Article
Immunology
Wanyu Wang, Lingchun Liu, Mingzhi Zhang, Ruihan Yang, Da Liu, Shunyu Yang, Qiang Meng
Summary: This study reports a case of autoimmune nodopathy with both serum and CSF IgG4 anti-NF155 and anti-GD1b antibodies. Treatment with IVIg, rituximab, and plasma exchange led to a decrease in anti-NF155 antibody titer, but the patient's symptoms did not significantly improve.
FRONTIERS IN IMMUNOLOGY
(2022)
Review
Clinical Neurology
Deepak Menon, Hans Dieter Katzberg, Vera Bril
Summary: Different variants of CIDP have distinct clinical features and treatment responses, with a need for more high-quality evidence to support their management strategies.
FRONTIERS IN NEUROLOGY
(2021)
Article
Clinical Neurology
Wenqing Wang, Chang Liu, Wei Li, Dong Zhang, Yi Shan, Jinfan Zheng, Jingli Shan, Yuying Zhao, Chuanzhu Yan, Qinzhou Wang
Summary: This study investigated the clinical features of Han Chinese patients with anti-neurofascin-155 (NF155) antibody-positive neuropathy. Specific phenotypes were observed in a significant proportion of patients, and there may be ambiguous staining patterns and potential for false positivity.
ANNALS OF CLINICAL AND TRANSLATIONAL NEUROLOGY
(2022)
Article
Immunology
Yunfei Bai, Wei Li, Chuanzhu Yan, Ying Hou, Qinzhou Wang
Summary: A Chinese male patient with anti-NF155 nodopathy undergoing rituximab treatment showed the presence of anti-rituximab antibodies (ARAs), which had a negative impact on the efficacy of rituximab. This is the first reported case of ARAs in patients with anti-NF155 antibodies, highlighting the need for early testing of these antibodies.
FRONTIERS IN IMMUNOLOGY
(2023)
Article
Multidisciplinary Sciences
Lucas M. P. Chataigner, Christos Gogou, Maurits A. den Boer, Catia P. Frias, Dominique M. E. Thies-Weesie, Joke C. M. Granneman, Albert J. R. Heck, Dimphna H. Meijer, Bert J. C. Janssen
Summary: Cell-surface expressed contactin 1 and neurofascin 155 play important roles in controlling wiring of the nervous system. The crystallographic structures of the binding regions of contactin 1 and neurofascin 155 provide insights into the diverse adhesion sites formed through competing and complementary interfaces, post-translational glycosylation, splice differences, and structural plasticity. The structures also explain how these proteins enable the formation of specific intercellular distances and bridging of larger distances in the synapse.
NATURE COMMUNICATIONS
(2022)
Article
Clinical Neurology
Alexandre Jentzer, Arthur Attal, Clemence Roue, Julie Raymond, Cinta Lleixa, Isabel Illa, Luis Querol, Guillaume Taieb, Jerome Devaux
Summary: This study found that monospecific and bivalent anti-Nfasc155 IgG4 antibodies are present in patients and are pathogenic. Transforming these bivalent antibodies into monovalent Fab or IgG4 through FAE significantly reduces paranodal damage.
NEUROLOGY-NEUROIMMUNOLOGY & NEUROINFLAMMATION
(2022)
Article
Immunology
Sheng-Hui Chang, Jing Wang, Xu Zhang, Ning Zhao, Kun Jia, Ming Yi, Qiu-Xia Zhang, Hui Zhai, Xiao-Wen Li, Chun-Sheng Yang, Li Yang, Lin-Jie Zhang
Summary: A small proportion of NMOSD patients have anti-NF155 antibodies, which are more likely to co-exist with autoimmune diseases. These patients with anti-NF155 antibodies tended to show mildly abnormal results in nerve conduction studies.
CLINICAL AND EXPERIMENTAL IMMUNOLOGY
(2021)
Review
Clinical Neurology
Joaquin A. Vizcarra, Taylor B. Harrison, Rocio Garcia-Santibanez
Summary: This paper provides an up-to-date review of immune-mediated neuropathies secondary to antibodies against nodal and paranodal proteins. Recent findings show that antibodies to neurofascins and contactin are associated with characteristic neuropathy presentations, with variable treatment response.NF155 may have a better response to plasma exchange and rituximab.
CURRENT TREATMENT OPTIONS IN NEUROLOGY
(2021)
Article
Clinical Neurology
Ning Zhao, Sheng-Hui Chang, Qiu-Xia Zhang, Lin-Jie Zhang, Shu-Min Jiang, Hui Zhai, Li Yang
Summary: This study found that a small portion of GBS patients have anti-NF155 antibodies, and GBS patients with positive anti-NF155 antibodies may have a more severe clinical course, less involvement of sensory nerves, higher proportion of axonal type, poorer therapeutic effect, and worse prognosis.
ACTA NEUROLOGICA SCANDINAVICA
(2022)
Review
Clinical Neurology
Jun-ichi Kira
Summary: CIDP is an immune-mediated demyelinating disease of the PNS, with NF155(+) CIDP characterized by demyelinating lesions in both the CNS and PNS, due to the upregulation of Th2 and Th1 cytokines resulting from IgG4 class switching, leading to spinal root inflammation and lack of macrophage infiltration in sural nerves.
FRONTIERS IN NEUROLOGY
(2021)
Review
Clinical Neurology
Pranjal Gupta, Igal Mirman, Shelly Shahar, Divyanshu Dubey
Summary: This review discusses the advancements made in the field of autoimmune neuropathies secondary to antibodies to nodal and paranodal proteins. Antibodies to nodal-paranodal antigens have emerged as a biomarker for a novel type of immune-mediated neuropathies, with distinct pathogenic mechanisms and clinicopathologic features. Treatment strategies may vary depending on the antibody isotype.
CURRENT NEUROLOGY AND NEUROSCIENCE REPORTS
(2023)
Article
Neurosciences
Lijun Wang, Jing Pan, Huanyu Meng, Zhao Yang, Lili Zeng, Jun Liu
Summary: This case study presents a patient with CIDP seropositive for anti-neurofascin (NF) 155 and NF186 IgG4 antibodies who showed clinical improvement after rituximab therapy. A literature review was conducted to explore the clinical characteristics of anti-NF155/NF186-positive autoimmune nodopathy (AN).
Article
Clinical Neurology
Anna Kostera-Pruszczyk, Anna Potulska-Chromik, Malgorzata Lukawska, Marta Lipowska, Dorota Hoffman-Zacharska, Beata Olchowik, Magdalena Figlerowicz, Karolina Kanabus, Edyta Rosiak
Summary: Childhood CIDP can manifest in atypical forms in over 50% of cases, including distal, pure motor, or pure sensory variants. While most children have a good prognosis, long-term treatment may be necessary for some patients.
FRONTIERS IN NEUROLOGY
(2021)
Article
Multidisciplinary Sciences
Shannon J. McKie, Alex S. Nicholson, Emily Smith, Stuart Fawke, Eve R. Caroe, James C. Williamson, Benjamin G. Butt, Denisa Kolarova, Ondrej Peterka, Michal Holcapek, Paul J. Lehner, Stephen C. Graham, Janet E. Deane
Summary: Myelin is a multilayered membrane that tightly wraps neuronal axons, enabling efficient, high-speed signal propagation. This study reveals that altered lipid metabolism affects the abundance of specific plasma membrane proteins, which play important roles in cell adhesion and signaling, and are implicated in neurological diseases. The study also demonstrates the direct interaction between the adhesion molecule neurofascin (NFASC) and the sphingolipid sulfatide, providing insights into the pathogenesis of demyelinating diseases.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
(2023)
Article
Clinical Neurology
Okan Sokmen, Mehmet Demirci, Ersin Tan
Summary: Combined central and peripheral demyelination (CCPD) is a rare condition involving simultaneous demyelination in the CNS and PNS, potentially due to a shared immune mechanism. A small number of CIDP patients have autoantibodies, with NF155 + CIDP typically affecting young adults and Rituximab showing promising results in treatment.
CLINICAL NEUROLOGY AND NEUROSURGERY
(2021)
Letter
Clinical Neurology
Andreas Meisel, Fulvio Baggi, Anthony Behin, Amelia Evoli, Anna Kostera-Pruszczyk, Renato Mantegazza, Raul Juntas Morales, Anna Rostedt Punga, Sabrina Sacconi, Michael Schroeter, Jan Verschuuren, Louise Crathorne, Kris Holmes, Maria-Isabel Leite
EUROPEAN JOURNAL OF NEUROLOGY
(2023)
Article
Clinical Neurology
Huma Fatima Ali, Lea Fast, Ahmed Khalil, Eberhard Siebert, Thomas Liman, Matthias Endres, Kersten Villringer, Anna Kufner
Summary: This study investigated the relationship between cerebrovascular risk factors and white matter hyperintensities (WMH) load, as well as the impact of WMH burden on recurrent vascular events, cognitive impairment, and depression in first-ever ischemic stroke patients. The findings revealed that higher WMH burden was significantly associated with a decline in cognition 3 years post-stroke.
JOURNAL OF NEUROLOGY
(2023)
Letter
Clinical Neurology
Sarah Hoffmann, Werner Stenzel, Andreas Meisel
JOURNAL OF THE NEUROLOGICAL SCIENCES
(2023)
Article
Clinical Neurology
Joao Pedro Marto, Davide N. Strambo, George Ntaios, Thanh Nguyen, Roman Herzig, Anna Czlonkowska, Jelle Demeestere, Ossama Yassin Mansour, Alexander Salerno, Susanne W. Wegener, Philipp Baumgartner, Carlo Cereda, Giovanni Bianco, Morin Beyeler, Marcel Arnold, Emmanuel Carrera, Paolo Machi, Valerian Altersberger, Leo Bonati, Henrik Gensicke, Manuel Bolognese, Nils Peters, Stephan Wetzel, Marta Magrico, Joao Nuno Ramos, Joao Sargento-Freitas, Rita Machado, Carolina Maia, Egidio Machado, Ana Paiva Nunes, Patricia Ferreira, Teresa Pinho e Melo, Mariana Carvalho Dias, Andre Paula, Manuel Alberto Correia, Pedro Castro, Elsa Azevedo, Luis Albuquerque, Jose Nuno Alves, Joana Ferreira-Pinto, Torcato Meira, Liliana Pereira, Miguel Rodrigues, Andre Pinho Araujo, Marta Rodrigues, Mariana Rocha, Angelo Pereira-Fonseca, Luis Ribeiro, Ricardo Varela, Sofia Malheiro, Manuel Cappellari, Cecilia Zivelonghi, Giulia Sajeva, Andrea Zini, Mauro Gentile, Stefano Forlivesi, Ludovica Migliaccio, Maria Sessa, Sara La Gioia, Alessandro Pezzini, Davide Sangalli, Marialuisa Zedde, Rosario Pascarella, Carlo Ferrarese, Simone Beretta, Susanna Diamanti, Ghil Schwarz, Giovanni Frisullo, Simona Marcheselli, Pierre Seners, Candice Sabben, Simon Escalard, Michel Piotin, Benjamin Maier, Guillaume Charbonnier, Fabrice Vuillier, Loic R. Legris, Pauline Cuisenier, Francesca Vodret, Gaultier Marnat, Jean-Sebastien Liegey, Igor Sibon, Fabian Flottmann, Gabriel O. Broocks, Nils-Ole Gloyer, Ferdinand Bohmann, Jan Hendrik J. Schaefer, Christian Nolte, Heinrich Audebert, Eberhard Siebert, Marek Sykora, Wilfried Lang, Julia Ferrari, Lukas Mayer-Suess, Michael Knoflach, Elke Ruth J. Gizewski, Jeffrey M. Stolp, Lotte Stolze, Jonathan Coutinho, Paul Nederkoorn, Ido van den Wijngaard, Joke De Meris, Robin Lemmens, Sylvie De Raedt, Fenne Vandervorst, Matthieu Pierre Rutgers, Antoine Guilmot, Anne Dusart, Flavio Bellante, Patricia Calleja-Castano, Fernando Ostos, Guillermo Gonzalez-Ortega, Paloma Martin-Jimenez, Sebastian Garcia-Madrona, Antonio Cruz-Culebras, Rocio Vera, Maria Consuelo Matute, Blanca Fuentes, Maria Alonso-de-Lecinana, Ricardo Rigual, Exuperio Diez-Tejedor, Soledad Perez-Sanchez, Joan Montaner, Fernando Diaz-Otero, Natalia Perez-de-la-Ossa, Belen Flores-Pina, Lucia Munoz-Narbona, Angel Chamorro, Alejandro Rodriguez-Vazquez, Arturo Renu, Oscar Ayo-Martin, Francisco Hernandez-Fernandez, Tomas Segura, Herbert Tejada-Meza, Daniel Sagarra-Mur, Marta Serrano-Ponz, Thant Hlaing, Isaiah See, Robert Simister, David Werring, Espen Saxhaug Kristoffersen, Annika Nordanstig, Katarina Jood, Alexandros Rentzos, Libor Simunek, Dagmar Krajickova, Antonin Krajina, Robert Mikulik, Martina Cvikova, Jan Vinklarek, David Skoloudik, Martin Roubec, Eva Hurtikova, Rostislav Hruby, Svatopluk Ostry, Ondrej Skoda, Marek Pernicka, Lubomir Jurak, Zuzana Eichlova, Martin Jira, Martin Kovar, Michal Pansky, Pavel Mencl, Hana Palouskova, Ales Tomek, Petr Jansky, Anna Olserova, Martin Sramek, Roman Havlicek, Petr Maly, Lukas Trakal, Jan Fiksa, Matej Slovak, Michal Adam Karlinski, Maciej Nowak, Halina Sienkiewicz-Jarosz, Anna Bochynska, Pawel Wrona, Tomasz Homa, Katarzyna Sawczynska, Agnieszka Slowik, Ewa Wlodarczyk, Marcin Wiacek, Izabella Tomaszewska-Lampart, Bartosz Sieczkowski, Halina Bartosik-Psujek, Marta Bilik, Anna Bandzarewicz, Malgorzata Dorobek, Justyna Zielinska-Turek, Marta Nowakowska-Kotas, Krystian Obara, Pawel Urbanowski, Slawomir Budrewicz, Maciej Guzinski, Milena Switonska, Iwona Rutkowska, Paulina M. Sobieszak-Skura, Beata Labuz-Roszak, Aleksander Debiec, Jacek Staszewski, Adam Stepien, Jacek Zwiernik, Grzegorz Wasilewski, Cristina Tiu, Elena Oana Terecoasa, Razvan Alexandru Radu, Anca Negrila, Bogdan Dorobat, Cristina Panea, Vlad Tiu, Simona Petrescu, Atilla Ozdemir, Mostafa Mahmoud, Hussam El-Samahy, Hazem Abdelkhalek, Jasem Al-Hashel, Ismail Ibrahim Ismail, Athari Salmeen, Abdoreza Ghoreishi, Sergiu Ionut Sabetay, Hana Gross, Piers Klein, Mohamad Abdalkader, Pascal Jabbour, Kareem El Naamani, Stavropoula Tjoumakaris, Rawad A. Abbas, Ghada Mohamed, Alex Chebl, Jiangyong Min, Majesta P. Hovingh, Jenney Tsai, Muhib Khan, Krishna Nalleballe, Sanjeeva Onteddu, Hesham Masoud, Mina Michael, Navreet Kaur, Laith G. Maali, Michael Abraham, Priyank Khandelwal, Ivo Bach, Melody Ong, Denis M. Babici, Ayaz Khawaja, Maryam Hakemi, Kumar Rajamani, Vanessa Cano-Nigenda, Antonio Arauz, Pablo Amaya, Natalia Llanos, Akemi Arango, Miguel Angel Vences, Jose Dominguo Barrientos Guerra, Rayllene Caetano, Rodrigo Targa Martins, Sergio Daniel Scollo, Patrick Matic Yalung, Shashank Nagendra, Abhijit Gaikwad, Kwon-Duk Seo, Georgios G. Georgiopoulos, Raul Nogueira, Patrik Michel
Summary: This retrospective study evaluated the safety and outcomes of revascularization treatments in patients with acute ischemic stroke and COVID-19. COVID-19 patients had higher rates of intracranial bleeding complications and worse clinical outcomes after treatment compared to non-COVID-19 patients.
Article
Clinical Neurology
Sarah Hoffmann, Patrick Waters, Leslie Jacobson, Markus Schuelke, Werner Stenzel, Tobias Ruck, Sophie Lehnerer, Frauke Stascheit, Corinna Preusse, Andreas Meisel
Summary: Autoantibody testing is essential for autoimmune myasthenia gravis diagnosis, but about 15% of patients still show negative results (seronegative MG). This study examined the prevalence of clustered AChR, MuSK, and LRP4 autoantibodies in a large German cohort of seronegative MG patients using a live cell-based assay. Out of 67 SNMG patients, 4.5% had clustered AChR autoantibodies, with two patients showing binding to both adult and fetal AChR. None of the patients tested positive for MuSK or LRP4 autoantibodies. Clinical characteristics were similar between patients with and without clustered AChR autoantibodies. Comparisons with a national MG registry showed broad similarities among seronegative MG patients in both cohorts.
NEUROMUSCULAR DISORDERS
(2023)
Article
Clinical Neurology
Erkan Celik, Lukas Goertz, Yigit Ozpeynirci, Marc Schlamann, Franziska Dorn, Nils Lehnen, Eberhard Siebert, Thomas Liebig, Christoph Kabbasch
Summary: A retrospective study compared the effectiveness of WEB embolization and standard coiling for treating ruptured basilar tip aneurysms. Both methods showed similar efficacy in preventing rebleeding, but WEB embolization required less stent-assistance, potentially beneficial for avoiding anti-platelet therapy in SAH patients.
Article
Clinical Neurology
Christoph Riegler, Janina R. Behrens, Claudia Gorski, Anselm Angermaier, Stephan Kinze, Ramanan Ganeshan, Andrea Rocco, Alexander Kunz, Tobias J. Muller, Andreas Bitsch, Albert Grueger, Joachim E. Weber, Eberhard Siebert, Kerstin Bollweg, Regina von Rennenberg, Heinrich J. Audebert, Christian H. Nolte, Hebun Erdur
Summary: This study compared the time-to-care metrics and outcomes of stroke patients undergoing interhospital transfer for mechanical thrombectomy in rural and metropolitan areas. The results showed that although rural patients had longer time from arrival at the primary stroke center to start of intravenous thrombolysis, they underwent groin puncture at comprehensive stroke centers faster. Despite longer transport distances in rural areas, there was no significant difference in the time from arrival at the primary stroke center to groin puncture compared to metropolitan areas. Overall, the time-to-care metrics were similar in both rural and metropolitan areas.
FRONTIERS IN NEUROLOGY
(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
Frauke Stascheit, Adela Della Marina, Andreas Meisel
Summary: The treatment of myasthenia gravis is focused on the patient's antibody status. Various therapies including steroids, immunosuppressive treatments, and thymectomy are commonly used. New therapeutic approaches have been developed for patients with acetylcholine receptor antibody and muscle-specific receptor tyrosine kinase antibody. The use of modern immunomodulators based on disease activity is recommended.
Article
Clinical Neurology
Antje Mevius, Lars Joeres, Jutta Biskup, Tanja Heidbrede, Milada Mahic, Thomas Wilke, Ulf Maywald, Sophie Lehnerer, Andreas Meisel
Summary: The aim of this retrospective cohort study was to estimate the prevalence and incidence of myasthenia gravis (MG) in Germany and understand the burden of disease and treatment patterns. The study found that the prevalence of MG in Germany was approximately 39.3/10 0,0 0 0, and the incidence in 2019 was 4.6/10 0,0 0 0. A significant proportion of MG patients remained untreated, and many patients experienced exacerbations or myasthenic crises. MG was associated with higher mortality compared to the general non-MG population.
NEUROMUSCULAR DISORDERS
(2023)
Article
Clinical Neurology
Thanh N. Nguyen, Muhammad M. Qureshi, Davide Strambo, Daniel Strbian, Silja Raety, Christian Herweh, Mohamad Abdalkader, Marta Olive-Gadea, Marc Ribo, Marios Psychogios, Urs Fischer, Anh Nguyen, Joji B. Kuramatsu, David Haupenthal, Martin Koehrmann, Cornelius Deuschl, Jordi Kuehne Escola, Shadi Yaghi, Liqi Shu, Volker Puetz, Daniel P. O. Kaiser, Johannes Kaesmacher, Adnan Mujanovic, Dominique Cornelius Marterstock, Tobias Engelhorn, Piers Klein, Diogo C. Haussen, Mahmoud H. Mohammaden, Hend Abdelhamid, Lorena Souza Viana, Bruno Cunha, Isabel Fragata, Michele Romoli, Francesco Diana, Pekka Virtanen, Kimmo Lappalainen, Judith Clark, Stavros Matsoukas, Johanna T. Fifi, Sunil A. Sheth, Sergio Salazar-Marioni, Joao Pedro Marto, Joao Nuno Ramos, Milena Miszczuk, Christoph Riegler, Ashutosh P. Jadhav, Shashvat M. Desai, Volker Maus, Maximilian Kaeder, Adnan H. Siddiqui, Andre Monteiro, Hesham E. Masoud, Neil Suryadevara, Maxim Mokin, Shail Thanki, James E. Siegler, Jane Khalife, Italo Linfante, Guilherme Dabus, Negar Asdaghi, Vasu Saini, Christian H. Nolte, Eberhard Siebert, Thomas R. Meinel, Stefanos Finitsis, Markus A. Moehlenbruch, Peter A. Ringleb, Anne Berberich, Raul G. Nogueira, Uta Hanning, Lukas Meyer, Patrik Michel, Simon Nagel
Summary: This study compared the clinical outcomes of endovascular therapy (EVT) and medical management (MM) in patients with isolated posterior cerebral artery occlusion. The results showed no difference between the two groups in terms of the modified Rankin Scale and National Institutes of Health Stroke Scale at 90 days. However, the EVT group had a higher likelihood of excellent outcome and complete vision recovery, despite higher rates of symptomatic intracranial hemorrhage and mortality.
Article
Medicine, General & Internal
Sonia Berrih-Aknin, Jacqueline Palace, Andreas Meisel, Kristl G. Claeys, Srikanth Muppidi, Francesco Sacca, Fatemeh Amini, Mark Larkin, Casey Quinn, Jon Beauchamp, Glenn Philips, Femke De Ruyck, Joyce Ramirez, Sandra Paci
Summary: This study aims to explore the impact of myasthenia gravis (MG) in the real world from a patient perspective. The study included MG patients from different countries and found that despite current treatments, patients still experience a significant burden.
Article
Clinical Neurology
Lea Fast, Uchralt Temuulen, Kersten Villringer, Anna Kufner, Huma Fatima Ali, Eberhard Siebert, Shufan Huo, Sophie K. K. Piper, Pia Sophie Sperber, Thomas Liman, Matthias Endres, Kerstin Ritter
Summary: This study compared the prediction of clinical outcomes in first-ever ischemic stroke patients using advanced machine learning techniques and identified the leading prognostic factors.
FRONTIERS IN NEUROLOGY
(2023)
Article
Multidisciplinary Sciences
Kathrin Goedde, Hella Fuegemann, Ute Goerling, Ulrike Grittner, Raphael Kohl, Andreas Meisel, Thomas Reinhold, Susanne J. Schnitzer, P. Markus Deckert, Nikolaj Frost, Stephan Schreiber, Nina Rieckmann, Christine Holmberg
Summary: This feasibility study aims to evaluate a patient-oriented navigation model that integrates data about barriers to care, vulnerable patient populations, and existing support services. The study consists of two randomized controlled trials and observational cohorts, with the intervention group receiving support from personal navigators for 12 months while the control group receives a brochure with regional support offers. The feasibility of the navigation model is evaluated in terms of acceptance, demand, practicality, and efficacy.