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https://www.readbyqxmd.com/read/28814166/late-presentation-of-nmosd-as-rapidly-progressive-leukoencephalopathy-with-atypical-clinical-and-radiological-findings
#1
Elia Sechi, Alberto Addis, Lucia Batzu, Sara Mariotto, Sergio Ferrari, Maurizio Conti, GianPietro Sechi
Brain abnormalities in neuromyelitis optica spectrum disorder (NMOSD) are highly heterogeneous and often non-specific. Extensive white matter involvement has been described and frequently manifests with encephalopathy requiring prompt intervention. Rarely, this may represent the only manifestation at onset without concurrent suggestive features of the disease, thus making diagnosis challenging. NMOSD may potentially occur at any age, but it seems that this disorder has distinctive clinical features in the elderly...
August 1, 2017: Multiple Sclerosis: Clinical and Laboratory Research
https://www.readbyqxmd.com/read/28803524/endocrinopathies-in-paediatric-onset-neuromyelitis-optica-spectrum-disorder-with-aquaporin-4-aqp4-antibody
#2
Yael Hacohen, Silvia Messina, Hoong-Wei Gan, Sukhvir Wright, Saleel Chandratre, Maria Isabel Leite, Penny Fallon, Angela Vincent, Olga Ciccarelli, Evangeline Wassmer, Ming Lim, Jacqueline Palace, Cheryl Hemingway
The involvement of the diencephalic regions in neuromyelitis optica spectrum disorder (NMOSD) may lead to endocrinopathies. In this study, we identified the following endocrinopathies in 60% (15/25) of young people with paediatric-onset aquaporin 4-Antibody (AQP4-Ab) NMOSD: morbid obesity ( n = 8), hyperinsulinaemia ( n = 5), hyperandrogenism ( n = 5), amenorrhoea ( n = 5), hyponatraemia ( n = 4), short stature ( n = 3) and central hypothyroidism ( n = 2) irrespective of hypothalamic lesions...
August 1, 2017: Multiple Sclerosis: Clinical and Laboratory Research
https://www.readbyqxmd.com/read/28780848/-clinical-characteristics-of-neuromyelitis-optica-spectrum-disorders-associated-with-syringomyelia
#3
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/28770374/mog-antibody-related-disorders-common-features-and-uncommon-presentations
#4
Álvaro Cobo-Calvo, Anne Ruiz, Hyacintha D'Indy, Anne-Lise Poulat, Maryline Carneiro, Nicolas Philippe, Françoise Durand-Dubief, Kumaran Deiva, Sandra Vukusic, Vincent Desportes, Romain Marignier
Myelin oligodendrocyte glycoprotein antibodies (MOG-Ab) have been reported in acute demyelinating encephalomyelitis (ADEM), optic neuritis (ON), and neuromyelitis optica spectrum disorders (NMOSD) in adults and pediatrics. We aimed to delineate the common features of MOG-Ab-related disorders in children and adults, and report uncommon presentations. Twenty-seven consecutive pediatric and adult patients testing positive for MOG-Ab, with a minimum follow-up of 6 months, were included. Comprehensive epidemiological, clinical, radiological, and laboratory data were retrospectively analyzed...
August 2, 2017: Journal of Neurology
https://www.readbyqxmd.com/read/28768844/prognostic-relevance-of-mog-antibodies-in-children-with-an-acquired-demyelinating-syndrome
#5
Eva-Maria Hennes, Matthias Baumann, Kathrin Schanda, Banu Anlar, Barbara Bajer-Kornek, Astrid Blaschek, Sigrid Brantner-Inthaler, Katharina Diepold, Astrid Eisenkölbl, Thaddäus Gotwald, Georgi Kuchukhidze, Ursula Gruber-Sedlmayr, Martin Häusler, Romana Höftberger, Michael Karenfort, Andrea Klein, Johannes Koch, Verena Kraus, Christian Lechner, Steffen Leiz, Frank Leypoldt, Simone Mader, Klaus Marquard, Imke Poggenburg, Daniela Pohl, Martin Pritsch, Markus Raucherzauner, Mareike Schimmel, Charlotte Thiels, Daniel Tibussek, Silvia Vieker, Carolin Zeches, Thomas Berger, Markus Reindl, Kevin Rostásy
OBJECTIVE: To assess the prognostic value of MOG antibodies (abs) in the differential diagnosis of acquired demyelinating syndromes (ADS). METHODS: Clinical course, MRI, MOG-abs, AQP4-abs, and CSF cells and oligoclonal bands (OCB) in children with ADS and 24 months of follow-up were reviewed in this observational prospective multicenter hospital-based study. RESULTS: Two hundred ten children with ADS were included and diagnosed with acute disseminated encephalomyelitis (ADEM) (n = 60), neuromyelitis optica spectrum disorder (NMOSD) (n = 12), clinically isolated syndrome (CIS) (n = 101), and multiple sclerosis (MS) (n = 37) after the first episode...
August 2, 2017: Neurology
https://www.readbyqxmd.com/read/28756870/anti-c1q-autoantibodies-in-patients-with-neuromyelitis-optica-spectrum-disorders
#6
Nobuaki Yoshikura, Akio Kimura, Yuichi Hayashi, Takashi Inuzuka
We examined anti-complement C1q (C1q) autoantibody levels in serum and cerebrospinal fluid (CSF) samples of patients with neuromyelitis optica spectrum disorders (NMOSD). We analyzed the correlations between anti-C1q autoantibody levels and the clinical and other CSF characteristics of NMOSD. Serum and CSF anti-C1q autoantibody levels increased during the acute phase of NMOSD, reverting to the same levels as controls during remission. CSF anti-C1q autoantibody levels during the acute phase correlated with several markers reflecting disease severity, Expanded Disability Status Scale worsening, spinal cord lesion length in cases with myelitis, CSF protein and interleukin-6 levels, and CSF/serum albumin ratios...
July 19, 2017: Journal of Neuroimmunology
https://www.readbyqxmd.com/read/28748449/first-report-of-persistent-dengue-1-associated-autoimmune-neurological-disturbance-neuromyelitis-optica-spectrum-disorder
#7
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/28746356/metabolomic-profiling-of-csf-in-multiple-sclerosis-and-neuromyelitis-optica-spectrum-disorder-by-nuclear-magnetic-resonance
#8
Hyun-Hwi Kim, In Hye Jeong, Ja-Shil Hyun, Byung Soo Kong, Ho Jin Kim, Sung Jean Park
Multiple sclerosis (MS) and neuromyelitis optica spectrum disorder (NMOSD) are inflammatory diseases of the central nervous system. Although several studies have characterized the metabolome in the cerebrospinal fluid (CSF) from MS and NMOSD patients, comparative analyses between them and between the relapse and the remission of each disease have not been performed. Both univariate and multivariate analyses were used to compare 1H-NMR spectra of CSF from MS, NMOSD, and healthy controls (HCs). The statistical analysis showed alterations of eight metabolites that were dependent on the disease...
2017: PloS One
https://www.readbyqxmd.com/read/28725551/cervical-cord-myelin-water-imaging-shows-degenerative-changes-over-one-year-in-multiple-sclerosis-but-not-neuromyelitis-optica-spectrum-disorder
#9
Anna J E Combes, Lucy Matthews, Jimmy S Lee, David K B Li, Robert Carruthers, Anthony L Traboulsee, Gareth J Barker, Jacqueline Palace, Shannon Kolind
Spinal cord pathology is a feature of both neuromyelitis optica spectrum disorder (NMOSD) and relapsing-remitting multiple sclerosis (MS). While subclinical disease activity has been described in MS using quantitative magnetic resonance imaging measures, current evidence suggests that neurodegeneration is absent between relapses in NMOSD, although most evidence comes from brain studies. We aimed to assess cross-sectional differences and longitudinal changes in myelin integrity in relapse-free MS and NMOSD subjects over one year...
2017: NeuroImage: Clinical
https://www.readbyqxmd.com/read/28699327/acute-onset-severe-occipital-neuralgia-associated-with-high-cervical-lesion-in-patients-with-neuromyelitis-optica-spectrum-disorder
#10
Yuichi Hayashi, Akihiro Koumura, Megumi Yamada, Akio Kimura, Toshirou Shibata, Takashi Inuzuka
OBJECTIVE: To address occipital neuralgia in patients with neuromyelitis optica spectrum disorder (NMOSD). BACKGROUND: NMOSD is an inflammatory demyelinating disease that commonly presents with pain; however, headache symptoms have received little attention. METHODS: We presented three cases of NMOSD in which the patients experienced acute-onset, severe, and steroid-responsive occipital neuralgia. All patients provided consent to use their demographic and imaging data retrospectively...
July 2017: Headache
https://www.readbyqxmd.com/read/28684532/longitudinal-analysis-of-myelin-oligodendrocyte-glycoprotein-antibodies-in-cns-inflammatory-diseases
#11
Jae-Won Hyun, Mark R Woodhall, Su-Hyun Kim, In Hye Jeong, Byungsoo Kong, Gayoung Kim, Yeseul Kim, Min Su Park, Sarosh R Irani, Patrick Waters, Ho Jin Kim
BACKGROUND: We evaluated the seroprevalence of myelin oligodendrocyte glycoprotein immunoglobulin G1 (MOG-IgG) and associated clinical features of patients from a large adult-dominant unselected cohort with mainly relapsing central nervous system (CNS) inflammatory diseases. We also investigate the clinical relevance of MOG-IgG through a longitudinal analysis of serological status over a 2-year follow-up period. METHODS: Serum samples from 505 patients with CNS inflammatory diseases at the National Cancer Center were analysed using cell-based assays for MOG-IgG and aquaporin-4 immunoglobulin G (AQP4-IgG)...
July 6, 2017: Journal of Neurology, Neurosurgery, and Psychiatry
https://www.readbyqxmd.com/read/28679367/relationship-between-azathioprine-metabolites-and-therapeutic-efficacy-in-chinese-patients-with-neuromyelitis-optica-spectrum-disorders
#12
Xindi Li, Shenghui Mei, Xiaoqing Gong, Heng Zhou, Li Yang, Anna Zhou, Yonghong Liu, Xingang Li, Zhigang Zhao, Xinghu Zhang
BACKGROUND: Neuromyelitis optica spectrum disorders (NMOSD) are demyelinating autoimmune diseases in the central nervous system (CNS) that are characterized by a high relapse rate and the presence of anti-aquaporin 4 antibodies (AQP4-IgG) in the serum. Azathioprine (AZA) is a first-line immunomodulatory drug that is widely used for the treatment of patients with NMOSD. However, the efficacy and safety of AZA vary in different individuals. METHOD: Thirty-two patients with NMOSD who regularly took AZA were enrolled in the study at Beijing Tiantan Hospital, Capital Medical University...
July 5, 2017: BMC Neurology
https://www.readbyqxmd.com/read/28674367/neuromyelitis-optica-spectrum-disorder-with-recurrent-intracranial-hemorrhage
#13
Hiroaki Yaguchi, Yasunori Mito, Ikkei Ohashi, Taichi Nomura, Ichiro Yabe, Yasutaka Tajima
The patient was a woman without hypertension who had previously experienced intracranial hemorrhage twice at 48 and 56 years of age. At 59 years of age, she was diagnosed with neuromyelitis optica spectrum disorder (NMOSD) based on the presence of a brain stem lesion and the detection of anti-aquaporin 4 (AQP4) antibodies. After 5 months of continuous treatment with prednisolone (15 mg/day), she presented with optic neuritis and intracranial bleeding. A recurrent attack of NMOSD and intracranial hemorrhage were concurrently diagnosed...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28670343/differential-diagnosis-of-neuromyelitis-optica-spectrum-disorders
#14
REVIEW
Sung-Min Kim, Seong-Joon Kim, Haeng Jin Lee, Hiroshi Kuroda, Jacqueline Palace, Kazuo Fujihara
Neuromyelitis optica spectrum disorder (NMOSD) is an inflammatory disorder of the central nervous system (CNS) mostly manifesting as optic neuritis and/or myelitis, which are frequently recurrent/bilateral or longitudinally extensive, respectively. As the autoantibody to aquaporin-4 (AQP4-Ab) can mediate the pathogenesis of NMOSD, testing for the AQP4-Ab in serum of patients can play a crucial role in diagnosing NMOSD. Nevertheless, the differential diagnosis of NMOSD in clinical practice is often challenging despite the phenotypical and serological characteristics of the disease because: (1) diverse diseases with autoimmune, vascular, infectious, or neoplastic etiologies can mimic these phenotypes of NMOSD; (2) patients with NMOSD may only have limited clinical manifestations, especially in their early disease stages; (3) test results for AQP4-Ab can be affected by several factors such as assay methods, serologic status, disease stages, or types of treatment; (4) some patients with NMOSD do not have AQP4-Ab; and (5) test results for the AQP4-Ab may not be readily available for the acute management of patients...
July 2017: Therapeutic Advances in Neurological Disorders
https://www.readbyqxmd.com/read/28657431/csf-and-clinical-data-are-useful-in-differentiating-cns-inflammatory-demyelinating-disease-from-cns-lymphoma
#15
Ryotaro Ikeguchi, Yuko Shimizu, Satoru Shimizu, Kazuo Kitagawa
BACKGROUND: It is often difficult to diagnose central nervous system (CNS) inflammatory demyelinating diseases (IDDs) because they are similar to CNS lymphoma and glioma. OBJECTIVE: To evaluate whether cerebrospinal fluid (CSF) analysis can differentiate CNS IDDs from CNS lymphoma and glioma. METHODS: We measured CSF cell counts; concentrations of proteins, glucose, interleukin (IL)-6, IL-10, soluble IL-2 receptor (sIL-2R), and myelin basic protein; and IgG index in patients with multiple sclerosis (MS, n = 64), neuromyelitis optica spectrum disorder (NMOSD, n = 35), tumefactive demyelinating lesion (TDL, n = 17), CNS lymphoma ( n = 12), or glioma ( n = 10)...
June 1, 2017: Multiple Sclerosis: Clinical and Laboratory Research
https://www.readbyqxmd.com/read/28643955/diffusion-tensor-imaging-of-normal-appearing-white-matter-in-patients-with-neuromyelitis-optica-spectrum-disorder-and-multiple-sclerosis
#16
S-H Kim, K Kwak, J-W Hyun, A Joung, S H Lee, Y-H Choi, J-M Lee, H J Kim
BACKGROUND AND PURPOSE: The occult changes in normal-appearing white matter (NAWM) were investigated and compared amongst patients with neuromyelitis optica spectrum disorder (NMOSD), patients with multiple sclerosis (MS) and healthy controls (HCs) by applying tract-based spatial statistics to diffusion tensor imaging (DTI) data. METHODS: Diffusion tensor imaging was performed with a 3-T scanner in 93 patients with NMOSD, 53 patients with MS and 43 HCs. Voxel-wise statistical analyses of the DTI data were performed using tract-based spatial statistics...
July 2017: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/28642888/aquaporin-4-antibodies-in-patients-treated-with-natalizumab-for-suspected-ms
#17
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/28641774/membranous-nephropathy-with-neuromyelitis-optica-spectrum-disorder
#18
Xiangling Li, YanQiang Wang
Membranous nephropathy (MN) accompanying Neuromyelitis optica spectrum disorders (NMOSD) has rarely been described previously. We recently presented a 45-year-old Chinese male presenting with recurrent lower extremity pitting edema, or eyelid edema, proteinuria and hyperlipidemia. especially intractable hiccup and vomiting, painful tonic spasm (PTS) as the revealing symptom of a demyelinating disorder of central nervous system. The kindey biopsy specimen showed MN stage 2. Serological testing revealed antibodies AQP4, MRI head and spine revealed medulla oblongata and C1-C2 cervical vertebra lesions...
July 2017: Multiple Sclerosis and related Disorders
https://www.readbyqxmd.com/read/28626410/neuromyelitis-optica-spectrum-disorder-coinciding-with-spinocerebellar-ataxia-type-31
#19
Yoshiaki Takahashi, Yasuhiro Manabe, Ryuta Morihara, Hisashi Narai, Toru Yamashita, Koji Abe
We report the unusual case of a 63-year-old man with spinocerebellar ataxia (SCA) type 31 who developed neuromyelitis optica spectrum disorder (NMOSD) 14 years after the onset of cerebellar symptoms. In addition to cerebellar atrophy, magnetic resonance imaging showed multiple high-intensity areas in the brain and a long thoracic cord lesion from Th1/2 to Th11. The combination of NMOSD and SCA31 is accidental. However, our case suggests that inflammatory processes could be involved in the pathogenesis of NMOSD and SCA31...
May 2017: Case Reports in Neurology
https://www.readbyqxmd.com/read/28615429/diagnostic-algorithm-for-relapsing-acquired-demyelinating-syndromes-in-children
#20
Yael Hacohen, Kshitij Mankad, W K Chong, Frederik Barkhof, Angela Vincent, Ming Lim, Evangeline Wassmer, Olga Ciccarelli, Cheryl Hemingway
OBJECTIVE: To establish whether children with relapsing acquired demyelinating syndromes (RDS) and myelin oligodendrocyte glycoprotein antibodies (MOG-Ab) show distinctive clinical and radiologic features and to generate a diagnostic algorithm for the main RDS for clinical use. METHODS: A panel reviewed the clinical characteristics, MOG-Ab and aquaporin-4 (AQP4) Ab, intrathecal oligoclonal bands, and Epstein-Barr virus serology results of 110 children with RDS. A neuroradiologist blinded to the diagnosis scored the MRI scans...
July 18, 2017: Neurology
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