17,256 results match your criteria Myasthenia Gravis


Nocebo effect in myasthenia gravis: systematic review and meta-analysis of placebo-controlled clinical trials.

Acta Neurol Belg 2019 Apr 19. Epub 2019 Apr 19.

Academic Department of Neurosciences, Sheffield Teaching Hospitals, NHS Trust, Sheffield, UK.

Nocebo refers to the adverse events (AEs) experienced when taking a placebo drug and is believed to be a centrally mediated process. We sought to examine the AEs following placebo administration in Randomised Controlled Trials (RCTs) for Myasthenia Gravis (MG) patients. A systematic literature search was performed on Medline and Web of Science for RCTs for MG pharmacological treatments. Read More

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http://dx.doi.org/10.1007/s13760-019-01143-1DOI Listing

Severe Myasthenic Manifestation of Leptospirosis Associated with New Sequence Type of Leptospira interrogans.

Emerg Infect Dis 2019 May;25(5):968-971

We report the rapid development of a myasthenic crisis as the first-time manifestation of myasthenia gravis. The symptoms developed in the course of acute leptospirosis associated with a new sequence type of Leptospira interrogans. Antibiotic treatment led to rapid amelioration of myasthenia. Read More

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http://wwwnc.cdc.gov/eid/article/25/5/18-1591_article.htm
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http://dx.doi.org/10.3201/eid2505.181591DOI Listing
May 2019
1 Read

Development of a dual-functional conjugate of antigenic peptide and Fc-III mimetics (DCAF) for targeted antibody blocking.

Chem Sci 2019 Mar 28;10(11):3271-3280. Epub 2019 Jan 28.

MOE Key Laboratory of Bioinformatics , Center for Synthetic and Systems Biology , School of Life Sciences , Tsinghua University , Beijing , China . Email:

Targeted antibody blocking enables characterization of binding sites on immunoglobulin G (IgG), and can efficiently eliminate harmful antibodies from organisms. In this report, we present a novel peptide-denoted as a dual-functional conjugate of antigenic peptide and Fc-III mimetics (DCAF)-for targeted blocking of antibodies. Synthesis of DCAF was achieved by native chemical ligation, and the molecule consists of three functional parts: a specific antigenic peptide, a linker and the Fc-III mimetic peptide, which has a high affinity toward the Fc region of IgG molecules. Read More

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http://xlink.rsc.org/?DOI=C8SC05273E
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http://dx.doi.org/10.1039/c8sc05273eDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6429600PMC
March 2019
1 Read

Third distant recurrence of benign thymoma in a patient with myasthenia gravis.

BMJ Case Rep 2019 Apr 14;12(4). Epub 2019 Apr 14.

Thoracic Surgery, Albany Medical Center Hospital, Albany, New York, USA.

Myasthenia gravis (MG) is an autoimmune disease where antibodies attack the presynaptic terminals at the neuromuscular junction causing progressive weakness. Associated with thymomas, resection can improve symptoms. A 29-year-old woman with MG who underwent two previous thymectomies, at ages 11 and 15 presented 14 years later with recurrent MG symptoms and an anterior mediastinal mass. Read More

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http://casereports.bmj.com/lookup/doi/10.1136/bcr-2018-22852
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http://dx.doi.org/10.1136/bcr-2018-228529DOI Listing
April 2019
2 Reads

Rechallenge of immune checkpoint inhibitor after pembrolizumab-induced myasthenia gravis.

Eur J Cancer 2019 Apr 12;113:72-74. Epub 2019 Apr 12.

Department of Dermatology, APHP Saint Louis Hospital, Paris, France; Université Paris 7 Diderot, Sorbonne Paris Cité, Paris, France.

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http://dx.doi.org/10.1016/j.ejca.2019.03.006DOI Listing

Autoimmune myositis and myasthenia gravis resulting from a combination therapy with nivolumab and ipilimumab for metastatic melanoma.

Eur J Rheumatol 2019 Apr 9. Epub 2019 Apr 9.

Department of Rheumatology, Yale New Haven Hospital, Connecticut, USA.

Checkpoint inhibitors are a novel option in the management of metastatic melanomas and many other malignancies. They are used to promote the activation of cytotoxic T-lymphocytes by inhibiting deactivation signals, enabling the immune response to the tumor. Numerous Immune-related adverse effects caused by checkpoint inhibitors have been reported in the literature. Read More

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http://dx.doi.org/10.5152/eurjrheum.2019.18159DOI Listing
April 2019
1 Read

Causes and Consequences of miR-150-5p Dysregulation in Myasthenia Gravis.

Front Immunol 2019 29;10:539. Epub 2019 Mar 29.

Center of Research in Myology, Sorbonne University, INSERM, Association Institute of Myology - UMRS 974, Paris, France.

Autoimmune Myasthenia gravis (MG) is a chronic neuromuscular disease mainly due to antibodies against the acetylcholine receptor (AChR) at the neuromuscular junction that induce invalidating muscle weaknesses. In early-onset MG, the thymus is the effector organ and is often characterized by B-cell infiltrations leading to ectopic germinal center (GC) development. The microRNA miR-150-5p has been previously characterized as a biomarker in MG due to its increase in the serum of patients and its decrease after thymectomy, correlated with an improvement of symptoms. Read More

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https://www.frontiersin.org/article/10.3389/fimmu.2019.00539
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http://dx.doi.org/10.3389/fimmu.2019.00539DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6450174PMC
March 2019
1 Read

A study of comorbidities in myasthenia gravis.

Acta Neurol Belg 2019 Apr 10. Epub 2019 Apr 10.

Department of Neurology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Raebareily Road, Lucknow, Uttar Pradesh, 226014, India.

Management of myasthenia gravis (MG) in the presence of comorbidities may be difficult. We report the effect of comorbidities in the outcome of MG. The patients with MG during 1991-2016 were included and evaluated including their demographic variables, clinical findings, Myasthenia Gravis Foundation of America (MGFA) score. Read More

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http://link.springer.com/10.1007/s13760-019-01102-w
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http://dx.doi.org/10.1007/s13760-019-01102-wDOI Listing
April 2019
8 Reads

Long-Term Safety and Efficacy of Fecal Microbiota Transplant in Active Ulcerative Colitis.

Drug Saf 2019 Apr 10. Epub 2019 Apr 10.

Medical Center for Digestive Diseases, The Second Affiliated Hospital, Nanjing Medical University, 121 Jiang Jia Yuan, Nanjing, 210011, China.

Introduction And Objective: The therapeutic role of fecal microbiota transplantation in ulcerative colitis varies across different reports. This study aims to evaluate the long-term safety and efficacy of a strategy called step-up fecal microbiota transplantation for ulcerative colitis.

Methods: Two clinical trials (NCT01790061, NCT02560727) for moderate-to-severe ulcerative colitis (Mayo score range 6-12) were performed from November 2012 to July 2017. Read More

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http://link.springer.com/10.1007/s40264-019-00809-2
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http://dx.doi.org/10.1007/s40264-019-00809-2DOI Listing
April 2019
1 Read

Retraction Note to: Juvenile-onset myasthenia gravis: autoantibody status, clinical characteristics and genetic polymorphisms.

J Neurol 2019 Apr 11. Epub 2019 Apr 11.

Department of Clinical Medicine, University of Bergen, Bergen, Norway.

The Joint Editors-in-Chief have retracted this article [1] at the request of the University of Bergen and the Norwegian Board of Health Supervision. Read More

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http://dx.doi.org/10.1007/s00415-019-09292-2DOI Listing

[Myasthenia gravis induced by inmuno checkpoints inhibitors: first case report secondary to avelumab therapy and review of published cases].

Rev Neurol 2019 Apr;68(8):333-338

Hospital Regional Universitario de Malaga, Malaga, Espana.

Introduction: The inmuno checkpoints inhibitors are new revolutionary treatment for many neoplastic diseases in advanced stadium. There are described several types of neurological complications induced by nivolumab: polyneuropathy, seizures, radiculitis and myasthenia gravis disease.

Case Report: A 65 years old man with metastatic lung adenocarcinoma who presented myasthenia gravis disease induced by avelumab therapy with good response to treatment with pyridostigmine and withdrawal of avelumab. Read More

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http://dx.doi.org/10.33588/rn.6808.2018497DOI Listing

Microscopic polyangiitis associated with thymic tumor: a case report and review of the literature.

BMC Nephrol 2019 Apr 8;20(1):123. Epub 2019 Apr 8.

Department of Nephrology, Tokyo Medical University Ibaraki Medical Center, 3-20-1 Chuo, Ami, Ibaraki, 300-0395, Japan.

Background: Thymic hyperplasia and thymic epithelial tumor (thymoma) have been associated with a variety of autoimmune diseases. Renal involvement has been reported in patients with thymoma. Minimal change disease and membranous nephropathy are frequently observed in glomerular lesions of thymoma patients, but ANCA-associated renal vasculitis is rare. Read More

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http://dx.doi.org/10.1186/s12882-019-1319-9DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6454690PMC
April 2019
1 Read

False-positive acetylcholine receptor antibody results in patients without myasthenia gravis.

J Neuroimmunol 2019 Apr 3;332:69-72. Epub 2019 Apr 3.

Nuffield Department of Clinical Neurosciences,West Wing, John Radcliffe Hospital, University of Oxford, Oxford OX3 9DS, UK.

Acetylcholine receptor antibodies are very specific for myasthenia. During a large prospective cohort study of myasthenia, we encountered five patients, positive for acetylcholine receptor (AChR) antibodies by radioimmunoprecipitation assay (RIA), whose clinical course revealed diagnoses other than myasthenia. Two patients had transiently raised AChR antibodies associated with Guillain-Barré syndrome. Read More

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http://dx.doi.org/10.1016/j.jneuroim.2019.04.001DOI Listing
April 2019
2 Reads

Gestational Gigantomastia in the Setting of Myasthenia Gravis.

J Obstet Gynaecol India 2019 Apr 30;69(Suppl 1):84-87. Epub 2018 Oct 30.

Department of Obstetrics and Gynaecology, Swami Rama Himalayan University, Dehradun, Uttarakhand India.

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http://dx.doi.org/10.1007/s13224-018-1184-7DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6431701PMC

Unusual cause of progressively impaired cognitive function: complex meningoencephalitis.

BMJ Case Rep 2019 Apr 3;12(4). Epub 2019 Apr 3.

Department of Internal Medicine, Akiota Hospital, Yamagata-gun, Hiroshima, Japan.

complex (MAC) is a rare cause of meningoencephalitis. Non-tuberculous mycobacterium meningoencephalitis including MAC meningoencephalitis is an important cause of rapidly progressive dementia. We present a case of MAC meningoencephalitis in an immunosuppressed woman who had progressively impaired cognitive function. Read More

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http://casereports.bmj.com/lookup/doi/10.1136/bcr-2018-22902
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http://dx.doi.org/10.1136/bcr-2018-229022DOI Listing
April 2019
4 Reads

Building the drug-GO function network to screen significant candidate drugs for myasthenia gravis.

PLoS One 2019 4;14(4):e0214857. Epub 2019 Apr 4.

Department of Neurology, The Second Affiliated Hospital, Harbin Medical University, Harbin, Heilongjiang Province, China.

Myasthenia gravis (MG) is an autoimmune disease. In recent years, considerable evidence has indicated that Gene Ontology (GO) functions, especially GO-biological processes, have important effects on the mechanisms and treatments of different diseases. However, the roles of GO functions in the pathogenesis and treatment of MG have not been well studied. Read More

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0214857PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6448860PMC
April 2019
3 Reads
3.234 Impact Factor

Upregulation of miR150-5p in generalized myasthenia gravis patients is associated with decreased serum levels of IL-17 and increased serum levels of IL-10.

Biomed Pap Med Fac Univ Palacky Olomouc Czech Repub 2019 Mar 29. Epub 2019 Mar 29.

Department of Neurology, Shenzhen Hospital, Southern Medical University, Shenzhen City, 518000, PR. China.

Background: MiR150-5p has been reported to be involved in generalized myasthenia gravis, in which different cytokines play critical roles. The regulatory network of cytokines in generalized myasthenia gravis has not been fully elucidated. Our study aimed to investigate the interactions between miR150-5p and different cytokines in generalized myasthenia gravis. Read More

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http://dx.doi.org/10.5507/bp.2019.009DOI Listing

Prominent T-Cell Responses against the Acetylcholine Receptor Subunit in Myasthenia Gravis.

Neurol Res Int 2019 3;2019:1969068. Epub 2019 Mar 3.

Department of Neurology, University of Tübingen, Tübingen, Germany.

The human acetylcholine receptor (AChR) is well characterized as the target antigen in myasthenia gravis (MG). Pathogenic antibody responses against the AChR alpha-chain have been investigated extensively and are of diagnostic and prognostic value. However, less is known on the pathogenetic relevance of T-cell responses against epitopes of the different AChR chains (alpha, epsilon, gamma). Read More

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https://www.hindawi.com/journals/nri/2019/1969068/
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http://dx.doi.org/10.1155/2019/1969068DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6421006PMC
March 2019
2 Reads

Non-Neoplastic and Benign Tumoral Lesions of the Thymic Gland: A Review and Update.

Adv Anat Pathol 2019 Mar 29. Epub 2019 Mar 29.

Department of Pathology, MD Anderson Cancer Center, Houston, TX.

The thymus is a dynamic organ that undergoes changes throughout life and can demonstrate a myriad of pathologic alterations. A number of benign entities of the thymus prove to be diagnostic dilemmas owing to their resemblance and association with true thymic tumors. These are usually discovered incidentally on routine imaging and most patients are either asymptomatic or present with signs and symptoms of compression of adjacent organs. Read More

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http://dx.doi.org/10.1097/PAP.0000000000000231DOI Listing

Corrigendum to: "low and high doses of ursolic acid ameliorate experimental autoimmune myasthenia gravis through different pathways" [Journal of Neuroimmunology 281 (2015) 61-67].

J Neuroimmunol 2019 May;330:181-183

Department of Neurology, Shandong Provincial Qianfoshan Hospital, Shandong University, Jinan 250014, PR China. Electronic address:

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http://dx.doi.org/10.1016/j.jneuroim.2019.03.016DOI Listing
May 2019
2 Reads

Relation of HLA-DRB1 to IgG4 autoantibody and cytokine production in muscle-specific tyrosine kinase myasthenia gravis (MuSK-MG).

Clin Exp Immunol 2019 Mar 31. Epub 2019 Mar 31.

Department of Physiology, Istanbul Medical Faculty, Istanbul University, Istanbul, Turkey.

A small subset of myasthenia gravis (MG) patients develop autoantibodies against muscle-specific kinase (MuSK), which are predominantly of the immunoglobulin (Ig)G4 isotype. MuSK-MG is strongly associated with HLA-DRB1*14, HLA-DRB1*16 and HLA-DQB1*05. In this study, the possible effects of these HLA associations on MuSK IgG autoantibody or cytokine production were investigated. Read More

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http://dx.doi.org/10.1111/cei.13302DOI Listing
March 2019
1 Read

Non-Myasthenia Gravis Immune Syndromes and the Thymus: Is There a Role for Thymectomy?

Thorac Surg Clin 2019 May 7;29(2):215-225. Epub 2019 Mar 7.

Division of Thoracic Surgery, Department of Cardiothoracic Surgery, Stanford University Hospitals and Clinics, 300 Pasteur Drive, Stanford, CA 94305, USA. Electronic address:

Thymectomy has long been considered, performed, and discussed for many different nonmyasthenic immune syndromes. Thymectomy is now an established treatment for MG, and has been performed for other immune syndromes with varying degrees of improvement. Although numerous reports document immune syndromes' association with thymoma, few address the role of thymectomy in symptom resolution. Read More

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http://dx.doi.org/10.1016/j.thorsurg.2018.12.008DOI Listing
May 2019
3 Reads

Surgical Decision Making: Thymoma and Myasthenia Gravis.

Thorac Surg Clin 2019 May 7;29(2):203-213. Epub 2019 Mar 7.

Thoracic Surgery Unit, Department of Cardiologic, Thoracic and Vascular Sciences, University Hospital, Padova, Italy. Electronic address:

About 15% of patients with myasthenia gravis are affected by thymoma. Precise tumor staging is necessary to plan the appropriate operation. In early stages, complete surgical resection is the mainstay of treatment. Read More

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http://dx.doi.org/10.1016/j.thorsurg.2018.12.007DOI Listing
May 2019
3 Reads

Technique for Myasthenia Gravis: Subxiphoid Approach.

Thorac Surg Clin 2019 May;29(2):195-202

Department of Thoracic Surgery, Shanghai Pulmonary Hospital, Tongji University School of Medicine, Shanghai 200433, China. Electronic address:

Kido and colleagues in 1999 used for the first time the subxiphoid approach. Recently, video-assisted thoracoscopic surgery (VATS) thymectomy has been improved for the advancement of surgical materials and new instruments. The most important aspect regarding the subxiphoid approach is the possibility to avoid the intercostal nerve damage with the consequence of a decreased use of postoperative analgesics, quite short surgical duration, fast discharge from hospital and a guarantee of successful cosmetics results. Read More

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http://dx.doi.org/10.1016/j.thorsurg.2018.12.010DOI Listing
May 2019
3 Reads

Uniportal Video-Assisted Transcervical Thymectomy.

Thorac Surg Clin 2019 May;29(2):187-194

Division of Thoracic Surgery, Innovation, Department of Surgery, University of Toronto, University Health Network, Toronto General Hospital, 200 Elizabeth Street 9N-946, Toronto, ON M5G 2C4, Canada. Electronic address:

Surgery has proven superiority over medical management for patients with nonthymomatous myasthenia gravis. The key is complete resection of the gland, which can be achieved with various techniques. The uniportal video-assisted transcervical technique allows minimally invasive surgery with a low complication rate, a good cosmetic result, and a short length of recovery. Read More

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http://dx.doi.org/10.1016/j.thorsurg.2018.12.009DOI Listing
May 2019
3 Reads

Surgical Techniques for Myasthenia Gravis: Robotic-Assisted Thoracoscopic Surgery.

Thorac Surg Clin 2019 May;29(2):177-186

Department of Surgery, Competence Center of Thoracic Surgery, Charité University Hospital Berlin, Charitéplatz 1, Berlin 10117, Germany. Electronic address:

Robotic-assisted thoracoscopic surgery (RATS) creates an extended approach for thymectomy in terms of locating contralateral phrenic nerve and upper poles of the thymus. RATS thymectomy is indicated in all non-thymomatous myasthenia gravis (MG) patients and thymomatous MG patients with resectable thymoma, typically Masaoka-Koga I and II. Left-sided RATS thymectomy is superior for anatomic reasons and in the special care patients with MG. Read More

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http://dx.doi.org/10.1016/j.thorsurg.2018.12.006DOI Listing
May 2019
3 Reads

Surgical Techniques for Myasthenia Gravis: Video-Assisted Thoracic Surgery.

Thorac Surg Clin 2019 May 7;29(2):165-175. Epub 2019 Mar 7.

Myasthenia Gravis Multidisciplinary Program, Tor Vergata University, Viale Oxford 81, Rome 00133, Italy; Department of Surgical Sciences, Tor Vergata University, Via Montpellier 1, Rome 00133, Italy; Division of Thoracic Surgery, Tor Vergata University Policlinic, Viale Oxford 81, Rome 00133, Italy. Electronic address:

We describe the various video-assisted thoracic surgery approaches to the thymus currently adopted in nonthymomatous and thymomatous myasthenic patients. Despite several controversies, video-assisted thoracic surgery thymectomy gained worldwide popularity. Classic 3-port approaches proved safe and effective. Read More

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http://dx.doi.org/10.1016/j.thorsurg.2018.12.005DOI Listing
May 2019
6 Reads

Surgical Approaches to Myasthenia Gravis: Perspective of Anatomy and Radicality in Surgery.

Thorac Surg Clin 2019 May 7;29(2):159-164. Epub 2019 Mar 7.

Department of the Thoracic Surgery, Pulmonary Hospital, Ul. Gładkie 1, Zakopane 34 500, Poland. Electronic address:

The effectiveness of thymectomy for nonthymomatous myasthenia gravis is analyzed with regard to extensiveness of removal of the adipose tissue of the mediastinum and the lower neck region with various techniques of thymectomy. The approach necessary to achieve maximal radicality must include access to both pleural cavity and the lower neck area up to the thyroid gland. Surgical techniques include extended transsternal approach, which might be combined with separate transcervical incision, bilateral videothoracoscopic (VATS) approach combined with transcervical incision, and extended subxiphoid approach, either uniportal or combined with bilateral VATS. Read More

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http://dx.doi.org/10.1016/j.thorsurg.2018.12.004DOI Listing
May 2019
2 Reads

History of Thymectomy for Myasthenia Gravis.

Authors:
Joel D Cooper

Thorac Surg Clin 2019 May;29(2):151-158

Department of Surgery, Hospital of the University of Pennsylvania, 3400 Spruce Street, 6 White, Philadelphia, PA 19104, USA. Electronic address:

In the early 1900s, chance observations of improved symptoms in several myasthenic patients undergoing thyroidectomy for goiters with concomitant resection of the adjacent thymus gland, first suggested a possible association between the thymus and myasthenia gravis. With the remarkable progress made in the understanding and treatment of myasthenia and in the anesthetic, surgical, and postoperative management of patients undergoing thoracic procedures, the initial high morbidity and mortality associated with thymectomy have been all but eliminated, and thymectomy is frequently incorporated into the long-term management strategy of this disease. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S15474127183016
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http://dx.doi.org/10.1016/j.thorsurg.2018.12.011DOI Listing
May 2019
8 Reads

A Neurologist's Perspective on Thymectomy for Myasthenia Gravis: Current Perspective and Future Trials.

Thorac Surg Clin 2019 May 7;29(2):143-150. Epub 2019 Mar 7.

Department of Neurology, Jacobs School of Medicine and Biomedical Sciences, University at Buffalo/SUNY, 1010 Main Street, 2nd Floor, Buffalo, NY 14202, USA. Electronic address:

The first randomized blinded study of thymectomy in nonthymomatous myasthenia gravis was designed to answer 3 questions: does the combination of prednisone and removal of the thymus gland via extended transsternal thymectomy after 3 years compared with an identical dosing protocol of prednisone alone (1) lead to better disease status for generalized MG patients with antiacetylcholine receptor antibodies, (2) reduce their prednisone requirements, and/or (3) reduce the side-effect burden from medications used to treat the disease? The study demonstrated that thymectomy confers these benefits for patients and sets the stage for inquiries into the benefits of less-invasive approaches to thymic resection. Read More

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http://dx.doi.org/10.1016/j.thorsurg.2018.12.003DOI Listing
May 2019
8 Reads

A Neurologist's Perspective on Understanding Myasthenia Gravis: Clinical Perspectives of Etiologic Factors, Diagnosis, and Preoperative Treatment.

Thorac Surg Clin 2019 May 7;29(2):133-141. Epub 2019 Mar 7.

Department of Neurology, George Washington University, 2150 Pennsylvania Avenue, NW 9th Floor, Washington, DC 20037, USA. Electronic address:

Myasthenia gravis (MG) is a disease of neuromuscular transmission caused by antibodies directed toward proteins concentrated at the neuromuscular junction. Mild to life-threatening weakness varies in severity over time and with level of activity. Therefore, clinical diagnosis is often challenging. Read More

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http://dx.doi.org/10.1016/j.thorsurg.2018.12.002DOI Listing
May 2019
2 Reads

Neuronal intranuclear inclusion disease showing blepharoptosis and positive serum anti-acetylcholine receptor antibody without myasthenia gravis.

J Neurol Sci 2019 May 16;400:119-121. Epub 2019 Mar 16.

Department of Neurology and Neurobiology of Aging, Kanazawa University Graduate School of Medical Sciences, Kanazawa, Japan. Electronic address:

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http://dx.doi.org/10.1016/j.jns.2019.03.013DOI Listing

Natural cholinesterase inhibitors from marine organisms.

Nat Prod Rep 2019 Mar 29. Epub 2019 Mar 29.

Department of Chemistry and Materials, RISE Research Institutes of Sweden, Box 857, SE-501 15 Borås, Sweden.

Covering: Published between 1974 up to 2018Inhibition of cholinesterases is a common approach for the management of several disease states. Most notably, cholinesterase inhibitors are used to alleviate the symptoms of neurological disorders like dementia and Alzheimer's disease and treat myasthenia gravis and glaucoma. Historically, most drugs of natural origin have been isolated from terrestrial sources and inhibitors of cholinesterases are no exception. Read More

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http://dx.doi.org/10.1039/c9np00010kDOI Listing

Rituximab as induction therapy in refractory myasthenia gravis: 18 month follow-up study.

J Neurol 2019 Mar 27. Epub 2019 Mar 27.

Department of Neurology, Neurosciences Centre, All India Institute of Medical Sciences, New Delhi, 110029, India.

Background: Myasthenia gravis is an immune-mediated disorder characterized by easy fatigability and diurnal variation in skeletal muscle weakness. Aim of therapy is to prevent crisis and maintain remission. However, despite standard therapy, some remain refractory to treatment. Read More

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http://dx.doi.org/10.1007/s00415-019-09296-yDOI Listing
March 2019
9 Reads

Cytometric cell-based assays for anti-striational antibodies in myasthenia gravis with myositis and/or myocarditis.

Sci Rep 2019 Mar 27;9(1):5284. Epub 2019 Mar 27.

Department of Neurology, Keio University School of Medicine, 35 Shinanomachi, Shinjuku-ku, Tokyo, 160-8582, Japan.

The purposes of the present study were to identify anti-striational antibodies in myasthenia gravis (MG) patients with myositis and/or myocarditis using a combination of cell-based assays and flow cytometry (cytometric cell-based assays) and to describe the main clinical implications. Among 2,609 stored samples collected from all over Japan between 2003 and 2016, we had serum samples from 30 MG patients with myositis and/or myocarditis. Cytometric cell-based assays with titin, ryanodine receptor, and voltage-gated Kv1. Read More

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http://dx.doi.org/10.1038/s41598-019-41730-zDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6437199PMC
March 2019
2 Reads

An update on thymectomy in myasthenia gravis.

Expert Rev Neurother 2019 Apr 5:1-11. Epub 2019 Apr 5.

b Fondazione Policlinico Gemelli , IRCCS , Roma , Italy.

Introduction: Myasthenia gravis (MG) is one of the best treatable autoimmune diseases. However, in most patients, treatment is necessarily long-term and related side effects are a serious burden. Thymectomy has a special place in the disease management as a non-pharmacological disease-modifying therapy. Read More

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http://dx.doi.org/10.1080/14737175.2019.1600404DOI Listing
April 2019
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Autoimmune Attack of the Neuromuscular Junction in Myasthenia Gravis: Nicotinic Acetylcholine Receptors and Other Targets.

ACS Chem Neurosci 2019 Apr 12. Epub 2019 Apr 12.

Laboratory of Molecular Neurobiology, Biomedical Research Institute (BIOMED) , UCA-CONICET , Av. Alicia Moreau de Justo 1600 , C1107AFF Buenos Aires , Argentina.

The nicotinic acetylcholine receptor (nAChR) family, the archetype member of the pentameric ligand-gated ion channels, is ubiquitously distributed in the central and peripheral nervous systems, and its members are the targets for both genetic and acquired forms of neurological disorders. In the central nervous system, nAChRs contribute to the pathological mechanisms of neurodegenerative disorders, such as Alzheimer and Parkinson diseases. In the peripheral nerve-muscle synapse, the vertebrate neuromuscular junction, "classical" myasthenia gravis (MG) and other forms of neuromuscular transmission disorders are antibody-mediated autoimmune diseases. Read More

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http://dx.doi.org/10.1021/acschemneuro.9b00041DOI Listing
April 2019
2 Reads

Pulmonary Function Testing in Elderly Patients Treated for a Myasthenia Gravis Exacerbation.

Neurohospitalist 2019 Apr 26;9(2):79-84. Epub 2018 Nov 26.

Department of Medicine (Critical Care), University of Ottawa, Ottawa, Ontario, Canada.

Background And Purpose: Pulmonary function testing is a standard part of care for patients admitted to hospital with a myasthenia gravis exacerbation. It may inform clinicians' decisions to intubate patients. It is known that pulmonary function declines with age in healthy adults. Read More

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http://dx.doi.org/10.1177/1941874418811249DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6429677PMC
April 2019
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Effect of Diaphragm Plication in Thymoma Patients with and without Myasthenia Gravis.

Tanaffos 2018 Mar;17(3):177-182

Lung Disease Research Center, Mashhad University of Medical Sciences, Mashhad, Iran.

Background: Thymoma is the most common tumor of the anterior mediastinum that has the most effective treatment, as it can be completely resected. In patients with advanced stage, phrenic nerve involvement can be seen and suggested treatment for these patients is unilateral phrenic excision and diaphragm plication. However in patients with myasthenia gravis, there are concerns in relation to this method of treatment. Read More

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6428380PMC
March 2018
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Eculizumab improves fatigue in refractory generalized myasthenia gravis.

Qual Life Res 2019 Mar 23. Epub 2019 Mar 23.

Department of Neurology, UNC School of Medicine, University of North Carolina at Chapel Hill, Chapel Hill, NC, USA.

Purpose: To evaluate the effect of eculizumab on perceived fatigue in patients with anti-acetylcholine receptor antibody-positive, refractory, generalized myasthenia gravis (MG) using the Quality of Life in Neurological Disorders (Neuro-QOL) Fatigue subscale, and to evaluate correlations between improvements in Neuro-QOL Fatigue and other clinical endpoints.

Methods: Neuro-QOL Fatigue, MG Activities of Daily Living (MG-ADL), Quantitative MG (QMG), and the 15-item MG Quality of Life (MG-QOL15) scales were administered during the phase 3, randomized, placebo-controlled REGAIN study (eculizumab, n = 62; placebo, n = 63) and subsequent open-label extension (OLE). Data were analyzed using repeated-measures models. Read More

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http://dx.doi.org/10.1007/s11136-019-02148-2DOI Listing
March 2019
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Cardiac autonomic function evaluation in pediatric and adult patients with congenital myasthenic syndromes.

Neuromuscul Disord 2019 Apr 19;29(4):290-295. Epub 2019 Feb 19.

Department of Pediatric Neurology, Hacettepe University Faculty of Medicine, Ankara, Turkey.

Cardiac autonomic dysfunction has been examined in myasthenia gravis but not in congenital myasthenic syndromes (CMS). We aimed to evaluate cardiac autonomic functions in genetically defined CMS. Patients diagnosed with and under treatment for CMS were reviewed for 24-hour cardiac rhythm monitoring. Read More

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http://dx.doi.org/10.1016/j.nmd.2019.02.004DOI Listing
April 2019
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2.638 Impact Factor

Comment on: Thymectomy in patients with myasthenia gravis increases the risk of autoimmune rheumatic diseases: a nationwide cohort study.

Rheumatology (Oxford) 2019 Mar 20. Epub 2019 Mar 20.

The Zabludowicz Center for Autoimmune Diseases, Sheba Medical Center, Tel-Aviv University, Tel-Hashomer, Israel.

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http://dx.doi.org/10.1093/rheumatology/kez093DOI Listing
March 2019
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Neurotoxicity associated with cancer immunotherapy: immune checkpoint inhibitors and chimeric antigen receptor T-cell therapy.

Curr Opin Neurol 2019 Mar 19. Epub 2019 Mar 19.

Department of Oncology.

Purpose Of Review: Immune checkpoint inhibitors (ICPI) and chimeric antigen receptor T cells (CAR-T) represent novel therapies recently approved to treat a number of human cancers. As both approaches modulate the immune system, they can generate a number of immune-related adverse events (irAEs), including a large spectrum of novel neurological toxicities. These are of special interest given their potential severity and risk of compromising further oncologic treatment. Read More

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http://dx.doi.org/10.1097/WCO.0000000000000686DOI Listing
March 2019
2 Reads

Results of robotic thymectomy performed in myasthenia gravis patients older than 60 years at onset.

Ann Thorac Surg 2019 Mar 15. Epub 2019 Mar 15.

Department of Surgery, Competence Center of Thoracic Surgery, Charité University Hospital Berlin, Charitéplatz 1, 10117 Berlin, Germany. Electronic address:

Background: Data are limited on the safety and efficacy of robotic thymectomy in myasthenia gravis (MG) patients older than 60 years at onset.

Methods: Patients older than 60 years at MG onset who underwent robotic thymectomy in Charite Universitaetsmedizin Berlin between 2003 and 2017 were potentially eligible for inclusion. The main outcomes were perioperative complications and clinical outcome according to the MG Foundation of America Post-Intervention Status (MGFA-PIS). Read More

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http://dx.doi.org/10.1016/j.athoracsur.2019.02.016DOI Listing
March 2019
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3.849 Impact Factor

The placebo response in myasthenia gravis assessed by quantitative myasthenia gravis score: A meta-analysis.

Muscle Nerve 2019 Mar 18. Epub 2019 Mar 18.

Department of Neuroscience, University of Turin Medical School, Corso Raffaello 30, 10125 Turin, Italy.

Introduction: This meta-analysis investigates the placebo response in generalized myasthenia gravis (MG) trials by means of Quantitative Myasthenia Gravis (QMG) scores.

Methods: PubMed, Scopus, Web of Science, Cochrane Controlled Trial Register, and EMBASE were searched. QMG score, dropouts rate, adverse events (AEs), and AEs responsible for dropouts were examined, together with treatment moderators. Read More

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http://dx.doi.org/10.1002/mus.26469DOI Listing
March 2019
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MuSK myasthenia gravis monoclonal antibodies: Valency dictates pathogenicity.

Neurol Neuroimmunol Neuroinflamm 2019 May 21;6(3):e547. Epub 2019 Feb 21.

Department of Neurology (M.G.H., Y.E.F.-G., I.E.v.E., J.J.P., J.J.V.), Department of Human Genetics (M.G.H., D.L.V., Y.F.-G., I.E.v.E., S.M.v.d.M.), Department of Hematology (M.T.K., H.V.), and Department of Rheumatology (L.M.S.), Leiden University Medical Center, The Netherlands.

Objective: To isolate and characterize muscle-specific kinase (MuSK) monoclonal antibodies from patients with MuSK myasthenia gravis (MG) on a genetic and functional level.

Methods: We generated recombinant MuSK antibodies from patient-derived clonal MuSK-specific B cells and produced monovalent Fab fragments from them. Both the antibodies and Fab fragments were tested for their effects on neural agrin-induced MuSK phosphorylation and acetylcholine receptor (AChR) clustering in myotube cultures. Read More

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http://dx.doi.org/10.1212/NXI.0000000000000547DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6410930PMC
May 2019
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Using Whole Genome Sequencing in an African Subphenotype of Myasthenia Gravis to Generate a Pathogenetic Hypothesis.

Front Genet 2019 1;10:136. Epub 2019 Mar 1.

Neurology Research Group, Division of Neurology, Department of Medicine, University of Cape Town, Cape Town, South Africa.

Myasthenia gravis (MG) is a rare, treatable antibody-mediated disease which is characterized by muscle weakness. The pathogenic antibodies are most frequently directed at the acetylcholine receptors (AChRs) at the skeletal muscle endplate. An ophthalmoplegic subphenotype of MG (OP-MG), which is characterized by treatment resistant weakness of the extraocular muscles (EOMs), occurs in a proportion of myasthenics with juvenile symptom onset and African genetic ancestry. Read More

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http://dx.doi.org/10.3389/fgene.2019.00136DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6406016PMC

Review of paraneoplastic syndromes in children.

Pediatr Radiol 2019 Apr 16;49(4):534-550. Epub 2019 Mar 16.

Department of Radiology, Boston Children's Hospital, 300 Longwood Ave., Boston, MA, 02215, USA.

Paraneoplastic syndromes are defined as clinical syndromes that are not related to direct tumor invasion or compression but are secondary to tumor secretion of functional peptides/hormones or related to immune cross-reactivity with normal host tissue. Paraneoplastic syndromes have a wide range of presentations and can present before the primary malignancy or tumor recurrence is diagnosed. They can mimic non-neoplastic processes, making detection, diagnosis and treatment difficult. Read More

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http://dx.doi.org/10.1007/s00247-019-04371-yDOI Listing
April 2019
5 Reads

Application of Plasma Exchange in Steroid-Responsive Encephalopathy.

Front Immunol 2019 27;10:324. Epub 2019 Feb 27.

Chongqing Key Laboratory of Neurology, Department of Neurology, the First Affiliated Hospital of Chongqing Medical University, Chongqing, China.

Plasma exchange has been widely used in autoimmune neurological diseases and is the standard treatment for myasthenia gravis crisis and Guillain-Barre syndrome. A growing body of research suggests that, in the clinical application of steroid-responsive encephalopathy, such as for Hashimoto's encephalopathy, limbic encephalitis, systemic lupus erythematosus encephalopathy, ANCA-associated vasculitis encephalopathy, and acute disseminated encephalomyelitis, plasma exchange is a safe, and effective option when steroids or other immunosuppressive therapies are ineffective in the short term or when contraindications are present. Additionally, plasma exchange can also be used alone or in combination with steroids, immunoglobulins, or other immunosuppressive agents to treat steroid-responsive encephalopathy. Read More

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http://dx.doi.org/10.3389/fimmu.2019.00324DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6400967PMC
February 2019
1 Read