keyword
https://read.qxmd.com/read/38449651/causal-relationship-between-the-immune-phenotype-of-monocytes-and-myasthenia-gravis-a-mendelian-randomization-study
#21
JOURNAL ARTICLE
Jing Dong, Rui-Sheng Duan, Peng Zhang
BACKGROUND: Monocytes play an essential role in developing autoimmune diseases; however, their association with myasthenia gravis (MG) development is unclear. METHODS: We performed a two-sample Mendelian randomization analysis to assess the causal relationship between monocyte-associated traits and MG, reviewing summary statistics of genome-wide association studies (GWAS). RESULTS: Using the inverse variance weighted method, the following were found to be causally associated with MG: HLA-DR on monocytes (OR, 1...
March 15, 2024: Heliyon
https://read.qxmd.com/read/38449066/small-extracellular-vesicle-micrornas-in-pediatric-myasthenia-gravis-plasma-and-skeletal-muscle
#22
JOURNAL ARTICLE
Mengying Zhu, Yilong Wang, Xuebin Xu, Xiaotong Guo, Yuchen Mao, Feng Gao
BACKGROUND: The diagnosis of myasthenia gravis (MG) in children remains difficult. Circulating small extracellular vesicle (sEV)-derived miRNAs (sEV-miRNAs) have been recognized as biomarkers of various diseases and can be excreted by different cell types. These biomarker candidates also play a vital role in autoimmune diseases via intercellular communication. METHODS: In the present study, we used sEV isolation and purification methods to extract the plasma-derived sEV-miRNAs from children with MG and healthy controls...
March 6, 2024: Postgraduate Medical Journal
https://read.qxmd.com/read/38448176/-diaphragmatic-dysfunction-post-covid-19-infection-report-of-two-cases
#23
REVIEW
D Yu, W Q Huang, Y Z Zhan, G H Xiao, J Li, W C Tong, S X Cai, L Y Liu
Following the global outbreak of COVID-19, many patients have suffered from multi-system complications and long-term sequelae caused by the virus. Diaphragm dysfunction is an obscure post-COVID-19 symptom. Although a few cases of diaphragm dysfunction caused by COVID-19 infection have been reported abroad, there are no relevant reports in China. Herein, we present two cases of patients with respiratory distress after COVID-19 infection. On admission, dynamic chest radiographs revealed diaphragm dysfunction in these patients...
March 12, 2024: Chinese Journal of Tuberculosis and Respiratory Diseases
https://read.qxmd.com/read/38446087/an-unusual-presentation-of-musk-myasthenia-gravis-with-bladder-incontinence-responding-to-rituximab-treatment-a-case-report-and-review-of-the-literature
#24
REVIEW
Abena Kwegyir-Aggrey, James B Meiling, Nicholas J Miller, Rachana K Gandhi Mehta
No abstract text is available yet for this article.
March 1, 2024: Journal of Clinical Neuromuscular Disease
https://read.qxmd.com/read/38444566/transient-neonatal-myasthenia-gravis-born-to-a-mother-with-asymptomatic-mg-a-case-report
#25
Jinrong Yang, Liping Pan, Yaping Liu, Yanrong Wang
Myasthenia gravis (MG) is an autoimmune disease which can impact pregnancy. We describe a transient neonatal myasthenia gravis (TNMG) born to an asymptomatic mother aged 26. The newborn presented cyanosis and generalized muscular weakness quickly after birth. Nasal continuous positive airway pressure (nCPAP) ventilation was performed immediately. On day 3, detailed family history showed that the neonate's 50-year-old maternal grandmother was diagnosed as ocular MG at the age of 40. Ryanodine receptor calcium release channel antibody (RyR-Ab) and acetylcholine receptor antibody (AChR-Ab) tested on day 5 were positive...
2024: Degenerative Neurological and Neuromuscular Disease
https://read.qxmd.com/read/38443765/respiratory-failure-as-first-presentation-of-myasthenia-gravis-a-case-report
#26
JOURNAL ARTICLE
Fangming Wang, Jinming Cheng, Xiaoli Niu, Litao Li
Myasthenia gravis (MG) is often complicated by respiratory failure, an exacerbation known as myasthenic crisis. However, most patients with MG develop respiratory symptoms during the late course of the disease. Respiratory failure as an exclusive initial and primary complaint in patients with MG is rare and seldom reported. We herein describe a woman in her late 50s who presented with respiratory failure and was diagnosed with obesity hypoventilation syndrome at a local hospital. Her condition gradually worsened during the next 4 months and became accompanied by dysphagia...
March 2024: Journal of International Medical Research
https://read.qxmd.com/read/38443029/congenital-myasthenia-syndrome-due-to-a-novel-dpagt1-gene-mutation-an-error-of-glycosylation-masquerading-as-a-congenital-myopathy
#27
JOURNAL ARTICLE
Aakash Mahesan, Gautam Kamila, Richa Tiwari, Sumanta Das, Mehar C Sharma, Prashant Jauhari, Biswaroop Chakrabarty, Sheffali Gulati
No abstract text is available yet for this article.
January 1, 2024: Neurology India
https://read.qxmd.com/read/38443018/myositis-in-h1n1-infection-compounds-to-myasthenic-crisis
#28
JOURNAL ARTICLE
Jayantee Kalita, Sarvesh K Chaudhary, Prakash C Pandey, Varun K Singh, Usha K Misra
Infection is an important trigger of myasthenic crisis (MC), and those infections manifest with pneumonia and muscle involvement may result in more frequent MC. We report two myasthenia gravis (MG) patients with H1N1 infection, and highlight the reasons for deterioration. Two patients with MG had H1N1 infection. The diagnosis of MG was confirmed by neostigmine, repetitive nerve stimulation, and anti-acetylcholine receptor antibody tests. H1N1 was confirmed by nucleic acid detection study, and myositis by creatinine kinase...
January 1, 2024: Neurology India
https://read.qxmd.com/read/38441928/-de-novo-hypercapnic-respiratory-failure-unmasking-neuromuscular-disorders-experiences-from-a-tertiary-care-center-and-review-of-literature
#29
REVIEW
Aditya Vijayakrishnan Nair, Madhavi Kandagaddala, Ajith Sivadasan, A T Prabhakar, Shalini Nair, Vivek Mathew, Sanjith Aaron, Mathew Alexander
OBJECTIVES: Neuromuscular disorders could have respiratory involvement early or late into illness. Rarely, patients may present with a hypercapnic respiratory failure (with minimal motor signs) unmasking an underlying disease. There are hardly any studies which have addressed the spectrum and challenges involved in management of this subset, especially in the real-world scenario. METHODS: A retrospective study comprising consecutive patients hospitalized with hypercapnic respiratory failure as the sole/dominant manifestation...
March 1, 2024: Journal of Clinical Neuromuscular Disease
https://read.qxmd.com/read/38437775/ptosis-secondary-to-orbital-metastasis-undetected-by-magnetic-resonance-imaging-a-case-report
#30
JOURNAL ARTICLE
V Chatzistergiou, A Bennedjai, A Morin, R Adam, J-P Nordmann
We report a patient with isolated ptosis secondary to orbital metastasis but no evidence of a neoplastic process on magnetic resonance imaging (MRI). A 69-year-old male was referred to our hospital with ptosis of the right upper eyelid and secondary frontalis muscle overaction on the left side for six months. The palpebral fissure was 3mm on the right and 16mm on the left, and levator function was 6mm and 19mm respectively. Three years previously, he had undergone surgical resection of the left lower lobe of the lung due to a T1 N0 M0 adenocarcinoma, and no recurrence had since been observed...
March 3, 2024: Journal Français D'ophtalmologie
https://read.qxmd.com/read/38436998/batoclimab-vs-placebo-for-generalized-myasthenia-gravis-a-randomized-clinical-trial
#31
JOURNAL ARTICLE
Chong Yan, Yaoxian Yue, Yuzhou Guan, Bitao Bu, Qing Ke, Ruisheng Duan, Hui Deng, Qun Xue, Haishan Jiang, Fei Xiao, Huan Yang, Ting Chang, Zhangyu Zou, Haifeng Li, Song Tan, Haibing Xiao, Hongyu Zhou, Hua Zhang, Qiang Meng, Wenyu Li, Wei Li, Junhong Guo, Yali Zhang, Zunbo Li, Jianglong Tu, Jianquan Shi, Wei Li, Michael Lee, Yu Chen, Xiaolu Tao, Shuai Zhao, Ping Li, Chongbo Zhao
IMPORTANCE: Myasthenia gravis (MG) is caused by autoantibodies that disrupt the neuromuscular junction. The neonatal fragment crystallizable receptor (FcRn) antagonists, efgartigimod and rozanolixizumab, reduce immunoglobulin G (IgG) level in the circulation and alleviate symptoms in patients with generalized MG. OBJECTIVE: To examine the efficacy and safety profile of batoclimab, a monoclonal IgG1 antibody, in patients with generalized MG. DESIGN, SETTING, AND PARTICIPANTS: This was a multicenter randomized clinical trial conducted from September 15, 2021, to June 29, 2022, at 27 centers in China...
March 4, 2024: JAMA Neurology
https://read.qxmd.com/read/38434198/epidemiology-of-myasthenia-gravis-in-the-united-states
#32
JOURNAL ARTICLE
Yun Ye, Dana Jorgensen Murdock, Chao Chen, Wolfgang Liedtke, Caitlin A Knox
INTRODUCTION: Global studies of epidemiology of myasthenia gravis (MG) have pointed to increasing prevalence of this rare autoimmune disorder affecting the neuromuscular synapse; however, no new data for the USA were available for decades. We aimed to estimate the incidence rate and prevalence of MG in a large-scale insured US population. METHODS: We conducted a population-based retrospective cohort study to estimate the annual incidence and prevalence of MG cases in the USA during 2017...
2024: Frontiers in Neurology
https://read.qxmd.com/read/38431900/the-efficacy-and-safety-of-fcrn-inhibitors-in-patients-with-myasthenia-gravis-a-systematic-review-and-meta-analysis
#33
REVIEW
Jiaxuan Li, Xin Wu, Tianchen Chu, Xin Tan, Shixin Wang, Ruisi Qu, Zhouqing Chen, Zhong Wang
BACKGROUND: Myasthenia gravis (MG) is an autoimmune disease that causes local or generalized muscle weakness. Complement inhibitors and targeting of the neonatal Fc receptor (FcRn) to block IgG cycling are two novel and successful mechanisms. METHODS: PubMed, EMBASE, the Cochrane Library, and ClinicalTrials.gov were systematically searched to identify relevant studies published before May 18, 2023. Review Manager 5.3 software was used to assess the data. RESULTS: We pooled 532 participants from six randomized controlled trials (RCTs)...
March 3, 2024: Journal of Neurology
https://read.qxmd.com/read/38430631/therapeutic-potential-of-natural-killer-cells-in-neuroimmunological-diseases
#34
REVIEW
Qing Zhang, Jing Lin, Mengge Yang, Zhijun Li, Min Zhang, Bitao Bu
Natural killer (NK) cells, a major component of the innate immune system, have prominent immunoregulatory, antitumor proliferation, and antiviral activities. NK cells act as a double-edged sword with therapeutic potential in neurological autoimmunity. Emerging evidence has identified NK cells are involved in the development and progression of neuroimmunological diseases such as multiple sclerosis, neuromyelitis optica spectrum disorders, autoimmune encephalitis, Guillain-Barré Syndrome, chronic inflammatory demyelinating polyneuropathy, myasthenia gravis, and idiopathic inflammatory myopathy...
March 1, 2024: Biomedicine & Pharmacotherapy
https://read.qxmd.com/read/38427273/constructing-and-validating-a-nomogram-model-for-short-term-prognosis-of-patients-with-achr-ab-gmg
#35
JOURNAL ARTICLE
Feng Liang, Zhaoxu Yin, Yaqian Li, Guanxi Li, Jing Ma, Huiqiu Zhang, Xiaoqian Xia, Make Yao, Xiaomin Pang, Juan Wang, Xueli Chang, Junhong Guo, Wei Zhang
OBJECTIVE: This study aimed to establish and validate a nomogram prognostic model for predicting short-term efficacy of acetylcholine receptor antibody-positive (AChR-Ab+) generalized myasthenia gravis (GMG). METHODS: A retrospective observational study was conducted at the First Hospital of Shanxi Medical University, enrolling patients diagnosed with AChR-Ab+ GMG from May 2020 to September 2022. The primary outcome was the change in the Myasthenia Gravis Foundation of America (MGFA) post-intervention status after 6 months of standard treatment...
March 1, 2024: Neurology and Therapy
https://read.qxmd.com/read/38423753/shrinking-lung-syndrome-mimicking-diaphragmatic-palsy-in-systemic-lupus-erythematosus
#36
JOURNAL ARTICLE
Shivam Mirg, Animesh Das, Awadh Kishor Pandit, M C Sharma, Achal K Srivastava
A 25-year-old woman presented with 1 year of progressive orthopnoea, initially explained as bilateral diaphragmatic paresis caused by seronegative myasthenia gravis. She required assisted ventilation and received pyridostigmine and corticosteroids. She had minimal (particularly proximal) symmetrical tetraparesis with apparent bilateral diaphragmatic weakness, but had normal sensation. Further investigation suggested an overlap myositis with shrinking lung syndrome from systemic lupus erythematosus. She improved following immunosuppression with pulse corticosteroids and rituximab, and at 3 months no longer needed bilevel positive airway pressure support...
February 29, 2024: Practical Neurology
https://read.qxmd.com/read/38423165/neurometabolic-and-brain-functional-alterations-associated-with-cognitive-impairment-in-patients-with-myasthenia-gravis-a-combined-1-h-mrs-and-fmri-study
#37
JOURNAL ARTICLE
Xiaoling Zhou, Yang Yang, Feng Zhu, Xiang Chen, Yunfei Zhu, Tiantian Gui, Yonggang Li, Qun Xue
Whether patients with myasthenia gravis (MG) exhibit cognitive impairment is controversial. Also the underlying mechanisms are unknown. We aimed to investigate alterations in cognitive function, neurometabolite levels, and brain function in patients with MG and to explore the associations between abnormal regional brain functional activity, neurometabolite concentrations in the MPFC and left thalamus, and cognitive activity in patients with MG. Neuropsychological tests, proton magnetic resonance spectroscopy, and resting-state functional magnetic resonance imaging were performed on 41 patients with MG and 45 race-, sex-, age-, and education-matched healthy controls (HCs)...
February 27, 2024: Neuroscience
https://read.qxmd.com/read/38422742/the-investigation-of-killer-cell-immunoglobulin-like-receptors-kirs-and-their-hla-ligands-in-iranian-patients-with-myasthenia-gravis
#38
JOURNAL ARTICLE
Mobina Jalalvand, Fereshteh Beigmohammadi, Samaneh Soltani, Soroush Ehsan, Sahebeh Rajabkhah, Elham Madreseh, Maryam Akhtari, Ahmadreza Jamshidi, Elham Farhadi, Mahdi Mahmoudi, Shahriar Nafissi
BACKGROUND: Myasthenia gravis (MG) is a disabling disease with the underlying pathophysiology of auto-antibodies attacking the postsynaptic acetylcholine receptors of neuromuscular junctions causing muscle weakness. Natural killer (NK) cells are innate immune cells that play an important regulative role in immune responses. The human killer-cell immunoglobulin-like receptors (KIRs) family is one of the receptors on NK cells that can either activate or inhibit NK cells. This study aimed to assess the possible role of KIR and their human leukocyte antigen (HLA) ligand genes susceptibility to MG in Iranian patients...
February 13, 2024: Clinical Neurology and Neurosurgery
https://read.qxmd.com/read/38421513/interactions-between-extracellular-vesicles-and-autophagy-in-neuroimmune-disorders
#39
REVIEW
Xiwen Ai, Haojun Yu, Yu Cai, Yangtai Guan
Neuroimmune disorders, such as multiple sclerosis, neuromyelitis optica spectrum disorder, myasthenia gravis, and Guillain-Barré syndrome, are characterized by the dysfunction of both the immune system and the nervous system. Increasing evidence suggests that extracellular vesicles and autophagy are closely associated with the pathogenesis of these disorders. In this review, we summarize the current understanding of the interactions between extracellular vesicles and autophagy in neuroimmune disorders and discuss their potential diagnostic and therapeutic applications...
February 29, 2024: Neuroscience Bulletin
https://read.qxmd.com/read/38421419/burden-of-disease-in-lambert-eaton-myasthenic-syndrome-taking-the-patient-s-perspective
#40
JOURNAL ARTICLE
Sophie Lehnerer, Meret Herdick, Regina Stegherr, Lea Gerischer, Frauke Stascheit, Maike Stein, Philipp Mergenthaler, Sarah Hoffmann, Andreas Meisel
BACKGROUND: Lambert-Eaton myasthenic syndrome (LEMS) is an autoimmune-mediated neuromuscular disorder leading to muscle weakness, autonomic dysregulation and hyporeflexia. Psychosocial well-being is affected. Previously, we assessed burden of disease for Myasthenia gravis (MG). Here, we aim to elucidate burden of disease by comparing health-related quality of life (HRQoL) of patients with LEMS to the general population (genP) as well as MG patients. METHODS: A questionnaire-based survey included sociodemographic and clinical data along with standardized questionnaires, e...
February 29, 2024: Journal of Neurology
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