) and demyelination...
September 15, 2016: Journal of Neuroimmunology
Yanbing Wang, Hong Yan, Qixing Ding, Cunhua Mao, Yelong Shen, Guangbin Wang
Neuromyelitis optica (NMO) is an acute or subacute lesion of demyelinating disease involving the optic nerve and spinal cord, and imaging techniques and their effects have been the focus of investigations. The aim of the present study was to examine the value of three-dimensional double inversion recovery (3D-DIR) in the early differential diagnostic and prognostic evaluation of NMO. Forty-eight patients with suspicious NMO were included into the study and underwent a combination of serum NMO-IgG quantitative detection and 3D-DIR examination...
September 2016: Experimental and Therapeutic Medicine
Elena H Martinez-Lapiscina, Maria Sepulveda, Ruben Torres-Torres, Salut Alba-Arbalat, Sara Llufriu, Yolanda Blanco, Ana M Guerrero-Zamora, Nuria Sola-Valls, Santiago Ortiz-Perez, Pablo Villoslada, Bernardo Sanchez-Dalmau, Albert Saiz
No abstract text is available yet for this article.
September 2016: Therapeutic Advances in Neurological Disorders
Rogier Q Hintzen, Russell C Dale, Rinze F Neuteboom, Soe Mar, Brenda Banwell
Approximately one-third of children with an acquired demyelinating syndrome (ADS) will be diagnosed with multiple sclerosis (MS), either at onset according to the 2010 McDonald criteria, or on the basis of clinical or MRI evidence of relapsing disease, in the majority of patients within 2-4 years. ADS in adolescents, female patients, and patients with polyfocal deficits is associated with the highest likelihood of MS, while children with acute disseminated encephalomyelitis, those with documented preceding infection, and ADS presentation in young children more commonly portends a monophasic outcome...
August 30, 2016: Neurology
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
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
Anna-Lotta Kaivorinne, Janne Lintunen, Peter Baumann
Neuromyelitis optica is a rare inflammatory, demyelinating disease of the central nervous system that predominantly targets the optic nerves and spinal cord. Our case represents an unusual and severe course of neuromyelitis optica. Despite several forms of treatment, our patient died after a severe and short-term attack.
August 2016: Clinical Case Reports
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
Mai Fujikura, Kazuki Yokokawa, Hirohiko Shizukawa, Shun Shimohama
A 57-year-old man initially developed chest discomfort, nausea, vomiting, headache and low-grade fever, followed by paraplegia, sensory disturbance below level Th5 and bilateral visual loss. He was admitted to our hospital on the 15th day of illness. MRI short T1 inversion recovery image showed multiple longitudinal lesions in the spinal cord below C3, and T2-weighted image showed abnormal hyperintensity within the left optic nerve. Cerebrospinal fluid (CSF) analysis revealed marked pleocytosis (1,719/μl) and hypoglycorrhachia (CSF glucose; 20 mg/dl)...
August 31, 2016: Rinshō Shinkeigaku, Clinical Neurology
Inas F Aboobakar, Pradeep Mettu, Mays A El-Dairi
Nonglaucomatous cupping is commonly encountered in neuro-ophthalmic practice. However, the progression of clinical and imaging findings over time has not been well described. We present serial fundus photographs and spectral domain optical coherence tomography from a pediatric patient with neuromyelitis optic spectrum disorder, which demonstrated progression of both cupping and optic atrophy in the setting of normal intraocular pressure.
July 27, 2016: Journal of Neuro-ophthalmology: the Official Journal of the North American Neuro-Ophthalmology Society
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
Carl Ellenberger
This chapter can guide the use of imaging in the evaluation of common visual syndromes: transient visual disturbance, including migraine and amaurosis fugax; acute optic neuropathy complicating multiple sclerosis, neuromyelitis optica spectrum disorder, Leber hereditary optic neuropathy, and Susac syndrome; papilledema and pseudotumor cerebri syndrome; cerebral disturbances of vision, including posterior cerebral arterial occlusion, posterior reversible encephalopathy, hemianopia after anterior temporal lobe resection, posterior cortical atrophy, and conversion blindness...
2016: Handbook of Clinical Neurology
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