Review
Medicine, General & Internal
Rossen T. Rousseff
Summary: The diagnosis of autoimmune Myasthenia Gravis relies on clinical history and physical examination, with additional tests and laboratory methods used to confirm the diagnosis and assess treatment effectiveness. Various tests are recommended for the diagnosis and follow-up of MG.
JOURNAL OF CLINICAL MEDICINE
(2021)
Article
Clinical Neurology
Zheng Yu, Meiying Zhang, Hongyang Jing, Peng Chen, Rangjuan Cao, Jinxiu Pan, Bin Luo, Yue Yu, Brandy M. Quarles, Wencheng Xiong, Michael H. Rivner, Lin Mei
Summary: The study aimed to investigate the pathogenic role of human anti-LRP4/agrin antibodies in mice and found that the antibodies led to MG symptoms in the mice. The antibodies impaired neuromuscular junction function and disrupted agrin-dependent LRP4-MuSK interaction, providing insights into the pathogenic mechanisms of these antibodies.
Article
Clinical Neurology
Zheng Yu, Meiying Zhang, Hongyang Jing, Peng Chen, Rangjuan Cao, Jinxiu Pan, Bin Luo, Yue Yu, Brandy M. Quarles, Wencheng Xiong, Michael H. Rivner, Lin Mei
Summary: The study found that antibodies against LRP4/agrin from a patient with MG can induce MG symptoms in mice, including weight loss and muscle weakness. These antibodies impair the neuromuscular junction by interfering with agrin-dependent LRP4-MuSK interaction, leading to compromised neuromuscular transmission and fragmentation of the neuromuscular junction.
Review
Clinical Neurology
Gianvito Masi, Kevin C. O'Connor
Summary: Recent research has provided a deeper understanding of the mechanisms underlying autoimmune myasthenia gravis, particularly in different disease subtypes. Additionally, a wider spectrum of treatment options has brought more benefits to patients. Furthermore, clinical observations have revealed new MG phenotypes, but the underlying immunobiology remains unclear.
CURRENT OPINION IN NEUROLOGY
(2022)
Article
Immunology
Jose A. Villegas, Jerome Van Wassenhove, Judith Merrheim, Karen Matta, Samy Hamadache, Clemence Flaugere, Pauline Pothin, Frederique Truffault, Sebastien Hascoet, Nicola Santelmo, Marco Alifano, Sonia Berrih-Aknin, Rozen le Panse, Nadine Dragin
Summary: Acetylcholine receptor (AChR) myasthenia gravis (MG) is a chronic autoimmune disease characterized by muscle weakness. The inflammatory IL-23/Th17 pathway in the thymus, blood and muscle plays a role in the disease's progression. This study investigated the potential therapeutic effects of targeting IL-23 in two preclinical MG models and found that it improved clinical symptoms and reduced inflammation and antibody production.
JOURNAL OF NEUROINFLAMMATION
(2023)
Article
Geriatrics & Gerontology
Li-Kai Tsai, I-Shin Chen, Chi-Chao Chao, Hsueh-Wen Hsueh, Hsin-Hsiung Chen, Yun-Hsin Huang, Rong-Wei Weng, Tzu-Yun Lai, Yi-Chieh Tsai, Yeou-Ping Tsao, Show-Li Chen
Summary: The study revealed the important role of NRIP in the neuromuscular junction, showing its co-localization and interaction with AChR-associated proteins. Anti-NRIP autoantibody was found in patients with myasthenia gravis, potentially exacerbating disease severity.
JOURNAL OF CACHEXIA SARCOPENIA AND MUSCLE
(2021)
Review
Cell Biology
Francisco J. Barrantes
Summary: This review discusses the autoimmune aggression in myasthenia gravis, focusing on the destruction of the neuromuscular junction. The use of superresolution optical microscopy to study the dynamics of nicotinic receptors in live cells is also highlighted.
NEURAL REGENERATION RESEARCH
(2021)
Article
Immunology
Yu Cai, Lu Han, Desheng Zhu, Jing Peng, Jianping Li, Jie Ding, Jiaying Luo, Ronghua Hong, Kan Wang, Wenbin Wan, Chong Xie, Xiajun Zhou, Ying Zhang, Yong Hao, Yangtai Guan
Summary: By establishing a stable cell line expressing clustered AChR, we achieved highly sensitive detection of AChR antibodies in MG patients and demonstrated its advantageous clinical application in CBAs.
FRONTIERS IN IMMUNOLOGY
(2021)
Review
Neurosciences
Claudia Vinciguerra, Liliana Bevilacqua, Antonino Lupica, Federica Ginanneschi, Giuseppe Piscosquito, Nicasio Rini, Alessandro Rossi, Paolo Barone, Filippo Brighina, Vincenzo Di Stefano
Summary: This narrative review examines the latest updates on seronegative myasthenia gravis (SnMG), defining the clinical characteristics of affected patients, diagnostic methods, management, and therapeutic scenarios.
Review
Clinical Neurology
Jieni Zhang, Yin Chen, Jiaxin Chen, Xin Huang, Haiyan Wang, Yan Li, Weibin Liu, Huiyu Feng
Summary: The study found that DSP-MG in southern China has more severe symptoms and poor prognosis compared to AChR-MG, with no significant differences compared to MuSK-MG. The researchers speculated that DSP-MG may be a subtype of MuSK-MG.
NEUROLOGICAL SCIENCES
(2021)
Article
Cell Biology
Virginia M. Smith, Huan Nguyen, John W. Rumsey, Christopher J. Long, Michael L. Shuler, James J. Hickman
Summary: The study established a MG model that mimics disease pathology by inducing reduced NMJ stability and complement activation through antibody treatment, affecting neuromuscular transmission. This research provides a new experimental model for the disease mechanisms of MG and simultaneously simulates clinical manifestations.
FRONTIERS IN CELL AND DEVELOPMENTAL BIOLOGY
(2021)
Article
Neurosciences
Shuuichi Mori, Shigeaki Suzuki, Tetsuro Konishi, Naoki Kawaguchi, Masahiko Kishi, Satoshi Kuwabara, Kei Ishizuchi, Heying Zhou, Futoshi Shibasaki, Hiroki Tsumoto, Takuya Omura, Yuri Miura, Seijiro Mori, Mana Higashihara, Shigeo Murayama, Kazuhiro Shigemoto
Summary: Autoantibodies to muscle-specific tyrosine kinase (MuSK) proteins at the neuromuscular junction (NMJ) cause refractory generalized myasthenia gravis (MG) with dyspnea more frequently than other MG subtypes. The secretion of MuSK proteins from muscles into the bloodstream is induced by ectodomain shedding triggered by neuro-muscular junction failure. MuSK may be used as a biomarker for other neuromuscular diseases in preclinical studies, clinical diagnostics, therapeutics, and drug discovery.
EXPERIMENTAL NEUROLOGY
(2023)
Review
Pediatrics
XiuShan Ge, CuiJie Wei, Hui Dong, YueHua Zhang, XinHua Bao, Ye Wu, DanYu Song, HongJun Hao, Hui Xiong
Summary: Myasthenia gravis is an autoimmune disease associated with acetylcholine receptor (AChR) and muscle-specific receptor tyrosine kinase (MuSK) antibodies. This case report highlights the rare coexistence of both antibodies in a patient with myasthenia gravis, and the successful use of rituximab therapy after conventional treatment failed.
FRONTIERS IN PEDIATRICS
(2022)
Article
Medicine, General & Internal
Caterina Maria Gambino, Luisa Agnello, Bruna Lo Sasso, Concetta Scazzone, Rosaria Vincenza Giglio, Giuseppina Candore, Anna Maria Ciaccio, Vincenzo Di Stefano, Filippo Brighina, Matteo Vidali, Marcello Ciaccio
Summary: The study compared the BIOCHIP mosaic with conventional ELISA in the diagnosis of MG in 71 patients, showing a concordance of 74% for anti-AChR reactivity and 84% for anti-MuSK reactivity. However, overall concordance among assays is not optimal.
Article
Biochemical Research Methods
Jaap J. Plomp, Maartje G. M. Huijbers, Jan J. G. M. Verschuuren, Anna Borodovsky
Summary: The study developed a new bioassay for studying complement-mediated synaptic damage at NMJs in patients with myasthenia gravis, allowing for independent investigation of this mechanism and optimizing care for individual patients.
JOURNAL OF NEUROSCIENCE METHODS
(2022)
