Robotic-assisted thoracoscopy thymectomy for juvenile myasthenia gravis
Publication date: Available online 30 July 2020Source: Journal of Pediatric Surgery Case ReportsAuthor(s): F. Grasso, L. De Leonibus, M. Bertozzi, M. Sica, R. Angotti, L. Luzzi, F. Molinaro, M. Messina, P. Paladini (Source: Journal of Pediatric Surgery Case Reports)
Source: Journal of Pediatric Surgery Case Reports - July 30, 2020 Category: Surgery Source Type: research

Inebilizumab: First Approval
This article summarizes the milestones in the development of inebilizumab leading to this first approval for the treatment of AQP4-IgG seropositive NMOSD. (Source: Drugs)
Source: Drugs - July 29, 2020 Category: Drugs & Pharmacology Source Type: research

Clinical and Molecular Spectrum of Myotonia and Periodic Paralyses Associated With Mutations in SCN4A in a Large Cohort of Italian Patients
Background: Four main clinical phenotypes have been traditionally described in patients mutated in SCN4A, including sodium-channel myotonia (SCM), paramyotonia congenita (PMC), Hypokaliemic type II (HypoPP2), and Hyperkaliemic/Normokaliemic periodic paralysis (HyperPP/NormoPP); in addition, rare phenotypes associated with mutations in SCN4A are congenital myasthenic syndrome and congenital myopathy. However, only scarce data have been reported in literature on large patient cohorts including phenotypes characterized by myotonia and episodes of paralysis.Methods: We retrospectively investigated clinical and molecular featur...
Source: Frontiers in Neurology - July 29, 2020 Category: Neurology Source Type: research

Myasthenia Gravis and Physical Exercise: A Novel Paradigm
The benefits of physical exercise for healthy individuals are well-established, particularly in relation to reducing the risks of chronic lifestyle related diseases. Furthermore, physical exercise has been seen to provide beneficial effects in many chronic diseases such as multiple sclerosis, rheumatoid arthritis, and chronic obstructive pulmonary disease and is therefore recommended as part of the treatment regimen. Myasthenia Gravis (MG) is a chronic autoimmune disease that causes neuromuscular transmission failure resulting in abnormal fatigable skeletal muscle weakness. In spite of this fluctuating skeletal muscle weak...
Source: Frontiers in Neurology - July 29, 2020 Category: Neurology Source Type: research

Myasthenia gravis therapy with individualized homeopathy: A case report
We present a 61 ‐year‐old man with severe myasthenia gravis, nonresponsive to conventional therapy. The patient was treated with individualized homeopathy, demonstrating significant improvement on his clinical status and no disease symptoms. AbstractWe present a 61 ‐year‐old man with severe myasthenia gravis, nonresponsive to conventional therapy. The patient was treated with individualized homeopathy, demonstrating significant improvement on his clinical status and no disease symptoms. (Source: Clinical Case Reports)
Source: Clinical Case Reports - July 29, 2020 Category: General Medicine Authors: Vitalie V ăcăraș, Cristina Nistor, Imelda Rahovan, Cristiana Văcăraş, George Vithoulkas Tags: CASE REPORT Source Type: research

Single-cell approaches to investigate B cells and antibodies in autoimmune neurological disorders.
Abstract Autoimmune neurological disorders, including neuromyelitis optica spectrum disorder, anti-N-methyl-D-aspartate receptor encephalitis, anti-MOG antibody-associated disorders, and myasthenia gravis, are clearly defined by the presence of autoantibodies against neurological antigens. Although these autoantibodies have been heavily studied for their biological activities, given the heterogeneity of polyclonal patient samples, the characteristics of a single antibody cannot be definitively assigned. This review details the findings of polyclonal serum and CSF studies and then explores the advances made by sing...
Source: Cellular and Molecular Immunology - July 29, 2020 Category: Molecular Biology Authors: Zou A, Ramanathan S, Dale RC, Brilot F Tags: Cell Mol Immunol Source Type: research

Myasthenia Gravis Masquerading as an Idiopathic Unilateral Facial Paralysis (Bell's Palsy) —A Very Rare and Unique Clinical Find
Myasthenia gravis (MG) is an uncommon autoimmune neuromuscular junction disorder manifesting as fluctuating weakness of skeletal muscles. To add to its repertoire of mimicking a wide range of neurological disorders, the present case report is, to the best of our knowledge, the very first to describe MG masquerading as an idiopathic unilateral facial paralysis (Bell's palsy, BP). Our case report is distinct, highlights a novel clinical occurrence, offers new insights of how different neurological disorders may overlap with each other, and reminds neurologists to have a very broad and thorough comprehension for effective dia...
Source: Frontiers in Neurology - July 28, 2020 Category: Neurology Source Type: research

Pharmacological strategy for congenital myasthenic syndrome with CHRNE mutations: a meta-analysis of case reports.
CONCLUSIONS: This meta-analysis provides evidence that (1) β2-adrenergic receptor agonist therapy could be the first choice of pharmacological strategy for treating CMS with CHRNE mutations; (2) a single-drug-regime, rather than a combination therapy, should be the first choice of treatment; and (3) it is never too late to initiate pharmacological treatment. PMID: 32727330 [PubMed - as supplied by publisher] (Source: Current Neuropharmacology)
Source: Current Neuropharmacology - July 28, 2020 Category: Drugs & Pharmacology Authors: Huang K, Luo YB, Bi FF, Yang H Tags: Curr Neuropharmacol Source Type: research

Diagnosing myasthenic crisis in SARS-CoV-2 infected patients requires adherence to appropriate criteria
With interest we read the article by Delly et al. about a 56  years old female with myasthenia gravis (MG) since 5y for which she received pyridostigmine (240 mg/d), prednisone (40 mg/d), and intravenous immunoglobulins (IVIG, 650 mg/kg within 2d every 2 weeks), and with undetermined mixed connective tissue disease for which she received chloroquine (400 mg/d), who developed myasthenic crisis with respiratory insufficiency during pneumonia due to infection with SARS-CoV-2 [1]. Though the patient received vancomycin, cefepime, and azithromycin and prednisone was increased to 80 mg/d, she lastly had to be intub...
Source: Journal of the Neurological Sciences - July 27, 2020 Category: Neurology Authors: Josef Finsterer, Fulvio A. Scorza, Carla A. Scorza Tags: Letter to the Editor Source Type: research

Reply from the authors: Myasthenic crises in COVID-19
Search Term: Viral Infection, Myasthenia Gravis, Myasthenic Crises, COVID-19. (Source: Journal of the Neurological Sciences)
Source: Journal of the Neurological Sciences - July 27, 2020 Category: Neurology Authors: Maryam J. Syed, Robert P. Lisak, Fadi Delly, Deepti Zutshi Tags: Response to letter to the Editor Source Type: research

[Drug exposure associated with exacerbation of symptoms in patients with myasthenia gravis].
CONCLUSIONS: Our patients were extensive and widespread exposed to drugs during the follow-up period but we did not find and association with severe exacerbation episodes. Just over half of the patients had at least one exacerbation episode during the study period, most of them were mild. Further studies with larger sample sizes are necessary to corroborate these conclusions and to study possible correlations between the use of drugs and the risk of exacerbation episodes. PMID: 32700310 [PubMed - in process] (Source: Revista de Neurologia)
Source: Revista de Neurologia - July 25, 2020 Category: Neurology Authors: Such-Díaz A, Díaz-Marín C, Sánchez-Pérez R, Iglesias-Peinado I Tags: Rev Neurol Source Type: research

Takotsubo cardiomyopathy in the setting of a myasthenic crisis
. (Source: International Journal of Neuroscience)
Source: International Journal of Neuroscience - July 24, 2020 Category: Neuroscience Authors: Adam Ranellone Michael G. Abraham Source Type: research

Early and long-term effect of the treatment with pyridostigmine in patients with gmppb-related cms
Congenital myasthenic syndromes (CMS) consist of a heterogeneous group of disorders due to mutations in genes involved in the signal transmission of the neuromuscular junction [1,2]. Different genes encoding proteins involved in glycosylation have been identified causing CMSs [3,4]. GMPPB is one of them, since it encodes the enzyme GDP-mannose pyrophosphorylase B that catalyses the conversion of mannose-1-phosphate and GTP to GDP-mannose [4], essential for the glycosylation of key proteins involved in the neuromuscular junction development and function. (Source: Neuromuscular Disorders)
Source: Neuromuscular Disorders - July 24, 2020 Category: Neurology Authors: Edna Julieth Bobadilla-Quesada, Daniel Natera-de Benito, Laura Carrera-Garc ía, Carlos Ortez, Jessica Exposito-Escudero, Cecilia Jimenez-Mallebrera, Cristina Jou, Anna Codina, Joan Corbera, Obdulia Moya, Veronica Saez, Lidia Gonzalez-Quereda, Pia Gallano Source Type: research

Management of Juvenile Myasthenia Gravis
Juvenile Myasthenia Gravis (JMG) is a rare disorder, defined as myasthenia gravis in children younger than 18 years of age. While clinical phenotypes are similar to adults, there are a number of caveats that influence management: broader differential diagnoses; higher rates of spontaneous remission; and the need to initiate appropriate treatment early, to avoid the long-term physical and psychosocial morbidity. Current practice is taken from treatment guidelines for adult MG or individual experience, with considerable variability seen across centers. We discuss our approach to treating JMG, in a large specialist JMG servic...
Source: Frontiers in Neurology - July 24, 2020 Category: Neurology Source Type: research

Serum level of soluble urokinase plasminogen activator receptor (suPAR) as a disease severity marker of myasthenia gravis: a pilot study.
In conclusion, serum suPAR levels can be a candidate for a novel biomarker of disease activity in anti-acetylcholine receptor antibody-positive MG. PMID: 32706905 [PubMed - as supplied by publisher] (Source: Clinical and Developmental Immunology)
Source: Clinical and Developmental Immunology - July 24, 2020 Category: Allergy & Immunology Authors: Uzawa A, Kojima Y, Ozawa Y, Yasuda M, Onishi Y, Akamine H, Kawaguchi N, Himuro K, Kuwabara S Tags: Clin Exp Immunol Source Type: research

MuSK-Associated Myasthenia Gravis: Clinical Features and Management
Muscle-specific tyrosine kinase (MuSK) myasthenia gravis (MG) is a rare, frequently more severe, subtype of MG with different pathogenesis, and peculiar clinical features. The prevalence varies among countries and ethnic groups, affecting 5–8% of all MG patients. MuSK-MG usually has an acute onset affecting mainly the facial-bulbar muscles. The symptoms usually progress rapidly, within a few weeks. Early respiratory crises are frequent. The disease may lead to generalized muscle weakness up to muscle atrophy. The main bulbar involvement, the absence of significant thymus alterations, and the association with HLA clas...
Source: Frontiers in Neurology - July 23, 2020 Category: Neurology Source Type: research

De novo thymic carcinoma or malignant transformation: a myasthenic patient presented with multiple mediastinal tumours
Synchronous triple thymic carcinomas and thymoma have not been reported. The finding of this case report supports the hypothesis of malignant transformation of thymoma to thymic carcinoma. AbstractA 63 ‐year‐old man presented with bilateral ptosis, and detailed evaluation confirmed ocular myasthenia gravis with three anterior mediastinal masses on computed tomography (CT) of the chest. Extended thymectomy was performed, and pathology revealed two thymic carcinoma and one thymoma. After surgery , the patient is free from recurrence. Synchronous triple thymic carcinomas and thymoma have not been reported. The finding of ...
Source: Respirology Case Reports - July 23, 2020 Category: Respiratory Medicine Authors: Chu ‐Pin Pai, Chih‐Ming Lin, Yi‐Chen Yeh, Chien‐Sheng Huang, Biing‐Shiun Huang Tags: Case Report Source Type: research

Slow-binding inhibitors of acetylcholinesterase of medical interest.
Abstract Certain ligands slowly bind to acetylcholinesterase. As a result, there is a slow establishment of enzyme-inhibitor equilibrium characterized by a slow onset of inhibition prior reaching steady state. Three mechanisms account for slow-binding inhibition: a) slow binding rate constant kon, b) slow ligand induced-fit following a fast binding step, c) slow conformational selection of an enzyme form. The slow equilibrium may be followed by a chemical step. This later that can be irreversible has been observed with certain alkylating agents and substrate transition state analogs. Slow-binding inhibitors presen...
Source: Neuropharmacology - July 23, 2020 Category: Drugs & Pharmacology Authors: Lushchekina SV, Masson P Tags: Neuropharmacology Source Type: research

Description of 3 patients with myasthenia gravis and COVID-19
The COVID-19 pandemic presents two main concerns for patients with myasthenia gravis (MG); chronic immunosuppression may put them at greater risk, and some proposed treatments for COVID-19 could cause MG exacerbation. (Source: Journal of the Neurological Sciences)
Source: Journal of the Neurological Sciences - July 22, 2020 Category: Neurology Authors: Netaniel Rein, Nitzan Haham, Efrat Orenbuch-Harroch, Marc Romain, Zohar Argov, Adi Vaknin-Dembinsky, Marc Gotkine Tags: Clinical short communication Source Type: research

Snake three-finger α-neurotoxins and nicotinic acetylcholine receptors: molecules, mechanisms and medicine.
Snake three-finger α-neurotoxins and nicotinic acetylcholine receptors: molecules, mechanisms and medicine. Biochem Pharmacol. 2020 Jul 22;:114168 Authors: Nirthanan S Abstract Snake venom three-finger α-neurotoxins (α-3FNTx) act on postsynaptic nicotinic acetylcholine receptors (nAChRs) at the neuromuscular junction (NMJ) to produce skeletal muscle paralysis. The discovery of the archetypal α-bungarotoxin (α-BgTx), almost six decades ago, exponentially expanded our knowledge of membrane receptors and ion channels. This included the localisation, isolation and characteriz...
Source: Biochemical Pharmacology - July 22, 2020 Category: Drugs & Pharmacology Authors: Nirthanan S Tags: Biochem Pharmacol Source Type: research

Congenital Myasthenic Syndromes
Congenital myasthenic syndromes comprise a rare heterogeneous group of diseases that impair neuromuscular transmission and are characterized by muscle fatigability and transient or permanent weakness. Symptoms are often present from birth or early childhood. These syndromes have a wide range of phenotypes and severity. Caused by genetic mutations in any of the numerous genes encoding for components of the neuromuscular junction. They are classified by where in the neuromuscular junction the mutated component is located: presynaptic, synaptic, or postsynaptic. Mutations in about 30 genes have been implicated. Diagnosis can ...
Source: Neurologic Clinics - July 21, 2020 Category: Neurology Authors: Stanley Jones P. Iyadurai Source Type: research

Genes, Vol. 11, Pages 821: A Family Case of Congenital Myasthenic Syndrome-22 Induced by Different Combinations of Molecular Causes in Siblings
In this study, we describe a unique case, in which two siblings with a rare disorder have different pathologic genotypes. (Source: Genes)
Source: Genes - July 19, 2020 Category: Genetics & Stem Cells Authors: Olga Shchagina Ludmila Bessonova Igor Bychkov Tatiana Beskorovainaya Aleksander Poliakov Tags: Brief Report Source Type: research

Idiopathic Orbital Inflammation Appearing on the Affected Side of Preceding Myasthenia Gravis.
Authors: Fujita S, Furuta N, Maruyama T, Tsukagoshi S, Nagashima K, Fujita Y, Nagai K, Kashima T, Tanaka M, Miki I, Yamazaki A, Ikota H, Oyama T, Ikeda Y Abstract The patient was a 70-year-old man with idiopathic orbital inflammation (IOI) that appeared on the severely affected side of preceding myasthenia gravis (MG). The patient was diagnosed with MG 5 years prior to the onset of IOI. When IOI was diagnosed, an edrophonium test was negative. IOI was considered because he complained of left orbital pain, eyelid swelling, and cerebral MRI exhibited the enhanced lesions along the left orbital periosteum. A biopsy sp...
Source: Internal Medicine - July 18, 2020 Category: Internal Medicine Tags: Intern Med Source Type: research

Autoimmune disease-related primary CNS lymphoma: systematic review and meta-analysis
ConclusionsAD requiring immunosuppression appear over-represented in the population of PCNSL patients. Aggressive polychemotherapy can accomplish long term OS in AD-PCNSL comparable to immunocompetent patients. Age  >  60 may serve as a prognostic factor. (Source: Journal of Neuro-Oncology)
Source: Journal of Neuro-Oncology - July 18, 2020 Category: Cancer & Oncology Source Type: research

GSE154573 Lack of desmin in mice causes structural and functional disorders of neuromuscular junctions
Contributors : Christian T Thiel ; Arif B Ekici ; Steffen UebeSeries Type : Expression profiling by high throughput sequencingOrganism : Mus musculusDesmin, the major intermediate filament (IF) protein in muscle cells, interlinks neighboring myofibrils and connects the whole myofibrillar apparatus to myonuclei, mitochondria, and the sarcolemma. However, desmin is also known to be enriched at postsynaptic membranes of neuromuscular junctions (NMJs). The pivotal role of the desmin IF cytoskeletal network is underscored by the fact that over 100 mutations of the human DES gene cause hereditary and sporadic myopathies and card...
Source: GEO: Gene Expression Omnibus - July 17, 2020 Category: Genetics & Stem Cells Tags: Expression profiling by high throughput sequencing Mus musculus Source Type: research

A New and Easily Used Modified Myasthenia Gravis Score
Myasthenia gravis (MG) is a common postsynaptic neuromuscular junction disease in which fluctuating muscular fatigability is the clinical hallmark. The muscles of the eyelids, limbs, pharynx, and respiration are commonly involved in sequence corresponding to the disease severity. The prevalence of MG ranged from 2 to7 per 10,000 persons, and it was more prevalent in females. [1] Since MG is a chronic neuromuscular weakness with fluctuating severity, a validated and reliable tool to quantify the severity of muscle weakness at different timing during a patient's course of illness is required. (Source: Neuromuscular Disorders)
Source: Neuromuscular Disorders - July 17, 2020 Category: Neurology Authors: Pornchai Sathirapanya, Katathep Wiputhanuphongs, Tippawan Liabsuetrakul, Pichamon Khanittanuphong, Warangkana Keeratichananont Source Type: research

Immune Checkpoint Inhibitor-Induced Myasthenia Gravis
The development of immune checkpoint inhibitors (ICIs) has been a major breakthrough in cancer immunotherapy. The increasing use of ICIs has led to the discovery of a broad spectrum of immune-related adverse events (irAEs). Immune-related myasthenia gravis (irMG) is a rare but life-threatening irAE. In this review, the clinical presentations of irMG are described and the risk of irMG-related mortality is examined using information from relevant studies. In 47 reported cases of irMG with clear causes of mortality, irMG appeared to be a distinct category of neuromuscular disorders and differed from classical MG in terms of i...
Source: Frontiers in Neurology - July 16, 2020 Category: Neurology Source Type: research

The immunosuppressant fingolimod ameliorates experimental autoimmune myasthenia gravis by regulating T-cell balance and cytokine secretion.
In conclusion, our results suggest that fingolimod has a preventive effect on experimental autoimmune myasthenia gravis by interfering with lymphocyte function. PMID: 32655793 [PubMed] (Source: American Journal of Translational Research)
Source: American Journal of Translational Research - July 15, 2020 Category: Research Tags: Am J Transl Res Source Type: research

Pembrolizumab-Induced Myasthenic Crisis with HyperCKemia in a Patient with Thymoma.
Authors: Shin JH, Choi YJ, Lee J, Baek SH PMID: 32657073 [PubMed] (Source: Journal of Clinical Neurology)
Source: Journal of Clinical Neurology - July 15, 2020 Category: Neurology Tags: J Clin Neurol Source Type: research

Myasthenia Gravis Treatment Updates
AbstractPurpose of reviewThis review presents an update of current treatment strategies for patients with myasthenia gravis (MG) depending on their clinical and immunological characteristics.Recent findingsNowadays, the available treatment options for MG are: (1) inhibitors of the acetylcholinesterase, useful as symptomatic treatment; (2) immunosuppressive drugs, the main treatment, initiated and escalated following international guidelines; (3) immunomodulatory drugs (intravenous immunoglobulin and plasma exchange), predominantly used in acute worsening or MG crisis; and (4) thymectomy, performed in patients with thymoma,...
Source: Current Treatment Options in Neurology - July 15, 2020 Category: Neurology Source Type: research

Plus-minus lid syndrome with ataxia and severe apathy & #8212;A rare manifestation of midbrain infarct
S Sheetal, R Thomas, AS Kumar, LG TomyJournal of Postgraduate Medicine 2020 66(3):162-164 Plus-minus lid syndrome is a rare manifestation of midbrain infarct, characterized by ptosis of one eye and lid retraction in the other eye. It has also been described in ocular myasthenia gravis, orbital myositis, or after lesions of the oculomotor nerve. Our patient was a 55-year-old man with hypertension and atrial fibrillation, who presented to us with acute onset left-sided ptosis and right-sided eyelid retraction. He was apathic and had right-sided ataxia. His MRI of the brain showed acute infarct involving the paramedian midbr...
Source: Journal of Postgraduate Medicine - July 14, 2020 Category: Internal Medicine Authors: S Sheetal R Thomas AS Kumar LG Tomy Source Type: research

High {kappa} free light chain is a potential biomarker for double seronegative and ocular myasthenia gravis
Conclusions Elevated serum FLC may serve as a biomarker for MG in suspected patients who are double seronegative and in those with only ocular manifestations when serology is inconclusive. Classification of evidence This study provides Class III evidence that high FLC levels distinguished patients with MG, including those who were double seronegative, from healthy controls. (Source: Neurology Neuroimmunology and Neuroinflammation)
Source: Neurology Neuroimmunology and Neuroinflammation - July 14, 2020 Category: Neurology Authors: Wilf-Yarkoni, A., Alkalay, Y., Brenner, T., Karni, A. Tags: Autoimmune diseases, Myasthenia, Class III Article Source Type: research

Ocular Myasthenia Gravis with Severe Blepharitis and Ocular Surface Disease: A Case Report
We describe a 16-year-old female who presented to our clinic with 1-month complaints of diplopia and strabismus, visual acuity deterioration, and ocular irritation. Her examination showed crossed diplopia and alternating exotropia of 25 prism diopters, severe blepharitis, conjunctival hyperemia, corneal pannus, epithelial irregularities, and subepithelial opacities. Workup included pediatric neurologic examination, laboratory tests, imaging, and electrophysiological tests. Diagnoses of OMG and blepharitis with ocular surface disease were made. Topical treatment included eyelid hygiene, tea tree oil scrubbing, topical stero...
Source: Case Reports in Ophthalmology - July 13, 2020 Category: Opthalmology Source Type: research

A case report of an intermediate phenotype between congenital myasthenic syndrome and D-2- and L-2-hydroxyglutaric aciduria due to novel SLC25A1 variants
Variants in the SLC25A1 gene are associated with a severe neurometabolic disease, D-2- and L-2-hydroxyglutaric aciduria (D/L-2-HGA). A report in 2014 presented the first account of congenital myasthenic syndrome ... (Source: BMC Neurology)
Source: BMC Neurology - July 13, 2020 Category: Neurology Authors: Wenhui Li, Min Zhang, Linmei Zhang, Yiyun Shi, Lei Zhao, Bingbing Wu, Xihua Li and Shuizhen Zhou Tags: Case report Source Type: research

A Practical Approach to Managing Patients With Myasthenia Gravis —Opinions and a Review of the Literature
When the diagnosis of myasthenia gravis (MG) has been secured, the aim of management should be prompt symptom control and the induction of remission or minimal manifestations. Symptom control, with acetylcholinesterase inhibitors such as pyridostigmine, is commonly employed. This may be sufficient in mild disease. There is no single universally accepted treatment regimen. Corticosteroids are the mainstay of immunosuppressive treatment in patients with more than mild MG to induce remission. Immunosuppressive therapies, such as azathioprine are prescribed in addition to but sometimes instead of corticosteroids when backgroun...
Source: Frontiers in Neurology - July 7, 2020 Category: Neurology Source Type: research

Qiangji Jianli Decoction promotes mitochondrial biogenesis in skeletal muscle of myasthenia gravis rats via AMPK/PGC-1α signaling pathway
Publication date: September 2020Source: Biomedicine & Pharmacotherapy, Volume 129Author(s): Wei Jiao, Fangyu Hu, Jinqiu Li, Jingwei Song, Jian Liang, Lanqi Li, Yafang Song, Zhiwei Chen, Qing Li, Lingling Ke (Source: Biomedicine and Pharmacotherapy)
Source: Biomedicine and Pharmacotherapy - July 6, 2020 Category: Drugs & Pharmacology Source Type: research

Noninvasive Ventilation in Myasthenia Gravis
Conclusion: With NIV, intubation was prevented in 7 and reintubation in 3 patients. NIV has lower incidence of complications. NIV should be tried in patients with impending MC. (Source: Neurology India)
Source: Neurology India - July 6, 2020 Category: Neurology Authors: Usha K Misra Surendra Kumar Varun Kumar Singh Deepanshu Dubey Jayantee Kalita Source Type: research

Rituximab as Adjunct Maintenance Therapy for Refractory Juvenile Myasthenia Gravis
The objective of our study was to assess the tolerability and efficacy of rituximab use in children with refractory JMG. (Source: Pediatric Neurology)
Source: Pediatric Neurology - July 6, 2020 Category: Neurology Authors: Carla D. Zingariello, Melissa E. Elder, Peter B. Kang Tags: Short Communication Source Type: research

Robotic surgery vs. open surgery for thymectomy, a retrospective case-match study
AbstractThe robotic approach in the treatment of thymus diseases has been described in many papers, but few studies have compared the early outcome of patients after robotic and open transsternal procedure. Our study aims to confirm the non-inferiority of the robotic technique in terms of feasibility, safety and postoperative patient recovery compared to the open standard. This is a retrospective cohort study in which we compare 114 patients who underwent thymectomy for a thymus disease at our thoracic surgery unit. Our robotic surgery programme started in February 2012 with the treatment of mediastinal diseases. Since the...
Source: Journal of Robotic Surgery - July 6, 2020 Category: Surgery Source Type: research

Establishment of (JUCTCi007-A) iPSC line from a patient with congenital myasthenic syndrome (CMS) carrying a homozygous mutation p.Arg331Trp (c.991C>T) in the CHRNE gene
Publication date: Available online 3 July 2020Source: Stem Cell ResearchAuthor(s): Nidaa A. Ababneh, Ban Alkurdi, Dema Ali, Duaa Abuarqoub, Raghda Barham, Abdi T. Ryalat, Abdalla Awidi (Source: Stem Cell Research)
Source: Stem Cell Research - July 5, 2020 Category: Stem Cells Source Type: research

Hendricks TM, Bhatti MT, Hodge DO, Chen JJ. Incidence, Epidemiology, and Transformation of Ocular Myasthenia Gravis: A Population-Based Study. Am J Ophthalmol 2019;205:99 –105. (Source: American Journal of Ophthalmology)
Source: American Journal of Ophthalmology - July 4, 2020 Category: Opthalmology Tags: Corrigendum Source Type: research

Effects of Teriflunomide on B Cell Subsets in MuSK-Induced Experimental Autoimmune Myasthenia Gravis and Multiple Sclerosis.
Authors: Yilmaz V, Ulusoy C, Hajtovic S, Turkoglu R, Kurtuncu M, Tzartos J, Lazaridis K, Tuzun E Abstract Antigen-specific immune responses are crucially involved in both multiple sclerosis (MS) and myasthenia gravis (MG). Teriflunomide is an immunomodulatory agent approved for treatment of MS through inhibition of lymphocyte proliferation. MG associated with muscle-specific tyrosine kinase (MuSK) antibodies often manifests with a severe disease course, prompting development of effective treatment methods. To evaluate whether teriflunomide treatment may ameliorate MuSK-autoimmunity, experimental autoimmune MG (EAMG...
Source: Immunological Investigations - July 2, 2020 Category: Allergy & Immunology Tags: Immunol Invest Source Type: research

Case Report: A Rare Case of Silent Sinus Syndrome and Vertical Diplopia
CONCLUSIONS Vertical diplopia is a common complaint in primary care optometry. It is important to delve into more rare etiologies when clinical, laboratory, and radiological studies do not support the more common diagnoses. (Source: Optometry and Vision Science)
Source: Optometry and Vision Science - July 1, 2020 Category: Opthalmology Tags: REPORTS Source Type: research

Fatigue in Patients with Myasthenia Gravis. A Systematic Review of the Literature.
Myasthenia Gravis (MG) is a chronic autoimmune disease (AID) with a prevalence of 1-2 per 10.000 [1]. The clinical hallmark is fluctuating weakness of striated muscles with antibodies directly affecting the neuromuscular junction. In approximately 85% of patients, the initial presenting symptoms are asymmetric ptosis and/or diplopia. Approximately 80% of initially ocular MG patients will develop generalized MG within two years of disease onset [2]. (Source: Neuromuscular Disorders)
Source: Neuromuscular Disorders - July 1, 2020 Category: Neurology Authors: Annabel M. Ruiter, Jan J.G.M. Verschuuren, Martijn R. Tannemaat Tags: Systematic review article Source Type: research

Management of immune checkpoint inhibitor-related acute hypoxic neuromuscular respiratory failure using high-flow nasal cannula.
We describe use of high-flow nasal cannula in a patient with acute hypoxic respiratory failure and psychological distress due to the immune-related adverse event of myasthenic crisis. PMID: 32675966 [PubMed] (Source: Baylor University Medical Center Proceedings)
Source: Baylor University Medical Center Proceedings - July 1, 2020 Category: Universities & Medical Training Authors: Mathews EP, Romito JW Tags: Proc (Bayl Univ Med Cent) Source Type: research

Fatigue in patients with myasthenia gravis. A systematic review of the literature
Myasthenia Gravis (MG) is a chronic autoimmune disease (AID) with a prevalence of 1 –2 per 10.000 [1]. The clinical hallmark is fluctuating weakness of striated muscles with antibodies directly affecting the neuromuscular junction. In approximately 85% of patients, the initial presenting symptoms are asymmetric ptosis and/or diplopia. Approximately 80% of initially ocular MG pati ents will develop generalized MG within two years of disease onset [2]. (Source: Neuromuscular Disorders)
Source: Neuromuscular Disorders - July 1, 2020 Category: Neurology Authors: Annabel M. Ruiter, Jan J.G.M. Verschuuren, Martijn R. Tannemaat Tags: Review Source Type: research

Novel Treatments in Myasthenia Gravis
Myasthenia gravis (MG) is the prototypical autoimmune disorder caused by specific autoantibodies at the neuromuscular junction. Broad-based immunotherapies, such as corticosteroids, azathioprine, mycophenolate, tacrolimus, and cyclosporine, have been effective in controlling symptoms of myasthenia. While being effective in a majority of MG patients many of these immunosuppressive agents are associated with long-term side effects, often intolerable for patients, and take several months to be effective. With advances in translational research and drug development capabilities, more directed therapeutic agents that can alter ...
Source: Frontiers in Neurology - June 30, 2020 Category: Neurology Source Type: research

Eculizumab for the treatment of myasthenia gravis
. (Source: Expert Opinion on Biological Therapy)
Source: Expert Opinion on Biological Therapy - June 30, 2020 Category: Drugs & Pharmacology Authors: Renato Mantegazza Paola Cavalcante Source Type: research

Bioactivity and molecular docking studies of some nickel complexes: New analogues for the treatment of Alzheimer, glaucoma and epileptic diseases.
In this study, enzyme inhibition of DSA (1-3) coordination compounds that were previously investigated for their anticancer and antibacterial properties was investigated. Also, DSA (1-3) had Ki values of 635.30 + 152.62, 184.01 + 90.05, and 163.03 ± 60.01 µM against human carbonic anhydrase I, 352.23 ± 143.09, 46.2 ± 15.47, and 54.117 ± 18.80 µM against AChE, 310.64 ± 97.35, 35.54 ± 7.01, and 101.51 ± 15.314 µM against BChE, respectively. The biological ...
Source: Bioorganic Chemistry - June 30, 2020 Category: Chemistry Authors: Kısa D, Korkmaz N, Taslimi P, Tuzun B, Tekin Ş, Karadag A, Şen F Tags: Bioorg Chem Source Type: research

Tolosa-Hunt syndrome with general myasthenia gravis involvement.
We present a patient with a probable coincidence of Tolosa-Hunt syndrome and general myasthenia gravis. A 63-year-old male exhibited episodes of unilateral headache with double vision, bilateral ptosis, vision decrease in the left eye and left facial hypoesthesia, muscle weakness in limbs and neck. The muscle weakness was fluctuating and could be relieved by rest. Blood analysis, cranial magnetic resonance imaging, magnetic resonance angiography/venogram) and orbit/mediastinum computed tomography demonstrated no abnormalities. Serum myasthenia gravis related antibodies detection showed positive titin- antibodies and ryanod...
Source: Journal of Integrative Neuroscience - June 30, 2020 Category: Neuroscience Authors: Li L, Wang Z, Lu MO Tags: J Integr Neurosci Source Type: research