Article
Immunology
Katharina Hecker, Julia Gruener, Beate Hartmannsberger, Luise Appeltshauser, Carmen Villmann, Claudia Sommer, Kathrin Doppler
Summary: The access of IgG autoantibodies against neurofascin-155 and contactin-1 to the paranodes and their pathogenic effect were explored through in vitro and in vivo experiments. Different pathogenic mechanisms and accessibility of paranodal and nodal structures were observed.
FRONTIERS IN IMMUNOLOGY
(2023)
Article
Medicine, Research & Experimental
Rhona McGonigal, Clare Campbell, Jennifer A. Barrie, Denggao Yao, Madeleine E. Cunningham, Colin L. Crawford, Simon Rinaldi, Edward G. Rowan, Hugh J. Willison
Summary: In Guillain-Barre syndrome (GBS), both axonal and demyelinating variants can be mediated by complement-fixing anti-GM1 ganglioside autoantibodies that target peripheral nerve axonal and Schwann cell (SC) membranes, respectively. This study investigated the mechanisms underlying direct axonal injury and secondary axonal degeneration following glial injury in GBS using genetically modified mice targeting different neuronal and glial membranes. The findings provide insights into primary and secondary axonal injury and contribute to the understanding of GBS pathogenesis.
JOURNAL OF CLINICAL INVESTIGATION
(2022)
Article
Behavioral Sciences
Juichi Fujimori, Kazuo Fujihara, Mike Wattjes, Ichiro Nakashima
Summary: The study found that cortical gray matter thickness reduction patterns in multiple sclerosis (MS) patients are mainly characterized by the degree of temporal lobe cortical atrophy, which may start in the relapsing-remitting phase. As the disease progresses, neurodegenerative changes in the temporal pole region may accelerate in the progressive phase.
BRAIN AND BEHAVIOR
(2021)
Review
Neurosciences
Ying Gao, Lingxin Kong, Shan Liu, Kangding Liu, Jie Zhu
Summary: This study examined the role of neurofascin in the peripheral nervous system, particularly in chronic inflammatory demyelinating polyneuropathy, and its mechanisms and effects. By dissecting the role of cell adhesion molecules and the structure of the node of Ranvier, a thorough analysis of the pathogenesis of CIDP was conducted.
FRONTIERS IN MOLECULAR NEUROSCIENCE
(2021)
Article
Clinical Neurology
Meng Dong, Hongfei Tai, Shuo Yang, Xiaozhen Gao, Hua Pan, Zaiqiang Zhang
Summary: This study detected the positive rates of antibodies against nodal-paranodal junction proteins in patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and found that patients with anti-NF155 antibodies had more severe nerve damage.
CLINICAL NEUROLOGY AND NEUROSURGERY
(2022)
Article
Neurosciences
Navnika Gupta, Afsaneh Shirani, Lakshman Arcot Jayagopal, Ezequiel Piccione, Elizabeth Hartman, Rana Khalil Zabad
Summary: Autoantibodies against nodal and paranodal proteins have been found in central and peripheral nervous system demyelinating disorders. This study retrospectively reviewed the charts of six patients who tested positive for these antibodies and described their clinical presentations and diagnostic findings. The study suggests different features that may help clinicians in checking for these antibodies.
Review
Pathology
Katharina Eikermann-Haerter, Susie Y. Huang
Summary: Migraine is a well-known risk factor for subclinical focal deep white matter lesions, with ischemic and inflammatory mechanisms proposed as underlying causes, negatively impacting both physical and cognitive functions.
AMERICAN JOURNAL OF PATHOLOGY
(2021)
Article
Neurosciences
Sanem A. Aykan, Hongyu Xie, James Han Lai, Yi Zheng, David Y. Chung, Sreekanth Kura, Maryam Anzabi, Kazutaka Sugimoto, Lauren M. McAllister, M. Abbas Yaseen, David A. Boas, Michael J. Whalen, Sava Sakadzic, Cenk Ayata
Summary: This study investigated the effects of focal lesions in the lateral corpus callosum induced by L-NIO injection in mice, observing that it resulted in decreased interhemispheric and intrahemispheric connectivity. The findings suggest that RSFC imaging can be a noninvasive method to detect acute disruption of connectivity after white matter injury and may complement neurocognitive testing in therapeutic and recovery studies.
Article
Clinical Neurology
Cheng-Yin Tan, Khean-Jin Goh, Ai-Wen Oh, Jerome Devaux, Nortina Shahrizaila
Summary: This study reported a cohort of patients with CIDP who had a prevalence of 8% of autoimmune nodopathies and poor response to IVIG treatment. Patients with IgG4 anti-NF155 and anti-CNTN1 antibodies showed similar clinical features and treatment responses as previous reports.
NEUROMUSCULAR DISORDERS
(2022)
Article
Neurosciences
Luise Appeltshauser, Janis Linke, Hannah S. Heil, Christine Karus, Joachim Schenk, Katherina Hemmen, Claudia Sommer, Kathrin Doppler, Katrin G. Heinze
Summary: This study used super-resolution fluorescence microscopy and deep learning analysis to study human nerve biopsies. The results revealed periodic protein arrangement in the node of Ranvier in healthy and diseased human peripheral nerves, with a weakened periodicity in disease conditions associated with cytoskeletal damage. The study demonstrates the potential of super-resolution imaging for clinical applications.
NEUROBIOLOGY OF DISEASE
(2023)
Article
Psychology, Multidisciplinary
Lidon Marin-Marin, Victor Costumero, Cesar avila, Christos Pliatsikas
Summary: This study investigates the non-linear effects of bilingual experiences on regional grey matter volume in the brain. The results show a non-linear relationship between bilingualism score and inferior frontal gyrus volume, as well as linear increases in putamen and cerebellum volumes as a function of bilingualism score.
FRONTIERS IN PSYCHOLOGY
(2022)
Letter
Clinical Neurology
S. Quigley, M. C. Yiannakas, P. Bede, J. Meaney, H. Kearney
Summary: Multiple sclerosis (MS) is often misdiagnosed using MRI abnormalities in the brain white matter. Detecting cortical lesions, which have a preference for regions with CSF stasis, could potentially reduce misdiagnosis. A pilot MR imaging study successfully uses high spatial resolution imaging of the insula and cingulate gyrus to identify cortical lesions in MS.
MULTIPLE SCLEROSIS AND RELATED DISORDERS
(2023)
Article
Clinical Neurology
Daniel Ontaneda, Praneeta C. Raza, Kedar R. Mahajan, Douglas L. Arnold, Michael G. Dwyer, Susan A. Gauthier, Douglas N. Greve, Daniel M. Harrison, Roland G. Henry, David K. B. Li, Caterina Mainero, Wayne Moore, Sridar Narayanan, Jiwon Oh, Raihaan Patel, Daniel Pelletier, Alexander Rauscher, William D. Rooney, Nancy L. Sicotte, Roger Tam, Daniel S. Reich, Christina J. Azevedo
Summary: Multiple sclerosis is not only a white matter disease, but also involves grey matter injury, especially in deep grey matter regions. The deep grey matter is uniquely suited for studying the mechanisms and clinical relevance of tissue injury in multiple sclerosis using magnetic resonance techniques. MRI characterization of deep grey matter properties has potential as clinical trial end points for neurodegenerative aspects of multiple sclerosis.
Article
Neurosciences
Wenhao Zhu, Hao Huang, Shiqi Yang, Xiang Luo, Wenzhen Zhu, Shabei Xu, Qi Meng, Chengchao Zuo, Yong Liu, Wei Wang
Summary: The study found that gray matter atrophy in white matter hyperintensities patients may lead to cognitive decline, especially in those with mild cognitive impairment. The reduction in gray matter volume in the thalamus and fronto-insular cortices is associated with cognitive decline, and these results were further validated.
NEUROSCIENCE BULLETIN
(2021)
Article
Clinical Neurology
Carmen Picon, Anusha Jayaraman, Rachel James, Catriona Beck, Patricia Gallego, Maarten E. Witte, Jack van Horssen, Nicholas D. Mazarakis, Richard Reynolds
Summary: The study suggests that in the cortex of multiple sclerosis, necroptosis induced by TNF may be more common than apoptosis, possibly initiated by chronic meningeal inflammation. Neuronal necroptosis represents a pathogenetic mechanism that is amenable to therapeutic intervention at several points in the signaling pathway.
ACTA NEUROPATHOLOGICA
(2021)
Review
Allergy
Francesco Borriello, Noemi Pasquarelli, Lisa Law, Kim Rand, Catarina Raposo, Wei Wei, Licinio Craveiro, Tobias Derfuss
Summary: This study provides the first normal reference ranges for B-cell levels in infants, by week of age, during the first year of life.
JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY
(2022)
Article
Radiology, Nuclear Medicine & Medical Imaging
Alexandra Ramona Todea, Lester Melie-Garcia, Muhamed Barakovic, Alessandro Cagol, Reza Rahmanzadeh, Riccardo Galbusera, Po-Jui Lu, Matthias Weigel, Esther Ruberte, Ernst-Wilhelm Radue, Sabine Schaedelin, Pascal Benkert, Yaldizli Oezguer, Tim Sinnecker, Stefanie Mueller, Lutz Achtnichts, Jochen Vehoff, Giulio Disanto, Oliver Findling, Andrew Chan, Anke Salmen, Caroline Pot, Patrice Lalive, Claire Bridel, Chiara Zecca, Tobias Derfuss, Luca Remonda, Franca Wagner, Maria Vargas, Renaud Du Pasquier, Emanuele Pravata, Johannes Weber, Claudio Gobbi, David Leppert, Jens Wuerfel, Tobias Kober, Benedicte Marechal, Ricardo Corredor-Jerez, Marios Psychogios, Johanna Lieb, Ludwig Kappos, Meritxell Bach Cuadra, Jens Kuhle, Cristina Granziera
Summary: This study evaluated the accuracy of LeMan-PV software for detecting new and enlarged white matter lesions in multiple sclerosis patients. The results showed that LeMan-PV had similar sensitivity in detecting new lesions compared to other recent studies using neural networks. Although its performance is not optimal, the main advantage of LeMan-PV is that it provides automated clinical decision support integrated into the routine radiological workflow.
JOURNAL OF MAGNETIC RESONANCE IMAGING
(2023)
Editorial Material
Clinical Neurology
Tanja Kuhlmann, Marcello Moccia, Timothy Coetzee, Jeffrey A. Cohen, Jorge Correale, Jennifer Graves, Ruth Ann Marrie, Xavier Montalban, V. Wee Yong, Alan J. Thompson, Daniel S. Reich
Summary: Traditionally, multiple sclerosis has been categorized into distinct clinical descriptors, but accumulating evidence suggests that it should be considered as a continuum with varying pathophysiological processes. A shift from acute injury to inflammation and neurodegeneration contributes to the progressive course, along with decreased neural resilience due to aging. Understanding the key mechanisms and quantifying progressive pathology can have implications for clinical care, treatment targets, and regulatory decisions.
Editorial Material
Clinical Neurology
Cristina Granziera, Tobias Derfuss, Ludwig Kappos
Article
Clinical Neurology
Stephanie Meier, Eline A. J. Willemse, Sabine Schaedelin, Johanna Oechtering, Johannes Lorscheider, Lester Melie-Garcia, Alessandro Cagol, Muhamed Barakovic, Riccardo Galbusera, Suvitha Subramaniam, Christian Barro, Ahmed Abdelhak, Simon Thebault, Lutz Achtnichts, Patrice Lalive, Stefanie Muller, Caroline Pot, Anke Salmen, Giulio Disanto, Chiara Zecca, Marcus D'Souza, Annette Orleth, Michael Khalil, Arabella Buchmann, Renaud Du Pasquier, Ozgur Yaldizli, Tobias Derfuss, Klaus Berger, Marco Hermesdorf, Heinz Wiendl, Fredrik Piehl, Marco Battaglini, Urs Fischer, Ludwig Kappos, Claudio Gobbi, Cristina Granziera, Claire Bridel, David Leppert, Aleksandra Maleska Maceski, Pascal Benkert, Jens Kuhle
Summary: This study found that serum glial fibrillary acidic protein (sGFAP) and serum neurofilament light chain (sNfL) are correlated with features of disease progression in multiple sclerosis (MS) and can predict disease progression. sGFAP may serve as a useful biomarker for disease progression in MS in individual patient management and drug development.
Article
Clinical Neurology
Heinz Wiendl, Klaus Schmierer, Suzanne Hodgkinson, Tobias Derfuss, Andrew Chan, Finn Sellebjerg, Anat Achiron, Xavier Montalban, Alexandre Prat, Nicola De Stefano, Frederik Barkhof, Letizia Leocani, Patrick Vermersch, Anita Chudecka, Claire Mwape, Kristina H. Holmberg, Ursula Boschert, Sanjeev Roy, MAGNIFY-MS Study Grp
Summary: Cladribine tablets cause a reduction in lymphocytes, with a predominant effect on B-cell and T-cell counts. The MAGNIFY-MS substudy reports the dynamic changes on multiple peripheral blood mononuclear cell (PBMC) subtypes and immunoglobulin (Ig) levels over 12 months after the first course of cladribine tablets in patients with highly active relapsing multiple sclerosis (MS).
NEUROLOGY-NEUROIMMUNOLOGY & NEUROINFLAMMATION
(2023)
Letter
Clinical Neurology
Daniel Engels, Simone Mader, Stefanie Foerderreuther, Markus Reindl, Joachim Havla, Edgar Meinl, Tania Kuempfel, Lisa Ann Gerdes
ANNALS OF NEUROLOGY
(2023)
Editorial Material
Clinical Neurology
Franziska S. Thaler, Edgar Meinl
Article
Clinical Neurology
Jannis Muller, Sabine Schadelin, Johannes Lorscheider, Pascal Benkert, Peter Hanni, Jurg Schmid, Jens Kuhle, Tobias Derfuss, Cristina Granziera, Ozgur Yaldizli
Summary: This study compared the effectiveness of dimethyl fumarate (DMF) and teriflunomide in a real-world setting and found that DMF treatment was associated with better clinical outcomes.
EUROPEAN JOURNAL OF NEUROLOGY
(2023)
Article
Clinical Neurology
Nuria Cerda-Fuertes, Marc Stoessel, Gintaras Mickeliunas, Silvan Pless, Alessandro Cagol, Muhamed Barakovic, Aleksandra Maleska Maceski, Cesar alvarez Gonzalez, Marcus D' Souza, Sabine Schaedlin, Pascal Benkert, Pasquale Calabrese, Konstantin Gugleta, Tobias Derfuss, Till Sprenger, Cristina Granziera, Yvonne Naegelin, Ludwig Kappos, Jens Kuhle, Athina Papadopoulou
Summary: This study examined the relative role of optical coherence tomography (OCT) in multiple sclerosis (MS) and compared it with magnetic resonance imaging (MRI) and serum markers of disability. The results showed that OCT measures were associated with cognitive and physical disability, independent of serum and brain MRI markers of neuroaxonal loss.
MULTIPLE SCLEROSIS JOURNAL
(2023)
Review
Clinical Neurology
Jannis Mueller, Alessandro Cagol, Johannes Lorscheider, Charidimos Tsagkas, Pascal Benkert, Ozgur Yaldizli, Jens Kuhle, Tobias Derfuss, Maria Pia Sormani, Alan Thompson, Cristina Granziera, Ludwig Kappos
Summary: Emerging evidence suggests that progression independent of relapse activity (PIRA) is a substantial contributor to long-term disability accumulation in relapsing-remitting multiple sclerosis (RRMS). To date, there is no uniform agreed-upon definition of PIRA, limiting the comparability of published studies.
Letter
Clinical Neurology
Vinicius Oliveira Boldrini, Simone Mader, Tania Kuempfel, Edgar Meinl
Summary: Ublituximab is a newly FDA-approved treatment for relapsing forms of Multiple Sclerosis (MS). It works by depleting B cells while sparing long-lived plasma cells. Different anti-CD20 monoclonal antibodies with varying dose regimens, routes of application, glycoengineering and mechanisms of action may lead to different clinical outcomes.
MULTIPLE SCLEROSIS AND RELATED DISORDERS
(2023)
Article
Clinical Neurology
Ilaria Callegari, Mika Schneider, Vera Aebischer, Margarete M. M. Voortman, Undine Proschmann, Tjalf Ziemssen, Raija Lindberg, Bettina Fischer-Barnicol, Michael Khalil, Ludwig Kappos, Jens Kuhle, Nicholas S. R. Sanderson, Tobias Derfuss
Summary: This study found that natalizumab can diffuse in different anatomical compartments, including cerebrospinal fluid and milk. By developing a flow-cytometry-based assay and applying it to quantify natalizumab in body fluids, including cerebrospinal fluid, breastmilk, and serum, a better understanding of the safety of therapeutic antibody administration during pregnancy and lactation can be achieved.
THERAPEUTIC ADVANCES IN NEUROLOGICAL DISORDERS
(2023)
Article
Clinical Neurology
Nuria Cerda-Fuertes, Sara Nagy, Sabine Schaedelin, Tim Sinnecker, Esther Ruberte, Athina Papadopoulou, Jens Wurfel, Jens Kuhle, Ozgur Yaldizli, Ludwig Kappos, Tobias Derfuss, Bernhard F. Decard
Summary: This study aimed to describe the frequency, severity, and potential risk factors for recurring disease activity (RDA) in patients with multiple sclerosis (pwMS) after discontinuation of fingolimod (FGL). The results showed that younger age, shorter disease duration, and MRI activity during FGL treatment were independent risk factors for post-FGL RDA. Individual risk assessment and early switch to highly effective therapy can help minimize the risk of post-FGL RDA.
THERAPEUTIC ADVANCES IN NEUROLOGICAL DISORDERS
(2023)
Article
Clinical Neurology
Ana Beatriz Ayroza Galvao Ribeiro Gomes, Laila Kulsvehagen, Patrick Lipps, Alessandro Cagol, Nuria Cerda-Fuertes, Tradite Neziraj, Julia Flammer, Jasmine Lerner, Anne-Catherine Lecourt, Nina De Oliveira S. Siebenborn, Rosa Cortese, Sabine Schaedelin, Vinicius Andreoli Schoeps, Aline de Moura Brasil Matos, Natalia Trombini Mendes, Clarissa dos Reis Pereira, Mario Luiz Ribeiro Monteiro, Samira Luisa dos Apostolos-Pereira, Patrick Schindler, Claudia Chien, Carolin Schwake, Ruth Schneider, Thivya Pakeerathan, Orhan Aktas, Urs Fischer, Matthias Mehling, Tobias Derfuss, Ludwig Kappos, Ilya Ayzenberg, Marius Ringelstein, Friedemann Paul, Dagoberto Callegaro, Jens Kuhle, Athina Papadopoulou, Cristina Granziera, Anne-Katrin Probstel
Summary: This study investigated the frequency and clinical features of IgA antibodies against myelin oligodendrocyte glycoprotein (MOG) in patients with seronegative demyelinating central nervous system (CNS) disease. The results showed that MOG-specific IgA was identified in a subgroup of patients who were double-seronegative for aquaporin 4-/MOG-IgG, suggesting that MOG-IgA may be a novel diagnostic biomarker for patients with CNS demyelination.
