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
Genetics & Heredity
Genaro Gabriel Ortiz, Blanca M. G. Torres-Mendoza, Javier Ramirez-Jirano, Jazmin Marquez-Pedroza, Jose J. Hernandez-Cruz, Mario A. Mireles-Ramirez, Erandis D. Torres-Sanchez
Summary: Demyelinating diseases affect the myelin or coating of nerve fibers in the central and peripheral nervous systems. Multiple sclerosis (MS) and neuromyelitis optica spectrum disorders (NMOSD) are two distinct disease categories with similar underlying mechanisms. By studying genetic factors, clinical manifestations, and lesion pathology, researchers have made progress in differential diagnosis and understanding disease pathogenesis.
Article
Clinical Neurology
Ren Wei, Xiaolu Xu, Yunyun Duan, Ningnannan Zhang, Jie Sun, Haiqing Li, Yuxin Li, Yongmei Li, Chun Zeng, Xuemei Han, Fuqing Zhou, Muhua Huang, Runzhi Li, Zhizheng Zhuo, Frederik Barkhof, James H. Cole, Yaou Liu
Summary: This study evaluated the clinical significance of deep learning-derived brain age prediction in NMOSD and RRMS. The results showed that both NMOSD and RRMS patients had a significantly higher brain age gap compared to healthy controls. The brain age gap was associated with baseline disability score, advanced brain volume loss, and disease duration. Additionally, the brain age gap predicted the worsening of disability status in both NMOSD and RRMS patients.
JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY
(2023)
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
Karolina Kania, Wojciech Ambrosius, Wojciech Kozubski, Alicja Kalinowska
Summary: Balo's concentric sclerosis (BCS) is a rare demyelinating disorder with a debated connection to classic multiple sclerosis. Our report presents a case of a patient who developed a symptomatic Balo-like lesion following years of treatment for relapsing-remitting multiple sclerosis with dimethyl fumarate.
FRONTIERS IN NEUROLOGY
(2022)
Article
Clinical Neurology
Juan Zhang, Mei-Jiao Chen, Gui-Xian Zhao, Hong-Fu Li, Lei Wu, Yong-Feng Xu, Yajin Liao, Zengqiang Yuan, Zhi-Ying Wu
Summary: The study explored the association between PRRC2A variants and susceptibility to NMOSD and MS in the Han Chinese population. Certain PRRC2A variants were found to alter susceptibility to AQP4(+)NMOSD and MS, with specific genotypes increasing the risk. Additionally, haplotypes and gene expression levels associated with these variants were identified, suggesting potential implications for personalized therapeutic approaches in these disease entities.
JOURNAL OF NEUROLOGY
(2021)
Article
Medicine, Research & Experimental
Anne Winkler, Claudia Wrzos, Michael Haberl, Marie-Theres Weil, Ming Gao, Wiebke Moebius, Francesca Odoardi, Dietmar R. Thal, Mayland Chang, Ghislain Opdenakker, Jeffrey L. Bennett, Stefan Nessler, Christine Stadelmann
Summary: In neuromyelitis optica spectrum disorder (NMOSD), polymorphonuclear leukocytes (PMNs) play a crucial role in disrupting the blood-brain barrier (BBB) and lesion development, rather than persistent loss of astrocytes. Their recruitment and activation could be promising therapeutic targets.
JOURNAL OF CLINICAL INVESTIGATION
(2021)
Review
Clinical Neurology
Trygve Holmoy, Rune Alexander Hoglund, Zsolt Illes, Kjell-Morten Myhr, Oivind Torkildsen
Summary: For NMOSD patients, monoclonal antibodies that deplete B cells or interfere with interleukin 6 signaling have superior efficacy compared to placebo; Rituximab, tocilizumab, and to some extent eculizumab have well-known safety profiles; Rituximab and azathioprine may be safe during pregnancy.
JOURNAL OF NEUROLOGY
(2021)
Article
Neurosciences
Eun Bin Cho, Se Young Jung, Jin-Hyung Jung, Yohwan Yeo, Hee Jin Kim, Kyungdo Han, Dong Wook Shin, Ju-Hong Min
Summary: A population-based study in South Korea found that patients with multiple sclerosis (MS) and neuromyelitis optica spectrum disorder (NMOSD) have an increased risk of developing dementia, with MS patients having a higher risk compared to NMOSD patients.
FRONTIERS IN NEUROSCIENCE
(2023)
Review
Cell Biology
Arshad Mehmood, Suleman Shah, Ruo-Yi Guo, Arsalan Haider, Mengya Shi, Hamid Ali, Ijaz Ali, Riaz Ullah, Bin Li
Summary: MECP2 and its product MeCP2 are associated with multiple sclerosis and neuromyelitis optica spectrum disorders. MeCP2 regulates gene expression in neurons, immune cells, and during development by modulating various mechanisms and pathways. A thorough understanding of MeCP2 function can provide new therapeutic strategies for these diseases.
CELLULAR AND MOLECULAR NEUROBIOLOGY
(2023)
Article
Clinical Neurology
Romina Mariano, Silvia Messina, Adriana Roca-Fernandez, Maria Leite, Yazhuo Kong, Jacqueline A. Palace
Summary: Spinal cord involvement is a key feature of multiple sclerosis, neuromyelitis optica with AQP4 antibodies, and MOG-antibody disease. Quantitative spinal cord MRI was used in a cross-sectional study to differentiate these conditions, showing significant damage in AQP4-antibody disease and multiple sclerosis, while MOG-antibody disease had a predominant central grey matter component. The study also found that relapsing MOG-antibody disease is a more severe phenotype and applied various analyses to discriminate between different diseases.
Article
Clinical Neurology
Brenda Banwell, Jeffrey L. Bennett, Romain Marignier, Ho Jin Kim, Fabienne Brilot, Eoin P. Flanagan, Sudarshini Ramanathan, Patrick Waters, Silvia Tenembaum, Jennifer S. Graves, Tanuja Chitnis, Alexander U. Brandt, Cheryl Hemingway, Rinze Neuteboom, Lekha Pandit, Markus Reindl, Albert Saiz, Douglas Kazutoshi Sato, Kevin Rostasy, Friedemann Paul, Sean J. Pittock, Kazuo Fujihara, Jacqueline Palace
Summary: Serum antibodies against MOG can help diagnose MOGAD, which is distinct from multiple sclerosis and neuromyelitis optica. The presence of MOG-IgG is a core criterion for the diagnosis. MOGAD can present with various symptoms and can be either monophasic or relapsing, and diagnostic accuracy relies on MOG-IgG cell-based assays.
Article
Clinical Neurology
Romain Marignier, Jeffrey L. Bennett, Ho Jin Kim, Brian G. Weinshenker, Sean J. Pittock, Dean Wingerchuk, Kazuko Fujihara, Friedemann Paul, Gary R. Cutter, Ari J. Green, Orhan Aktas, Hans-Peter Hartung, Fred D. Lublin, Ian M. Williams, Jorn Drappa, Dewei She, Daniel Cimbora, William Rees, Michael Smith, John N. Ratchford, Eliezer Katz, Bruce A. C. Cree
Summary: In the N-MOmentum trial, inebilizumab reduced the risk of 3-month disability progression compared to placebo in participants with NMOSD. Baseline subgroups did not affect the treatment effect observed with inebilizumab, and participants treated with inebilizumab were more likely to have a favorable outcome on the mRS.
NEUROLOGY-NEUROIMMUNOLOGY & NEUROINFLAMMATION
(2021)
Article
Multidisciplinary Sciences
Norman K. Gigengack, Frederike C. Oertel, Seyedamirhosein Motamedi, Charlotte Bereuter, Ankelien Duchow, Rebekka Rust, Judith Bellmann-Strobl, Klemens Ruprecht, Tanja Schmitz-Hubsch, Friedemann Paul, Alexander U. Brandt, Hanna G. Zimmermann
Summary: Optic neuritis in NMOSD leads to more profound vision loss compared to MS and MOGAD, and is associated with neuroaxonal and retinal damage.
SCIENTIFIC REPORTS
(2022)