Review
Medicine, General & Internal
Dean M. Wingerchuk, Claudia F. Lucchinetti
Summary: Neuromyelitis Optica and its spectrum is a relapsing demyelinating disorder of the central nervous system, encompassing six syndromes and associated with aquaporin-4 autoantibodies. Effective treatment is able to prevent relapses.
NEW ENGLAND JOURNAL OF MEDICINE
(2022)
Review
Medicine, General & Internal
Dean M. Wingerchuk, Claudia F. Lucchinetti
Summary: Neuromyelitis Optica, previously known as Devic's disease, is a relapsing demyelinating disorder of the central nervous system that encompasses six syndromes and is associated with aquaporin-4 autoantibodies. Effective treatment is crucial in preventing relapses.
NEW ENGLAND JOURNAL OF MEDICINE
(2022)
Review
Biochemistry & Molecular Biology
Koon-Ho Chan, Chi-Yan Lee
Summary: NMOSD is an autoimmune disorder that predominantly affects females, leading to serious CNS inflammatory disorders. The pathogenesis involves AQP4-IgG autoantibodies targeting aquaporin-4, triggering astrocytopathy and neuroinflammation.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2021)
Review
Clinical Neurology
Shabeer Paul, Gouranga Prasad Mondal, Ramesh Bhattacharyya, Kartik Chandra Ghosh, Imtiyaz Ahmad Bhat
Summary: Over the past two decades, the disease concept of NMOSD has significantly changed with the detection of MOG antibody and the understanding of immune astrocytopathy. The revised diagnostic criteria have widened the clinical spectrum of NMOSD, allowing for earlier diagnosis and prompt initiation of effective immunosuppression for better long-term outcomes. Challenges still remain in treating seronegative NMOSD due to limited treatment options.
JOURNAL OF THE NEUROLOGICAL SCIENCES
(2021)
Article
Clinical Neurology
Yong Guo, Vanda A. Lennon, Joseph E. Parisi, Bogdan Popescu, Christina Vasquez, Sean J. Pittock, Charles L. Howe, Claudia F. Lucchinetti
Summary: Neuromyelitis optica is an autoimmune inflammatory disorder that affects CNS astrocytes. This study reveals the complex astrogliotic reactions and their role in the evolution and potential for repair of lesions, independent of aquaporin-4 loss or lysis.
Review
Immunology
Tingjun Chen, Dale B. Bosco, Yanlu Ying, Dai-Shi Tian, Long-Jun Wu
Summary: NMO is an autoantibody-triggered neuro-inflammatory disease that mainly affects the spinal cord and optic nerve. Studies have shown significant microglial activation in NMO lesions, indicating a potential role for microglia in NMO pathology.
FRONTIERS IN IMMUNOLOGY
(2021)
Review
Clinical Neurology
Michael Levy, Kazuo Fujihara, Jacqueline Palace
Summary: Neuromyelitis optica spectrum disorder is a rare autoimmune disease affecting the CNS, with recent trials showing benefits of new therapies in preventing future attacks. However, differences in efficacy, safety, tolerability, and practical considerations of these therapies may impact their use in real-world populations of patients. Future research should focus on unmet needs, including aquaporin-4 seronegative disease and treatments for acute relapses and recovery from autoimmune attacks in the CNS.
Review
Medicine, General & Internal
Yachinee Naphattalung, Wanicha Leetiratanai Chuenkongkaew, Niphon Chirapapaisan, Poramaet Laowanapiban, Supattra Sawangkul
Summary: The current data from this systematic review are insufficient to conclude definitively whether therapeutic plasma exchange (PLEX) is effective in improving visual acuity in cases of neuromyelitis optica (NMO) or neuromyelitis optica spectrum disorder (NMOSD). The objective of this study was to evaluate whether PLEX effectively improves visual function for acute optic neuritis (ON) in NMO or NMOSD. Twelve studies were included in the analysis, but the data were inconclusive in determining the effectiveness of PLEX for treating acute ON in NMO/NMOSD.
ANNALS OF MEDICINE
(2023)
Review
Dermatology
Laurent Misery, Steeve Genestet, Fabien Zagnoli
Summary: Neuromyelitis optica spectrum disorder is a disease characterized by attacks of optic neuritis and/or longitudinally extensive transverse myelitis and the presence of anti-aquaporin-4 autoantibodies. The disease worsens over a few days and slowly improves in the weeks or months after reaching the maximum clinical deficit, but recovery is usually incomplete. Early diagnosis and emergency treatment are crucial for managing the disease. Dermatologists can help in achieving this and neuropathic pruritus can be useful for diagnosing the disease. New skin infections or sexually transmitted diseases can trigger relapses of neuromyelitis optica.
Article
Clinical Neurology
Raffaella Pizzolato Umeton, Michael Waltz, Gregory S. Aaen, Leslie Benson, Mark Gorman, Manu Goyal, Jennifer S. Graves, Yolanda Harris, Lauren Krupp, Timothy E. Lotze, Nikita M. Shukla, Soe Mar, Jayne Ness, Mary Rensel, Teri Schreiner, Jan-Mendelt Tillema, Shelly Roalstad, Moses Rodriguez, John Rose, Emmanuelle Waubant, Bianca Weinstock-Guttman, Charles Casper, Tanuja Chitnis
Summary: This retrospective cohort study evaluated cases of children with NMOSD and found that the use of disease-modifying treatments, particularly rituximab, is associated with a lowered annualized relapse rate in children with NMOSD AQP4+.
Article
Clinical Neurology
Oliver Schmetzer, Elisa Lakin, Ben Roediger, Ankelien Duchow, Susanna Asseyer, Friedemann Paul, Nadja Siebert
Summary: This study examined the role of anti-AQP4-IgG in patients with Neuromyelitis optica spectrum disorder (NMOSD). The results suggest that anti-AQP4-IgG may not play a predominant role in driving NMOSD, but rather may be associated with the disease and could be a relevant factor in only a subgroup of patients.
FRONTIERS IN NEUROLOGY
(2021)
Review
Immunology
Edgar Carnero Contentti, Jorge Correale
Summary: Neuromyelitis optica (NMO) is a chronic inflammatory autoimmune disease characterized by acute optic neuritis and transverse myelitis. It is caused by a pathogenic serum IgG antibody targeting the water channel aquaporin 4 (AQP4). Recent studies have shown significant progress in identifying new treatments for NMOSD.
JOURNAL OF NEUROINFLAMMATION
(2021)
Article
Clinical Neurology
Frederike Cosima Oertel, Svenja Specovius, Hanna G. Zimmermann, Claudia Chien, Seyedamirhosein Motamedi, Charlotte Bereuter, Lawrence Cook, Marco Aurelio Lana Peixoto, Mariana Andrade Fontanelle, Ho Jin Kim, Jae-Won Hyun, Jacqueline Palace, Adriana Roca-Fernandez, Maria Isabel Leite, Srilakshmi Sharma, Fereshteh Ashtari, Rahele Kafieh, Alireza Dehghani, Mohsen Pourazizi, Lekha Pandit, Anitha D'Cunha, Orhan Aktas, Marius Ringelstein, Philipp Albrecht, Eugene May, Caryl Tongco, Letizia Leocani, Marco Pisa, Marta Radaelli, Elena H. Martinez-Lapiscina, Hadas Stiebel-Kalish, Sasitorn Siritho, Jerome de Seze, Thomas Senger, Joachim Havla, Romain Marignier, Alvaro Cobo Calvo, Denis Bichuetti, Ivan Maynart Tavares, Nasrin Asgari, Kerstin Soelberg, Ayse Altintas, Rengin Yildirim, Uygur Tanriverdi, Anu Jacob, Saif Huda, Zoe Rimler, Allyson Reid, Yang Mao-Draayer, Ibis Soto de Castillo, Axel Petzold, Ari J. Green, Michael R. Yeaman, Terry Smith, Alexander U. Brandt, Friedemann Paul
Summary: This study highlights the importance of attack prevention to avoid severe neuroaxonal damage and vision loss caused by ON in NMOSD. Therapies to ameliorate attack-related damage, especially during the first attack, are a clinical need. Mild neuroaxonal changes in ON-unaffected eyes may be solely due to contralateral ON attacks and do not indicate clinically relevant progression, but require further investigation.
NEUROLOGY-NEUROIMMUNOLOGY & NEUROINFLAMMATION
(2021)
Article
Neurosciences
Daissy Liliana Mora Cuervo, Gisele Hansel, Douglas Kazutoshi Sato
Summary: Neuromyelitis optica spectrum disorder (NMOSD) is a rare autoimmune inflammatory disease that is characterized by the presence of autoantibodies targeting the water channel aquaporin-4 (AQP4-IgG). The core clinical manifestations of NMOSD include optic neuritis, longitudinally extensive myelitis, and area postrema syndrome. Diagnosis is based on clinical manifestations, magnetic resonance imaging findings, and the presence of AQP4-IgG. Recent advances in the understanding of NMOSD immunobiology have led to approved treatments such as eculizumab, satralizumab, and inebilizumab.
CURRENT OPINION IN NEUROBIOLOGY
(2022)
Article
Clinical Neurology
Sean J. Pittock, Michael Barnett, Jeffrey L. Bennett, Achim Berthele, Jerome de Seze, Michael Levy, Ichiro Nakashima, Celia Oreja-Guevara, Jacqueline Palace, Friedemann Paul, Carlo Pozzilli, Marcus Yountz, Kerstin Allen, Yasmin Mashhoon, Ho Jin Kim
Summary: The CHAMPION-NMOSD study evaluated the efficacy and safety of ravulizumab in adult patients with anti-aquaporin-4 antibody-positive neuromyelitis optica spectrum disorder. Ravulizumab, which has a longer half-life compared to eculizumab, significantly reduced relapse risk in these patients.
ANNALS OF NEUROLOGY
(2023)
Article
Clinical Neurology
Maria Pia Sormani, Irene Schiavetti, Doriana Landi, Luca Carmisciano, Nicola De Rossi, Cinzia Cordioli, Lucia Moiola, Marta Radaelli, Paolo Immovilli, Marco Capobianco, Vincenzo Brescia Morra, Maria Trojano, Gioacchino Tedeschi, Giancarlo Comi, Mario Alberto Battaglia, Francesco Patti, Yara Dadalti Fragoso, Sedat Sen, Aksel Siva, Roberto Furlan, Marco Salvetti
Summary: The study assessed the relationship between disease-modifying therapy (DMT) and the development of anti-SARS-CoV-2 antibodies in patients with multiple sclerosis (MS), finding that patients treated with anti-CD20 drugs had a lower likelihood of developing antibodies.
MULTIPLE SCLEROSIS JOURNAL
(2022)
Article
Endocrinology & Metabolism
Andrea Laurenzi, Amelia Caretto, Chiara Molinari, Alessia Mercalli, Raffaella Melzi, Rita Nano, Cristina Tresoldi, Patrizia Rovere Querini, Fabio Ciceri, Vito Lampasona, Emanuele Bosi, Marina Scavini, Lorenzo Piemonti
Summary: This study aimed to assess whether dysglycemia diagnosed during COVID-19 pneumonia could become a potential public health problem after the infection is resolved. The research found that COVID-19-associated dysglycemia is unlikely to be a lasting public health problem, thereby cautioning against alarmist claims on the risk of diabetes after COVID-19 pneumonia.
JOURNAL OF CLINICAL ENDOCRINOLOGY & METABOLISM
(2022)
Article
Clinical Neurology
Francesco Benedetti, Mariagrazia Palladini, Greta 'Orsi, Roberto Furlan, Fabio Ciceri, Patrizia Rovere-Querini, Mario Gennaro Mazza, The COVID- BioB Outpatient Clinic group Study
Summary: This study found that COVID-19 survivors have negative thinking styles associated with depressive psychopathology. These survivors experienced negative emotions and affects, hindering their recovery. This cognitive vulnerability has been observed in medical conditions associated with depression, but never explored in post-COVID depression. The findings suggest that the severity of depression is related to dimensions of negative thinking styles, which is important for the treatment of depressed COVID-19 survivors.
JOURNAL OF AFFECTIVE DISORDERS
(2022)
Article
Clinical Neurology
Gisella Guerrera, Alessandra Mandelli, Annamaria Finardi, Mario Orrico, Silvia D'Orso, Mario Picozza, Maddalena Noviello, Valeria Beretta, Bruno Bonetti, Massimiliano Calabrese, Damiano Marastoni, Nicola De Rossi, Ruggero Capra, Marco Salvetti, Maria Chiara Buscarinu, Matilde Inglese, Antonio Uccelli, Lucia Moiola, Catarina Raposo, Erwan Muros-Le Rouzic, Rosetta Pedotti, Massimo Filippi, Chiara Bonini, Luca Battistini, Giovanna Borsellino, Roberto Furlan
Summary: Development of long-lasting anti-SARS-CoV-2 T-cell responses is not impaired in ocrelizumab-treated pwMS.
MULTIPLE SCLEROSIS JOURNAL
(2022)
Article
Radiology, Nuclear Medicine & Medical Imaging
Marta Lancione, Paolo Bosco, Mauro Costagli, Anna Nigri, Domenico Aquino, Irene Carne, Stefania Ferraro, Giovanni Giulietti, Antonio Napolitano, Fulvia Palesi, Luigi Pavone, Alice Pirastru, Giovanni Savini, Fabrizio Tagliavini, Maria Grazia Bruzzone, Claudia A. M. Gandini Wheeler-Kingshott, Michela Tosetti, Laura Biagi
Summary: Quantitative Susceptibility Mapping (QSM) is a technique in MRI that quantifies iron content and myelination in the brain. The RIN - Neuroimaging Network established an optimized protocol for QSM across multiple sites with 3T MRI systems, showing good reproducibility. This protocol can be used as a quantitative biomarker in multicentric studies.
PHYSICA MEDICA-EUROPEAN JOURNAL OF MEDICAL PHYSICS
(2022)
Article
Biochemistry & Molecular Biology
Luana Gilio, Fabio Buttari, Luigi Pavone, Ennio Iezzi, Giovanni Galifi, Ettore Dolcetti, Federica Azzolini, Antonio Bruno, Angela Borrelli, Marianna Storto, Roberto Furlan, Annamaria Finardi, Tatjana Pekmezovic, Jelena Drulovic, Georgia Mandolesi, Diego Fresegna, Valentina Vanni, Diego Centonze, Mario Stampanoni Bassi
Summary: Fatigue in multiple sclerosis (MS) is associated with central inflammation and disease progression. Inflammation may exacerbate fatigue. By exploring clinical characteristics and biomarkers, a negative correlation was found between fatigue levels and the cerebrospinal fluid levels of the anti-inflammatory molecule IL-10 at diagnosis.
Article
Health Care Sciences & Services
Bruno Bernardini, Luigi Baratto, Costanza Pizzi, Annibale Biggeri, Giovanna Cerina, Viviana Colantonio, Carla Corsini, Sara Ghirmai, Marco Pagani, Stefania Fracchia, Marisa Gardella, Dolores Catelan, Maria Luisa Malosio, Elisa Malagamba
Summary: Objective: To develop and validate the Functional Risk Index for Dependence in Ambulation (FRIDA) score, a nomogram for predicting individual risk of dependence in ambulation at rehabilitation discharge. Results: The FRIDA score demonstrated good discrimination and calibration in predicting dependence in ambulation, both temporally and spatially. It is a clinically useful tool for predicting individual risk of dependence in ambulation and reflecting the case-mix composition of rehabilitation facilities.
JOURNAL OF CLINICAL EPIDEMIOLOGY
(2023)
Article
Biochemistry & Molecular Biology
Angela Genchi, Elena Brambilla, Francesca Sangalli, Marta Radaelli, Marco Bacigaluppi, Roberto Furlan, Annapaola Andolfo, Denise Drago, Cinzia Magagnotti, Giulia Maria Scotti, Raffaella Greco, Paolo Vezzulli, Linda Ottoboni, Marco Bonopane, Daniela Capilupo, Francesca Ruffini, Daniela Belotti, Benedetta Cabiati, Stefania Cesana, Giada Matera, Letizia Leocani, Vittorio Martinelli, Lucia Moiola, Luca Vago, Paola Panina-Bordignon, Andrea Falini, Fabio Ciceri, Anna Uglietti, Maria Pia Sormani, Giancarlo Comi, Mario Alberto Battaglia, Maria A. A. Rocca, Loredana Storelli, Elisabetta Pagani, Giuseppe Gaipa, Gianvito Martino
Summary: This study conducted a phase 1 clinical trial evaluating the feasibility, safety and tolerability of intrathecally transplanted human fetal neural precursor cells (hfNPCs) in patients with progressive multiple sclerosis (PMS). The results demonstrated that hfNPC therapy is feasible, safe and tolerable in PMS patients.
Article
Biology
Valentina Murtaj, Silvia Penati, Sara Belloli, Maria Foti, Angela Coliva, Angela Papagna, Cecilia Gotti, Elisa Toninelli, Remy Chiaffarelli, Stefano Mantero, Susanna Pucci, Michela Matteoli, Maria Luisa Malosio, Rosa Maria Moresco
Summary: The study found that male mice exposed to a high fat diet are more susceptible to metabolic changes compared to female mice, showing faster and higher weight gain, elevated cholesterol and liver enzyme levels, and more prominent brain inflammation.
COMMUNICATIONS BIOLOGY
(2022)
Article
Endocrinology & Metabolism
Ilaria Marzinotto, David L. L. Pittman, Alistair J. K. Williams, Anna E. E. Long, Peter Achenbach, Michael Schlosser, Beena Akolkar, William E. E. Winter, Vito Lampasona
Summary: The Islet Autoantibody Standardization Program (IASP) aims to improve the performance and consistency of immunoassays measuring autoantibodies in type 1 diabetes. Results from IASP workshops in 2018 and 2020 showed variability in assay performance, with in-house radiobinding assay (RBA) still considered the gold standard. However, non-radioactive IAA immunoassays showed promising results and could be potential alternatives to RBAs.
Article
Biochemistry & Molecular Biology
Claudia Vanetti, Vito Lampasona, Marta Stracuzzi, Claudio Fenizia, Mara Biasin, Irma Saulle, Fiona Limanaqi, Ahmed Abdelsalam, Cristian Loretelli, Laura Paradiso, Emma Longoni, Lucia Barcellini, Lorenzo Piemonti, Ilaria Marzinotto, Stefania Dispinseri, Antonella Amendola, Clara Fappani, Elisabetta Tanzi, Mario Salvatore Clerici, Gabriella Scarlatti, Gian Vincenzo Zuccotti, Vania Giacomet, Daria Trabattoni
Summary: This study analyzed the immune profiles of 18 hospitalized children with SARS-CoV-2 infection and found that different severity levels of children cases showed different immune characteristics. Infants with severe symptoms exhibited high inflammatory response and extreme antibody response, while mild cases had lower levels of inflammation and antibodies. Overall, the immune response in children is directly correlated with the clinical severity.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2023)
Article
Clinical Neurology
David Leppert, Mitsuru Watanabe, Sabine Schaedelin, Fredrik Piehl, Roberto Furlan, Matteo Gastaldi, Jeremy Lambert, Bjorn Evertsson, Katharina Fink, Takuya Matsushita, Katsuhisa Masaki, Noriko Isobe, Jun-ichi Kira, Pascal Benkert, Aleksandra Maceski, Eline Willemse, Johanna Oechtering, Annette Orleth, Stephanie Meier, Jens Kuhle
Summary: This study aimed to determine the differences in granulocyte activation markers (GAM) between neuromyelitis optica spectrum disorder (NMOSD) and multiple sclerosis (MS), and whether they are associated with neurological impairment. The results showed that GAM and adhesion molecules were higher in acute NMOSD compared to RRMS and correlated with clinical disability scores. The composite of GAM can be used as a reliable biomarker to differentiate NMOSD from MS, including aAQP4(-) NMOSD.
JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY
(2023)
Article
Clinical Neurology
Paride Schito, Tommaso Russo, Teuta Domi, Alessandra Mandelli, Laura Pozzi, Ubaldo Del Carro, Paola Carrera, Federica Agosta, Angelo Quattrini, Roberto Furlan, Massimo Filippi, Nilo Riva
Summary: The study aims to determine the features at onset that can differentiate between primary lateral sclerosis (PLS) and amyotrophic lateral sclerosis (ALS), evaluate the diagnostic performance of a serum biomarker panel, and identify prognostic factors for patients with upper motor neuron syndrome.
Article
Clinical Neurology
Nicolas Fissolo, Pascal Benkert, Jaume Sastre-Garriga, Neus Mongay-Ochoa, Andreu Vilaseca-Jolonch, Sara Llufriu, Yolanda Blanco, Harald Hegen, Klaus Berek, Francisco Perez-Miralles, Konrad Rejdak, Luisa M. Villar, Enric Monreal, Roberto Alvarez-Lafuente, Onder K. Soylu, Ahmed Abdelhak, Franziska Bachhuber, Hayrettin Tumani, Sergio Martinez-Yelamos, Antonio J. Sanchez-Lopez, Antonio Garcia-Merino, Lucia Gutierrez, Tamara Castillo-Trivino, Jan Lycke, Igal Rosenstein, Roberto Furlan, Massimo Filippi, Nieves Tellez, Lluis Ramio-Torrenta, Jan D. Lunemann, Heinz Wiendl, Sara Eichau, Michael Khalil, Jens Kuhle, Xavier Montalban, Manuel Comabella
Summary: This study aimed to investigate the potential of serum biomarker levels in predicting disability progression in patients with primary progressive multiple sclerosis (PPMS). The study found that levels of neurofilament light chain (sNfL), glial fibrillar acidic protein (sGFAP), and chitinase 3-like 1 (sCHI3L1) were associated with changes in the Expanded Disability Status Scale (EDSS) over different time periods.
JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY
(2023)
Article
Cell Biology
Sinduya Krishnarajah, Florian Ingelfinger, Ekaterina Friebel, Dilay Cansever, Ana Amorim, Myrto Andreadou, David Bamert, Gioana Litscher, Mirjam Lutz, Maud Mayoux, Sarah Mundt, Frederike Ridder, Colin Sparano, Sebastian Anton Stifter, Can Ulutekin, Susanne Unger, Marijne Vermeer, Pascale Zwicky, Melanie Greter, Sonia Tugues, Donatella De Feo, Burkhard Becher
Summary: This study provides a comprehensive analysis of age-related changes in immune cell populations across 12 tissues in mice. The findings reveal distinct phenotypic changes in the lymphoid and myeloid lineages in aged mice, including a decrease in natural killer cells and plasmacytoid dendritic cells. This atlas serves as a valuable resource for understanding the age-dependent alterations in the mammalian immune system.