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Neurology® Neuroimmunology & Neuroinflammation

Johan Lim, Anke Rietveld, Jan L De Bleecker, Umesh A Badrising, Christiaan G J Saris, Anneke J van der Kooi, Marianne de Visser
Objectives: To investigate the characteristics of different clinico-serologic subgroups of immune-mediated necrotizing myopathy (IMNM). Methods: We retrospectively reviewed data from medical charts of 64 patients diagnosed with IMNM between 2012 and 2017 in 3 neuromuscular referral centers in The Netherlands and 1 in Belgium. Results: Seventeen patients had anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR) autoantibodies (Abs), of whom 11 had a history of statin use, 15 had anti-signal recognition particle (SRP) Abs, 2 had anti-melanoma differentiation-associated protein 5 (MDA5) Abs, 22 patients were seronegative, and 9 patients did not have a complete Ab assessment...
January 2019: Neurology® Neuroimmunology & Neuroinflammation
Michael Levy
No abstract text is available yet for this article.
November 2018: Neurology® Neuroimmunology & Neuroinflammation
Elsebeth Staun-Ram, Eiman Najjar, Anat Volkowich, Ariel Miller
Objective: To elucidate the immunomodulatory effects of dimethyl fumarate (DMF) on B cells in patients with relapsing MS receiving DMF as a "1st-line" vs "2nd-line" therapy. Methods: B cells were isolated from 43 patients with MS at baseline and after 15-week DMF therapy. Phenotype and functional markers and cytokine profile were assessed by flow cytometry. Analysis included clinical and MRI parameters recorded during a 1-year follow-up . Results: 1st-line and 2nd-line patients presented several differences in their baseline immune profile, which corresponded with differences in their immunologic response to DMF treatment...
November 2018: Neurology® Neuroimmunology & Neuroinflammation
Ikjae Lee, Henry J Kaminski, Tarrant McPherson, Michelle Feese, Gary Cutter
Objective: Prednisone is a first-line immunosuppressive treatment for myasthenia gravis (MG), whereas short-term and long-term adverse effects (AEs) are a limiting factor in its usage. Method: The MG patient registry is a patient-driven, nation-wide database with patients of age ≥18 years, who were diagnosed with MG and live in the United States. Custom-designed "prednisone-steroid use and MG" survey was sent out to MG registry participants as part of semi-annual follow-up...
November 2018: Neurology® Neuroimmunology & Neuroinflammation
Yujie Wang, James Andrews, Paula Jenkins Colon, Annette Wundes
No abstract text is available yet for this article.
November 2018: Neurology® Neuroimmunology & Neuroinflammation
Ingo Kleiter, Anna Gahlen, Nadja Borisow, Katrin Fischer, Klaus-Dieter Wernecke, Kerstin Hellwig, Florence Pache, Klemens Ruprecht, Joachim Havla, Tania Kümpfel, Orhan Aktas, Hans-Peter Hartung, Marius Ringelstein, Christian Geis, Christoph Kleinschnitz, Achim Berthele, Bernhard Hemmer, Klemens Angstwurm, Jan-Patrick Stellmann, Simon Schuster, Martin Stangel, Florian Lauda, Hayrettin Tumani, Christoph Mayer, Markus Krumbholz, Lena Zeltner, Ulf Ziemann, Ralf Linker, Matthias Schwab, Martin Marziniak, Florian Then Bergh, Ulrich Hofstadt-van Oy, Oliver Neuhaus, Uwe K Zettl, Jürgen Faiss, Brigitte Wildemann, Friedemann Paul, Sven Jarius, Corinna Trebst
Objective: To analyze whether 1 of the 2 apheresis techniques, therapeutic plasma exchange (PE) or immunoadsorption (IA), is superior in treating neuromyelitis optica spectrum disorder (NMOSD) attacks and to identify predictive factors for complete remission (CR). Methods: This retrospective cohort study was based on the registry of the German Neuromyelitis Optica Study Group, a nationwide network established in 2008. It recruited patients with neuromyelitis optica diagnosed according to the 2006 Wingerchuk criteria or with aquaporin-4 (AQP4-ab)-antibody-seropositive NMOSD treated at 6 regional hospitals and 16 tertiary referral centers until March 2013...
November 2018: Neurology® Neuroimmunology & Neuroinflammation
Barbara Spanò, Giovanni Giulietti, Valerio Pisani, Manuela Morreale, Elisa Tuzzi, Ugo Nocentini, Ada Francia, Carlo Caltagirone, Marco Bozzali, Mara Cercignani
Objectives: To apply advanced diffusion MRI methods to the study of normal-appearing brain tissue in MS and examine their correlation with measures of clinical disability. Methods: A multi-compartment model of diffusion MRI called neurite orientation dispersion and density imaging (NODDI) was used to study 20 patients with relapsing-remitting MS (RRMS), 15 with secondary progressive MS (SPMS), and 20 healthy controls. Maps of NODDI were analyzed voxel-wise to assess the presence of abnormalities within the normal-appearing brain tissue and the association with disease severity...
November 2018: Neurology® Neuroimmunology & Neuroinflammation
Amal P R Samaraweera, Yasser Falah, Alain Pitiot, Robert A Dineen, Paul S Morgan, Nikos Evangelou
Objective: To determine whether the assessment of brain white matter lesion (WML) central veins differentiate patients with primary progressive MS (PPMS) from relapsing-remitting MS (RRMS) and ischemic small vessel disease (SVD) using 3T MRI. Methods: In this cross-sectional study, 71 patients with PPMS, RRMS, and SVD were imaged using a T2*-weighted sequence. Two blinded raters identified the total number of WMLs, proportion of WMLs in periventricular, deep white matter (DWM) and juxtacortical regions, and proportion of WMLs with central veins in all patient groups...
November 2018: Neurology® Neuroimmunology & Neuroinflammation
Andrea Marcinnò, Fabiana Marnetto, Paola Valentino, Serena Martire, Alessia Balbo, Aurora Drago, Maria Leto, Marco Capobianco, Giancarlo Panzica, Antonio Bertolotto
Objective: To evaluate the long-term effects of rituximab (RTX) on total and specific immunoglobulins (Igs) in patients with neuromyelitis optica spectrum disorders (NMOSDs). Methods: Total IgG, IgA, and IgM levels were evaluated in 15 patients with NMOSDs treated with RTX (median follow-up 70 months). Anti-aquaporin 4 (AQP4)-IgG titration was performed on samples from 9 positive patients. Anti-tetanus (TET), anti-varicella-zoster virus (VZV), and anti-Epstein-Barr virus nuclear antigen (EBNA) IgGs were also tested in patients with NMOSDs and in 6 healthy controls (HCs)...
November 2018: Neurology® Neuroimmunology & Neuroinflammation
Rocio Vazquez Do Campo, Alana Stephens, I Vanessa Marin Collazo, Devon I Rubin
No abstract text is available yet for this article.
November 2018: Neurology® Neuroimmunology & Neuroinflammation
Keisuke Yoshikawa, Motoi Kuwahara, Miyuki Morikawa, Yuta Fukumoto, Masaki Yamana, Yuko Yamagishi, Susumu Kusunoki
Objective: To investigate the relationship between antibody reactivities against glycolipid complexes and clinical features in Miller Fisher syndrome (MFS), Bickerstaff brainstem encephalitis (BBE), and Guillain-Barré syndrome with ophthalmoplegia (GBS-OP). Methods: Using glycoarray, antibodies against 10 glycolipid antigens (GM1, GM2, GM4, GD1a, GD1b, GQ1b, galactocerebroside, lactosylceramide, GA1, and sulfatide) and 45 glycolipid complexes consisting 2 of the glycolipids were examined in the sera of 63 patients with GBS-OP, 37 patients with MFS, and 27 patients with BBE...
November 2018: Neurology® Neuroimmunology & Neuroinflammation
Madalina E Carter-Timofte, Anders F Hansen, Maibritt Mardahl, Sébastien Fribourg, Franck Rapaport, Shen-Ying Zhang, Jean-Laurent Casanova, Søren R Paludan, Mette Christiansen, Carsten S Larsen, Trine H Mogensen
Objective: Deficiency in the cytosolic DNA sensor RNA Polymerase III (POL III) was recently described in children with severe varicella-zoster virus (VZV) infection in the CNS or lungs. Here, we describe a pair of monozygotic female twins, who both experienced severe recurrent CNS vasculitis caused by VZV reactivation. The clinical presentation and findings included recurrent episodes of headache, dizziness, and neurologic deficits, CSF with pleocytosis and intrathecal VZV antibody production, and MRI of the brain showing ischemic lesions...
November 2018: Neurology® Neuroimmunology & Neuroinflammation
Giulia Longoni, Robert A Brown, Berengere Aubert-Broche, Stephanie A Grover, Helen M Branson, Dumitru Fetco, Amit Bar-Or, Ruth Ann Marrie, Robert W Motl, D Louis Collins, Sridar Narayanan, Douglas L Arnold, Brenda Banwell, E Ann Yeh
Objective: To assess the association between daily moderate-to-vigorous physical activity (MVPA) and dentate gyrus volume (DGv) in pediatric patients with acquired demyelinating syndromes (ADSs) of the CNS. Methods: Cross-sectional analysis of accelerometry (7 days) and research protocol MRI data from 12 pediatric MS and 18 children with monophasic ADS (monoADS). Total brain and DGv were quantified using standardized methods. The association of daily minutes of MVPA with normalized DGv (nDGv) was assessed using multivariable generalized linear models...
November 2018: Neurology® Neuroimmunology & Neuroinflammation
Natalie E Parks, Sean J Pittock, Jay Mandrekar, Orhun H Kantarci, Claudia F Lucchinetti, Brian G Weinshenker, B Mark Keegan, W Oliver Tobin, Jan-Mendelt Tillema, Michel Toledano, Eoin P Flanagan
Objective: To determine the persistence of no evident disease activity (NEDA) in a population-based relapsing-remitting MS (RRMS) cohort. Methods: All incident cases of RRMS in Olmsted County between 2000 and 2011 were identified using a medical records linkage system. Persistence of NEDA after RRMS diagnosis was determined by retrospective chart review. MRI activity, relapse, or Expanded Disability Status Scale (EDSS) worsening resulted in failure of NEDA. Results: We identified 93 incident cases of RRMS including 82 individuals with sufficient follow-up to determine the persistence of NEDA...
November 2018: Neurology® Neuroimmunology & Neuroinflammation
Devon A Cohen, A Sebastian Lopez-Chiriboga, Sean J Pittock, Avi Gadoth, Anastasia Zekeridou, Barry A Boilson, William J Hogan, John J Poterucha, Katelynn M Wilton, Yi Lin, Eoin P Flanagan
No abstract text is available yet for this article.
November 2018: Neurology® Neuroimmunology & Neuroinflammation
Josep Dalmau
No abstract text is available yet for this article.
September 2018: Neurology® Neuroimmunology & Neuroinflammation
Franziska Wagner, Helena Radbruch, Otto W Witte, Christian Geis
No abstract text is available yet for this article.
September 2018: Neurology® Neuroimmunology & Neuroinflammation
Noah Ayadi, Jan Dörr, Seyedamirhosein Motamedi, Kay Gawlik, Judith Bellmann-Strobl, Janine Mikolajczak, Alexander U Brandt, Hanna Zimmermann, Friedemann Paul
Objective: To examine temporal visual resolution assessed as critical flicker frequency (CFF) in patients with MS and to investigate associations with visual system damage and general disability and cognitive function. Methods: Thirty-nine patients with MS and 31 healthy controls (HCs) were enrolled in this cross-sectional study and underwent CFF testing, high- and low-contrast visual acuity, alertness and information processing speed using the paced auditory serial addition task (PASAT), and retinal optical coherence tomography (OCT)...
September 2018: Neurology® Neuroimmunology & Neuroinflammation
Keren Regev, Brian C Healy, Anu Paul, Camilo Diaz-Cruz, Maria Antonietta Mazzola, Radhika Raheja, Bonnie I Glanz, Pia Kivisäkk, Tanuja Chitnis, Maja Jagodic, Fredrik Piehl, Tomas Olsson, Mohsen Khademi, Stephen Hauser, Jorge Oksenberg, Samia J Khoury, Howard L Weiner, Roopali Gandhi
Objective: To identify circulating microRNAs (miRNAs) linked to disease, disease stage, and disability in MS across cohorts. Methods: Samples were obtained from the Comprehensive Longitudinal Investigation of Multiple Sclerosis (CLIMB, Boston, MA), EPIC (San Francisco, CA), AMIR (Beirut, Lebanon) as part of the SUMMIT consortium, and Stockholm Prospective Assessment of Multiple Sclerosis (Stockholm, Sweden) cohorts. Serum miRNA expression was measured using locked nucleic acid-based quantitative PCR...
September 2018: Neurology® Neuroimmunology & Neuroinflammation
Gülden Akdal, Hülya Ertaşoğlu Toydemir, Ali Osman Saatci, Uğur Uygunoğlu, Burcu Altunrende, Sabahattin Saip, Aylin Yaman, Pembe Keskinoğlu, Suzan Güven Yilmaz, Neşe Çelebisoy, Meltem Söylev Bajin, Aksel Siva, Gülşen Akman-Demir
Objective: We present the clinical profile, features, and neuroimaging findings of 25 patients with Behçet disease (BD), and optic neuropathy (ON), which has been rarely reported in BD. Methods: Data from 5 university hospitals were retrospectively reviewed, and patients with BD and ON were evaluated. There were 2 groups: (1) those already diagnosed with BD when ON developed (BD → ON group) and (2) those diagnosed with BD during the evaluation of ON (ON → BD group)...
September 2018: Neurology® Neuroimmunology & Neuroinflammation
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