Article
Immunology
Daidi Zhao, Kaixi Ren, Jiarui Lu, Zhiqin Liu, Zunbo Li, Jun Wu, Zhihao Xu, Songdi Wu, Tao Lei, Chao Ma, Sijia Zhao, Miao Bai, Hongzeng Li, Jun Guo
Summary: This study evaluated the efficacy and safety of a lower-dose rituximab (RTX) therapy for neuromyelitis optica spectrum disorder (NMOSD) based on a multicenter, open-label, prospective follow-up study. The results demonstrated that the lower-dose RTX strategy significantly reduced relapse rate, improved disability score, and decreased spinal cord lesion. Early therapy initiation showed better outcomes compared to delayed therapy.
FRONTIERS IN IMMUNOLOGY
(2023)
Article
Hematology
Philip A. Thompson, Alexandre Bazinet, William G. Wierda, Constantine S. Tam, Susan M. O'Brien, Satabdi Saha, Christine B. Peterson, William Plunkett, Michael J. Keating
Summary: Chemoimmunotherapy with FCR achieves durable remissions, especially in patients with mutated immunoglobulin heavy chain variable gene (IGHV-M). The median progression-free survival (PFS) for IGHV-M patients is 14.6 years, compared to 4.2 years for patients with unmutated IGHV (IGHV-UM). A risk-benefit assessment is necessary when counseling patients.
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
Oncology
Jorge J. Castillo, Kirsten Meid, Joshua N. Gustine, Carly Leventoff, Timothy White, Catherine A. Flynn, Shayna Sarosiek, Maria G. Demos, Maria L. Guerrera, Amanda Kofides, Xia Liu, Manit Munshi, Nicholas Tsakmaklis, Lian Xu, Guang Yang, Andrew R. Branagan, Elizabeth O'Donnell, Noopur Raje, Andrew J. Yee, Christopher J. Patterson, Zachary R. Hunter, Steven P. Treon
Summary: Ibrutinib monotherapy showed durable responses in treatment-naive patients with Waldenstrom macroglobulinemia, with CXCR4 mutations impacting VGPR attainment, time to major response, and 4-year PFS rate.
Article
Clinical Neurology
Jie Lin, Binbin Xue, Jia Li, Ruofan Zhu, Juyuan Pan, Zhibo Chen, Xu Zhang, Xiang Li, Junhui Xia
Summary: Long-term use of low-dose RTX and MMF showed efficacy and tolerability in Chinese patients with NMOSD. RTX was more effective in reducing ARR compared to MMF.
FRONTIERS IN NEUROLOGY
(2022)
Article
Clinical Neurology
Su-Hyun Kim, Hyunmin Jang, Na Young Park, Yeseul Kim, So-Yeon Kim, Min Young Lee, Jae-Won Hyun, Ho Jin Kim
Summary: Discontinuation of IST in NMOSD patients may lead to an increased risk of relapse, even after 5 years of remission. Caution is advised when considering IST discontinuation, especially in patients who have experienced severe attacks prior to IST.
NEUROLOGY-NEUROIMMUNOLOGY & NEUROINFLAMMATION
(2021)
Article
Clinical Neurology
Paula Barreras, Eleni S. Vasileiou, Angeliki G. Filippatou, Kathryn C. Fitzgerald, Michael Levy, Carlos A. Pardo, Scott D. Newsome, Ellen M. Mowry, Peter A. Calabresi, Elias S. Sotirchos
Summary: Rituximab treatment reduces the annualized relapse rate in AQP4-IgG-seropositive NMOSD, especially when administered consistently without gaps in treatment or B-cell reconstitution. In MOGAD patients, although a decrease in relapses was observed after rituximab initiation, this effect appeared to be less pronounced compared to AQP4-IgG-seropositive NMOSD. Severe infections and hypogammaglobulinemia were seen in a significant number of patients, underscoring the importance of close monitoring for infectious complications.
Article
Immunology
A. Razzaque Ahmed, Merve Aksoy
Summary: This study investigated the dynamics of IgM levels in patients with autoimmune mucocutaneous blistering diseases (AIMBD), finding a low incidence of decreased serum IgM levels. While decreased IgM did not affect clinical parameters or CD19+ B cell depletion, patients in the study still achieved clinical and serological remission without developing infections, tumors, or other autoimmune diseases. Studies on IgM deficiency in knockout mice provided valuable insights into the biological role of IgM in health and disease.
FRONTIERS IN IMMUNOLOGY
(2021)
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)
Article
Oncology
S. Morteza Seyed Jafari, Flavia Folini-Huesser, Simone Cazzaniga, Robert E. Hunger
Summary: The study aimed to investigate the long-term efficacy of imiquimod 5% cream for the management of lentigo maligna (LM), with a focus on disease recurrence and prognostic factors of disease-free survival (DFS). If surgical excision is not possible due to age/comorbidities or critical cosmetic localization, imiquimod could provide optimal outcomes with a low risk of relapse.
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.
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
Fereshteh Ashtari, Roshanak Mehdipour, Ahmad Eini, Arshia Ghalamkari
Summary: The purpose of this study is to understand the clinical characteristics and disease activity of NMOSD patients in the study. The results showed that NMOSD is more prevalent in the southwest region of Isfahan province, with the main features being transverse myelitis and optic neuritis, accompanied by brain stem syndromes. Through long-term follow-up and research, we can better understand the course of the disease and its treatment methods.
MULTIPLE SCLEROSIS AND RELATED DISORDERS
(2023)
Article
Clinical Neurology
Konrad Rejdak, Ewa Papuc
Summary: Cladribine treatment was safe and effective in patients with NMOSD over a 24-month period, resulting in a significant decrease in annualized relapse rate and no progression of disability status.
EUROPEAN JOURNAL OF NEUROLOGY
(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
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)
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
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
Clinical Neurology
Ana Klein, Martina Flaskamp, Achim Berthele, Friederike Held, Harisa Muratovic, Bernhard Hemmer
Summary: Background: Disease-modifying therapies (DMTs) in multiple sclerosis (MS) have varying effects on immune system and its relation to infectious complications. This study analyzed the effect of DMTs on serum immunoglobulin (Ig) levels, considering patient demographics and therapy duration. The results showed that except for interferon-beta (IFN) and glatiramer acetate (GA), DMTs were associated with a decrease in Ig levels and had differential effects on Ig subclasses. Monitoring of Ig levels should be considered in patients on long-term treatment with DMTs, particularly those on B-cell depleting therapies (BCDT), to identify patients at risk of low immunoglobulin levels.
THERAPEUTIC ADVANCES IN NEUROLOGICAL DISORDERS
(2023)
Article
Clinical Neurology
Alexander Hapfelmeier, Begum Irmak On, Mark Muehlau, Jan S. Kirschke, Achim Berthele, Christiane Gasperi, Ulrich Mansmann, Alexander Wuschek, Matthias Bussas, Martin Boeker, Antonios Bayas, Makbule Senel, Joachim Havla, Markus C. Kowarik, Klaus Kuhn, Ingrid Gatz, Helmut Spengler, Benedikt Wiestler, Lioba Grundl, Dominik Sepp, Bernhard Hemmer
Summary: The study aims to support treatment decision-making for patients with early RRMS and CIS by using an algorithm to create a treatment decision score (MS-TDS). By integrating multiple data sources, the MS-TDS predicts the probability of new or enlarging lesions in cerebral MRIs between 6 and 24 months after the initial MRI. The results show that the MS-TDS can estimate individualized treatment success probabilities and identify patients who benefit from early platform medication.
THERAPEUTIC ADVANCES IN NEUROLOGICAL DISORDERS
(2023)