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https://www.readbyqxmd.com/read/29342314/fatigue-is-a-relevant-outcome-in-patients-with-myasthenia-gravis
#1
Christopher Tran, Vera Bril, Hans D Katzberg, Carolina Barnett
INTRODUCTION: Patients with myasthenia gravis often experience fatigue, but its effect on quality of life (QoL) is underestimated, and fatigue is rarely measured in clinical trials. METHODS: 257 myasthenic patients completed the Neuro-QoL-Fatigue and measures of disease severity and QoL. We studied the relationship between fatigue, clinical, and demographic variables. Finally, we studied the responsiveness of the Neuro-QoL-Fatigue in 95 patients receiving treatments for myasthenia, and estimated the minimal important difference (MID)...
January 17, 2018: Muscle & Nerve
https://www.readbyqxmd.com/read/29332184/neurologic-complications-of-immune-checkpoint-inhibitors
#2
Avi Fellner, Chen Makranz, Michal Lotem, Felix Bokstein, Alisa Taliansky, Shai Rosenberg, Deborah T Blumenthal, Jacob Mandel, Suzana Fichman, Elena Kogan, Israel Steiner, Tali Siegal, Alexander Lossos, Shlomit Yust-Katz
Immune checkpoint inhibitors (ICPIs) have recently emerged as a novel treatment for cancer. These agents, transforming the field of oncology, are not devoid of toxicity and cause immune-related side effects which can involve any organ including the nervous system. In this study, we present 9 patients (7 men and 2 women) with neurologic complications secondary to ICPI treatment. These included meningoencephalitis, limbic encephalitis, polyradiculitis, cranial polyneuropathy, myasthenic syndrome and myositis...
January 13, 2018: Journal of Neuro-oncology
https://www.readbyqxmd.com/read/29316835/clinical-outcome-and-predictive-factors-of-postoperative-myasthenic-crisis-in-173-thymomatous-myasthenia-gravis-patients-methodological-issues
#3
Ahad Ashrafi-Asgarabad, Saeid Safiri
No abstract text is available yet for this article.
January 10, 2018: International Journal of Neuroscience
https://www.readbyqxmd.com/read/29315608/clinical-and-research-strategies-for-limb-girdle-congenital-myasthenic-syndromes
#4
REVIEW
Emily O'Connor, Ana Töpf, René Zahedi, Sally Spendiff, Daniel Cox, Andreas Roos, Hanns Lochmüller
Congenital myasthenic syndromes (CMS) are a group of rare disorders that cause fatigable muscle weakness due to defective signal transmission at the neuromuscular junction, a specialized synapse between peripheral motor neurons and their target muscle fibers. There are now over 30 causative genes that have been reported for CMS. Of these, there are 10 that are associated with a limb-girdle pattern of muscle weakness and are thus classed as LG-CMS. Next-generation sequencing and advanced methods of data sharing are likely to uncover further genes that are associated with similar clinical phenotypes, contributing to better diagnosis and effective treatment of LG-CMS patients...
January 5, 2018: Annals of the New York Academy of Sciences
https://www.readbyqxmd.com/read/29315390/probable-vancomycin-induced-myasthenic-crisis-a-case-report
#5
Cynthia T Nguyen, John Nanfro
No abstract text is available yet for this article.
January 5, 2018: Journal of Antimicrobial Chemotherapy
https://www.readbyqxmd.com/read/29313588/paraneoplastic-inverse-myasthenic-syndrome-as-a-presentation-of-bronchogenic-carcinoma
#6
G S Chowdhary, Malav Jhala
Tumours may produce growth factors and cytokines responsible for signs and symptoms distant to the primary or metastatic site. This may be the first sign of a malignancy and its recognition may be critical for early cancer detection. Moreover, proper diagnosis spares the patient of extensive and expensive search for an alternate cause of the neurological dysfunction. In neurological paraneoplastic syndromes like Lambert Eaton Myasthenic syndrome associated with small cell lung cancer, evidence of autoimmunity against presynaptic neuro-muscular junction by anti voltage gated calcium channel anti bodies is well documented...
September 2017: Journal of the Association of Physicians of India
https://www.readbyqxmd.com/read/29311015/tubular-aggregates-in-congenital-myasthenic-syndrome
#7
Amalia Feresiadou, Olivera Casar-Borota, Anca Dragomir, Carola Hedberg Oldfors, Erik Stålberg, Anders Oldfors
No abstract text is available yet for this article.
November 24, 2017: Neuromuscular Disorders: NMD
https://www.readbyqxmd.com/read/29307077/analysis-of-ct-features-and-quantitative-texture-analysis-in-patients-with-thymic-tumors-correlation-with-grading-and-staging
#8
Angelo Iannarelli, Beatrice Sacconi, Francesca Tomei, Marco Anile, Flavia Longo, Mario Bezzi, Alessandro Napoli, Luca Saba, Michele Anzidei, Giulia D'Ovidio, Roberto Scipione, Carlo Catalano
OBJECTIVES: To evaluate potential relationship between qualitative CT features, quantitative texture analysis (QTA), histology, WHO staging, Masaoka classification and myasthenic syndrome in patients with thymic tumors. MATERIALS AND METHODS: Sixteen patients affected by histologically proven thymic tumors were retrospectively included in the study population. Clinical information, with special regard to myasthenic syndrome and serological positivity of anti-AchR antibodies, were recorded...
January 6, 2018: La Radiologia Medica
https://www.readbyqxmd.com/read/29249499/spontaneous-ventilation-thoracoscopic-thymectomy-without-muscle-relaxant-for-myasthenia-gravis-comparison-with-standard-thoracoscopic-thymectomy
#9
Long Jiang, Lieven Depypere, Gaetano Rocco, Jin-Shing Chen, Jun Liu, Wenlong Shao, Hanyu Yang, Jianxing He
OBJECTIVES: Myasthenia gravis (MG) benefits from thymectomy. However, its unpredictable response to muscle relaxants and volatile anesthetic agents may result in muscle weakness and subsequently in postoperative myasthenic crisis. The aim of this study was to determine the surgical outcomes after spontaneous ventilation compared with conventional intubated video-assisted thoracoscopic thymectomy (spontaneous-ventilation video-assisted thoracic thymectomy [SV-VATT] vs intubated video-assisted thoracic thymectomy) in patients with MG...
November 21, 2017: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/29225302/role-of-surgery-in-the-treatment-of-masaoka-stage-iva-thymoma
#10
Erkan Kaba, Berker Ozkan, Suat Erus, Salih Duman, Berk Cimenoglu, Alper Toker
PURPOSE: To analyze the role of surgery in patients with Masaoka stage IVa thymoma treated with multimodality therapy. METHODS: Of 191 patients undergoing surgery for thymoma in our department between January 2002 and December 2015, 39 (20.4%) had Masaoka stage IVa. Histopathological tumor type, myasthenic status of the Osserman-Genkins score, Masaoka stage at the first surgery, neoadjuvant treatment, number and type of surgeries, and survival rates were recorded...
December 8, 2017: Annals of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/29221974/criteria-for-postoperative-mechanical-ventilation-after-thymectomy-in-patients-with-myasthenia-gravis-a-retrospective-analysis
#11
Keerthi Chigurupati, Shrinivas Gadhinglajkar, Rupa Sreedhar, Muraleedharan Nair, Madathipat Unnikrishnan, Manjusha Pillai
OBJECTIVE: To determine the criteria for postoperative mechanical ventilation after thymectomy in patients with Myasthenia Gravis. DESIGN: Retrospective study. SETTING: Teritiary care centre. PARTICIPANTS: 77 Myasthenia gravis patients operated for thymectomy were studied. INTERVENTIONS: After obtaining clearance from Institutional ethics committee, medical records of 77 patients with MG, who were operated for thymectomy between January 2005 and December 2015 were reviewed in a retrospective manner...
June 27, 2017: Journal of Cardiothoracic and Vascular Anesthesia
https://www.readbyqxmd.com/read/29221139/epstein-barr-virus-in-tumor-infiltrating-b-cells-of-myasthenia-gravis-thymoma-an-innocent-bystander-or-an-autoimmunity-mediator
#12
Paola Cavalcante, Stefania Marcuzzo, Sara Franzi, Barbara Galbardi, Lorenzo Maggi, Teresio Motta, Raffaella Ghislandi, Antonella Buzzi, Luisella Spinelli, Lorenzo Novellino, Fulvio Baggi, Carlo Antozzi, Fabio Conforti, Tommaso Martino De Pas, Massimo Barberis, Pia Bernasconi, Renato Mantegazza
The thymus plays a key role in myasthenia gravis (MG), a B cell-mediated autoimmune disorder affecting neuromuscular junction. Most MG patients have thymic abnormalities, including hyperplasia and thymoma, a neoplasm of thymic epithelial cells. Epstein-Barr virus (EBV) is associated with autoimmune diseases and tumors. Recently, we showed EBV persistence and reactivation in hyperplastic MG thymuses, suggesting that EBV might contribute to intra-thymic B cell dysregulation in MG patients. Here, we investigated EBV involvement in thymoma-associated MG, by searching for EBV markers in MG (n=26) and non-MG (n=14) thymomas...
November 10, 2017: Oncotarget
https://www.readbyqxmd.com/read/29205338/s1p-receptor-antagonists-fingolimod-and-siponimod-do-not-improve-the-outcome-of-experimental-autoimmune-myasthenia-gravis-mice-after-disease-onset
#13
Andreas Pelz, Hanne Schaffert, Radharani Diallo, Falk Hiepe, Andreas Meisel, Siegfried Kohler
Myasthenia gravis (MG) is an autoimmune disease characterized by muscle weakness and fatigue in the presence of circulating antibodies against components of the neuromuscular junction. Most patients have a good prognosis, but some are refractory to standard-of-care immunosuppressive treatment and suffer from recurrent myasthenic crises. Functional sphingosine-1-phosphate (S1P) antagonists like fingolimod and siponimod (BAF312) are successfully used for the treatment of multiple sclerosis, and fingolimod was shown to prevent the development of myasthenic symptoms in experimental autoimmune myasthenia gravis (EAMG), the standard model of MG...
December 4, 2017: European Journal of Immunology
https://www.readbyqxmd.com/read/29200116/paraneoplastic-disorders
#14
Eric Lancaster
PURPOSE OF REVIEW: Paraneoplastic neurologic syndromes target specific areas of the nervous system with pathogenic autoantibodies or T-cell responses. Each syndrome conveys a risk of particular tumors. Expanded paraneoplastic antibody testing has led to improved diagnosis but created challenges involving appropriate interpretation of test results. RECENT FINDINGS: Peripheral nervous system paraneoplastic disorders such as myasthenia gravis and Lambert-Eaton myasthenic syndrome involve pathogenic autoantibodies...
December 2017: Continuum: Lifelong Learning in Neurology
https://www.readbyqxmd.com/read/29195055/neuromuscular-junction-formation-aging-and-disorders
#15
Lei Li, Wen-Cheng Xiong, Lin Mei
Synapses, the fundamental unit in neuronal circuits, are critical for learning and memory, perception, thinking, and reaction. The neuromuscular junction (NMJ) is a synapse formed between motoneurons and skeletal muscle fibers that is wrapped by Schwann cells (SCs). It is essential for controlling muscle contraction. NMJ formation requires intimate interactions among motoneurons, muscles, and SCs. Deficits in NMJ formation and maintenance cause neuromuscular disorders, including congenital myasthenic syndrome and myasthenia gravis...
December 1, 2017: Annual Review of Physiology
https://www.readbyqxmd.com/read/29191521/the-possibility-of-obtaining-marketing-authorization-of-orphan-pharmaceutical-compounding-preparations-3-4-dap-for-lambert-eaton-myasthenic-syndrome
#16
Sofieke de Wilde, Maria G H de Jong, Alexander F Lipka, Henk-Jan Guchelaar, Kirsten J M Schimmel
BACKGROUND: Pharmaceutical compounding preparations, produced by (hospital) pharmacies, usually do not have marketing authorization. As a consequence, some of these pharmaceutical compounding preparations can be picked-up by a pharmaceutical company to obtain marketing authorization, often leading to price increases. An example is the 3,4-diaminopyridine slow release (3,4-DAP SR) tablets for Lambert-Eaton Myasthenic Syndrome (LEMS). In 2009 marketing authorization was given for the commercial immediate release phosphate salt of the drug, including a fifty-fold price increase compared to the pharmaceutical compounding preparation...
November 27, 2017: European Journal of Pharmaceutical Sciences
https://www.readbyqxmd.com/read/29189923/congenital-myasthenic-syndrome-with-episodic-apnoea-clinical-neurophysiological-and-genetic-features-in-the-long-term-follow-up-of-19-patients
#17
Grace McMacken, Roger G Whittaker, Teresinha Evangelista, Angela Abicht, Marina Dusl, Hanns Lochmüller
BACKGROUND: Congenital myasthenic syndrome with episodic apnoea (CMS-EA) is a rare but potentially treatable cause of apparent life-threatening events in infancy. The underlying mechanisms for sudden and recurrent episodes of respiratory arrest in these patients are unclear. Whilst CMS-EA is most commonly caused by mutations in CHAT, the list of associated genotypes is expanding. METHODS: We reviewed clinical information from 19 patients with CMS-EA, including patients with mutations in CHAT, SLC5A7 and RAPSN, and patients lacking a genetic diagnosis...
November 30, 2017: Journal of Neurology
https://www.readbyqxmd.com/read/29189554/paraneoplastic-lambert-eaton-myasthenic-syndrome-with-limbic-encephalitis-clinical-correlation-with-the-coexistence-of-anti-vgcc-and-anti-gabab-receptor-antibodies
#18
Jonathan J Cho, James P Wymer
OBJECTIVE: To characterize Lambert-Eaton myasthenic syndrome and limbic encephalitis with coexistent voltage-gated calcium channel (VGCC) antibody and γ-aminobutyric acid (GABA) B receptor antibody. METHODS: Case study. RESULTS: A 57-year-old man presented with 6 months of weakness, unsteadiness, and vision difficulties. Examination revealed proximal weakness and diminished reflexes. Electrodiagnostic study revealed low-amplitude motor potentials and facilitation on high-frequency stimulation...
December 2017: Journal of Clinical Neuromuscular Disease
https://www.readbyqxmd.com/read/29189551/distinguishing-features-of-the-repetitive-nerve-stimulation-test-between-lambert-eaton-myasthenic-syndrome-and-myasthenia-gravis-50-year-reappraisal
#19
Shin J Oh
OBJECTIVE: To reappraise the distinguishing features of the repetitive nerve stimulation (RNS) tests in the abductor digiti quinti muscle between myasthenia gravis (MG) and Lambert-Eaton myasthenic syndrome (LEMS) 50 years after the 1965's Lambert seminal paper. METHODS: The various parameters of the RNS test were compared between 34 patients with LEMS and 140 patients with MG to assess their diagnostic sensitivity. RESULTS: RNS test was abnormal in all (100%) patients with LEMS and 76 (54%) patients with MG...
December 2017: Journal of Clinical Neuromuscular Disease
https://www.readbyqxmd.com/read/29156018/risk-factors-of-myasthenic-crisis-after-thymectomy-for-thymoma-patients-with-myasthenia-gravis
#20
Liang Xue, Lin Wang, Jihong Dong, Yunfeng Yuan, Hong Fan, Yi Zhang, Qun Wang, Jianyong Ding
OBJECTIVES: Total thymectomy should be performed on thymoma patients with myasthenia gravis. The aim of the present study was to investigate the risk factors of postoperative myasthenic crisis (POMC) occurrence in these patients. METHODS: The clinical records of 127 thymoma patients with myasthenia gravis (68 men, 59 women; median age, 50 years) who underwent total thymectomy at our institution from 2005 to 2014 were retrospectively reviewed. The following factors were analysed in relation to POMC: gender, age, duration of symptoms, bulbar symptoms, smoking history, history of myasthenic crisis, comorbidities, perioperative pyridostigmine and prednisolone therapy, spirometric and blood gas parameters, Osserman stage, operation approach, major complications, World Health Organization (WHO) histologic classification, Masaoka stage and use of immunoglobulins or plasmapheresis...
October 1, 2017: European Journal of Cardio-thoracic Surgery
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