Article
Multidisciplinary Sciences
Valentina Damato, Jakob Theorell, Adam Al-Diwani, Anne-Kathrin Kienzler, Mateusz Makuch, Bo Sun, Adam Handel, Deniz Akdeniz, Antonio Berretta, Sudarshini Ramanathan, Andrew Fower, Daniel Whittam, Emily Gibbons, Nicholas McGlashan, Edward Green, Saif Huda, Mark Woodhall, Jacqueline Palace, Fintan Sheerin, Patrick Waters, Maria Leite, Anu Jacob, Sarosh R. Irani
Summary: This study reveals the relationship between clinical relapses and germinal center activity in patients with neuromyelitis optica spectrum disorders (NMOSDs), and shows that rituximab can suppress germinal center activity and reduce the production of AQP4 antibodies.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
(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
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)
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)
Article
Immunology
Liang Wang, Lei Du, Qinying Li, Fang Li, Bei Wang, Yuanqi Zhao, Qiang Meng, Wenyu Li, Juyuan Pan, Junhui Xia, Shitao Wu, Jie Yang, Heng Li, Jianhua Ma, Jingzi ZhangBao, Wenjuan Huang, Xuechun Chang, Hongmei Tan, Jian Yu, Lei Zhou, Chuanzhen Lu, Min Wang, Qiang Dong, Jiahong Lu, Chongbo Zhao, Chao Quan
Summary: This study investigates the predictors of relapse, and severe visual or motor disability in patients with NMOSD and develops corresponding predictive models. Early identification of patients at risk of unfavorable outcomes is crucial for informing treatment decisions.
FRONTIERS IN IMMUNOLOGY
(2022)
Article
Immunology
Yi Bu, Heng Liu, XuDong Qian, Fan Sun, ChengBo Li, Jingzhe Han
Summary: Neuromyelitis optica spectrum disease (NMOSD) is a debilitating autoimmune inflammatory demyelinating disease of the central nervous system. The relationship between harboring an infection and NMOSD is currently unclear and needs further investigation. This article reports meningoencephalitis-like manifestations in a patient with serum AQP4 antibody-positive area postrema syndrome (APS), suggesting a possible association with NMOSD.
FRONTIERS IN IMMUNOLOGY
(2022)
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)
Article
Clinical Neurology
Liang Wang, Hongmei Tan, Wenjuan Huang, Xuechun Chang, Jingzi ZhangBao, Lei Zhou, Chuanzhen Lu, Min Wang, Jiahong Lu, Chongbo Zhao, Chao Quan
Summary: This study evaluated the clinical characteristics and prognosis of late onset neuromyelitis optica spectrum disorder (LO-NMOSD) and compared them with early onset NMOSD (EO-NMOSD) and NMOSD with different antibody serostatuses. The study found that patients with LO-NMOSD, especially those with AQP4 antibodies, had a worse prognosis. Age at disease onset and antibody serostatus were predictors of blindness and motor dysfunction.
EUROPEAN JOURNAL OF NEUROLOGY
(2022)
Review
Biochemistry & Molecular Biology
Yoichiro Abe, Masato Yasui
Summary: The discovery of NMO-IgG in patients with neuromyelitis optica spectrum disorder (NMOSD) has highlighted the importance of the water channel aquaporin-4 (AQP4) in autoimmune diseases of the central nervous system. The unique structure of AQP4 and its subcellular localization may play a crucial role in the pathogenesis of NMOSD, in addition to the complement-dependent cytotoxic effects of NMO-IgG. Studies have also shown complement-independent cytotoxic effects of NMO-IgG, potentially involving antibody-induced endocytosis of AQP4.
Article
Clinical Neurology
Tetsuya Akaishi, Toshiyuki Takahashi, Kazuo Fujihara, Tatsuro Misu, Juichi Fujimori, Yoshiki Takai, Shuhei Nishiyama, Michiaki Abe, Tadashi Ishii, Masashi Aoki, Ichiro Nakashima
Summary: NMOSD patients positive for AQP4-IgG have a higher association with Sjogren's syndrome, with a higher prevalence of comorbid SjS among female patients and a higher relapse rate.
JOURNAL OF 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
Marina Herwerth, Selin Kenet, Martina Schifferer, Anne Winkler, Melanie Weber, Nicolas Snaidero, Mengzhe Wang, Melanie Lohrberg, Jeffrey L. Bennett, Christine Stadelmann, Bernhard Hemmer, Thomas Misgeld
Summary: Researchers have identified a distinct form of immune-mediated axonal injury in a model of neuromyelitis optica, which can be prevented by microtubule stabilization. These findings suggest a promising strategy for neuroprotection in NMO and related diseases.
Article
Medicine, Research & Experimental
Leung-Wah Yick, Oscar Ka-Fai Ma, Ethel Yin-Ying Chan, Krystal Xiwing Yau, Jason Shing-Cheong Kwan, Koon-Ho Chan
Summary: NMO spectrum disorders (NMOSD) are autoimmune inflammatory disorders of the CNS mediated by AQP4-IgGs. In a mouse model of NMOSD, dysregulated Tfh cells play a role in the pathogenesis, and targeting Tfh cells can ameliorate the symptoms of NMOSD.
Article
Immunology
Yasunobu Hoshino, Daisuke Noto, Shuhei Sano, Yuji Tomizawa, Kazumasa Yokoyama, Nobutaka Hattori, Sachiko Miyake
Summary: This study demonstrates that B cells in patients with NMOSD are abnormally skewed towards antibody-secreting cells at the transcriptome level during the early differentiation phase, and IL-2 might participate in this pathogenic process. CD25(+) naive B cells are identified as a novel candidate precursor of antibody-secreting cells in autoimmune diseases.
JOURNAL OF NEUROINFLAMMATION
(2022)
Article
Clinical Neurology
Jonathan A. Gernert, Rebecca Wicklein, Bernhard Hemmer, Tania Kumpfel, Benjamin Knier, Joachim Havla
Summary: Peripapillary hyperreflective ovoid mass-like structures (PHOMS) has been identified as a new marker in optical coherence tomography (OCT). This study found that PHOMS occurred in 17% of AQP4+ NMOSD patients and 14% of MOGAD patients, which is comparable to the prevalence in MS patients.
JOURNAL OF NEUROLOGY
(2023)
Article
Clinical Neurology
Alexander Wuschek, Matthias Bussas, Malek El Husseini, Laura Harabacz, Viktor Pineker, Viola Pongratz, Achim Berthele, Isabelle Riederer, Claus Zimmer, Bernhard Hemmer, Jan S. Kirschke, Mark Muhlau
Summary: In patients with early multiple sclerosis (MS), abnormal tibial nerve somatosensory evoked potentials (tSSEP) were less frequent than spinal cord MRI lesions. Higher disability scores were significantly associated with abnormal tSSEP but not abnormal spinal cord MRI. Therefore, in early MS, lesions detected by MRI are the main driver of abnormal tSSEP.
JOURNAL OF NEUROLOGY
(2023)
Article
Clinical Neurology
Isabel Lutfullin, Maria Eveslage, Stefan Bittner, Gisela Antony, Martina Flaskamp, Felix Luessi, Anke Salmen, Barbara Gisevius, Luisa Klotz, Catharina Korsukewitz, Achim Berthele, Sergiu Groppa, Florian Then Bergh, Brigitte Wildemann, Antonios Bayas, Hayrettin Tumani, Sven G. Meuth, Corinna Trebst, Uwe K. Zettl, Friedemann Paul, Christoph Heesen, Tania Kuempfel, Ralf Gold, Bernhard Hemmer, Frauke Zipp, Heinz Wiendl, Jan D. Luenemann
Summary: Obesity is associated with higher disease severity and poorer outcome in newly diagnosed patients with multiple sclerosis. The presence of obesity at disease onset is linked to higher disability at baseline and at follow-up periods of 2, 4, and 6 years. Obesity management could potentially improve the clinical outcome of multiple sclerosis.
JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY
(2023)
Article
Clinical Neurology
Josephine Wauschkuhn, Gilberto Solorza Buenrostro, Lilian Aly, Susanna Asseyer, Rebecca Wicklein, Julia Maria Hartberger, Klemens Ruprecht, Mark Muehlau, Tanja Schmitz-Huebsch, Claudia Chien, Achim Berthele, Alexander U. U. Brandt, Thomas Korn, Friedemann Paul, Bernhard Hemmer, Hanna G. G. Zimmermann, Benjamin Knier
Summary: Thinning of retinal combined ganglion cell and inner plexiform layer (GCIP) as measured by OCT is common in patients with multiple sclerosis. This study aimed to investigate whether a single retinal OCT analysis allows prediction of future disease activity after a first demyelinating event. The results showed that GCIP thickness measurement provides information for early risk stratification and progression of multiple sclerosis after the initial manifestation.
EUROPEAN JOURNAL OF NEUROLOGY
(2023)
Article
Clinical Neurology
Johanna Haertl, Julia Hartberger, Silke Wunderlich, Isabell Cordts, Cemsel Bafligil, Marc Sturm, Dominik Westphal, Tobias Haack, Bernhard Hemmer, Benno David Ikenberg, Marcus Deschauer
Summary: This study found that genetic variants have a crucial impact on the occurrence of ischemic stroke in young individuals. Genetic screening can help identify genetic diseases causing stroke and provide valuable information for the medical clarification of acute stroke.
JOURNAL OF NEUROLOGY
(2023)
Article
Clinical Neurology
Bernhard Hemmer, Heinz Wiendl, Karsten Roth, Hendrik Wessels, Josef Hoefler, Cyrill Hornuss, Bernd Liedert, Krzysztof Selmaj
Summary: This study aimed to evaluate the matching efficacy, safety, and immunogenicity between biosim-NTZ and ref-NTZ in patients with RRMS. The results showed that biosim-NTZ matched ref-NTZ in terms of efficacy, safety, and immunogenicity. This study supports the use of biosim-NTZ as a biosimilar alternative for treating RRMS.
Article
Clinical Neurology
Susana Otero-Romero, Christine Lebrun-Frenay, Saul Reyes, Maria Pia Amato, Magda Campins, Mauricio Farez, Massimo Filippi, Yael Hacohen, Bernhard Hemmer, Rosa Juuti, Melinda Magyari, Celia Oreja-Guevara, Aksel Siva, Sandra Vukusic, Mar Tintore
Summary: This study developed a European consensus on vaccination strategies for people with multiple sclerosis (pwMS) who are candidates for disease-modifying therapies (DMTs). A multidisciplinary working group used formal consensus methodology to formulate recommendations based on the quality of evidence and risk-benefit balance. A total of 53 recommendations were agreed upon, aiming to homogenize immunization practices in pwMS.
EUROPEAN JOURNAL OF NEUROLOGY
(2023)
Review
Clinical Neurology
Susana Otero-Romero, Christine Lebrun-Frenay, Saul Reyes, Maria Pia Amato, Magda Campins, Mauricio Farez, Massimo Filippi, Yael Hacohen, Bernhard Hemmer, Rosa Juuti, Melinda Magyari, Celia Oreja-Guevara, Aksel Siva, Sandra Vukusic, Mar Tintore
Summary: This study aims to develop an evidence-based vaccination strategy for pwMS who are candidates for disease-modifying therapies. A multidisciplinary working group conducted a systematic literature search and formulated 53 recommendations based on the quality of evidence. The goal of this study is to homogenize immunization practices in pwMS.
MULTIPLE SCLEROSIS JOURNAL
(2023)
Editorial Material
Clinical Neurology
Felix Hess, Paula Uibel, Achim Berthele, Bernhard Hemmer
Article
Clinical Neurology
Johanna Haertl, Martin Renz, Silke Wunderlich, Bernhard Hemmer, Benedikt Hofauer, Jens Gempt, Michael Kallmayer, Tobias Boeckh-Behrens, Jan S. Kirschke, Benno David Ikenberg
Summary: Bone or cartilage anomalies can cause ischemic stroke, which is referred to as bony stroke. There is no standardized diagnostic and therapeutic approach for bony strokes due to their rarity. In this study, we retrospectively analyzed the diagnostic and therapeutic workup of patients with bony strokes at our center between January 2017 and March 2022.
Article
Clinical Neurology
Ali M. Afzali, Philipp Moog, Sudhakar Reddy Kalluri, Benedikt Hofauer, Andreas Knopf, Jan Stefan Kirschke, Bernhard Hemmer, Achim Berthele
Summary: The study aimed to assess the prevalence, clinical characteristics, and therapeutic outcomes of CNS demyelinating disease in patients with primary Sjogren's syndrome (pSS). Among 194 pSS patients, 22 had CNS manifestations, including 19 with demyelination. The CNS manifestations in pSS can mimic multiple sclerosis (MS), and treatment with B-cell-depleting agents may be more effective.
FRONTIERS IN NEUROLOGY
(2023)
Article
Clinical Neurology
Sonia Darvishi, Ewan Donnachie, Christiane Gasperi, Alexander Hapfelmeier, Bernhard Hemmer
Summary: This study found that patients with MS have a lower vaccination frequency despite the risk of infection. Increasing awareness about the importance of vaccination is needed, especially in MS patients receiving immunotherapies.
MULTIPLE SCLEROSIS JOURNAL
(2023)
Article
Multidisciplinary Sciences
Isabel J. Bauer, Ping Fang, Katrin F. Lammle, Sofia Tyystjarvi, Dominik Alterauge, Dirk Baumjohann, Hongsup Yoon, Thomas Korn, Hartmut Wekerle, Naoto Kawakami
Summary: This study explores the impact of microbiota on T cell activation and their role in triggering CNS inflammation using intravital imaging. The results show that microbiota stimulation in the small intestine leads to calcium signaling in T cells, which is microbiota and MHC class II dependent. This stimulation induces the expression of Th17-axis genes in encephalitogenic T cells and facilitates their migration into the CNS.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
(2023)
Article
Multidisciplinary Sciences
Dominic Bertram, Thanos Tsaktanis, Achim Berthele, Thomas Korn
Summary: Intrathecal synthesis of free light chains kappa (FLCK) is a reliable marker for differentiating inflammatory CNS pathologies, particularly autoimmune encephalitis (AIE), from non-inflammatory etiologies in subacute onset neuropsychiatric syndromes.
SCIENTIFIC REPORTS
(2023)