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Myasthenia gravis

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https://www.readbyqxmd.com/read/28433474/beneficial-effect-of-tocilizumab-in-myasthenia-gravis-refractory-to-rituximab
#1
Dagur Ingi Jonsson, Ritva Pirskanen, Fredrik Piehl
Muscle fatigue associated with myasthenia gravis is caused by autoantibodies interfering with neuromuscular transmission. Immunomodulating treatment is widely used in moderate to severe myasthenia, although the use of newer biological drugs except rituximab is rare. We describe the effect of tocilizumab, a blocker of interleukin-6 signalling, in two female myasthenia patients with high titres of serum acetylcholine receptor antibodies and insufficient response to rituximab. The first patient had been treated with high dose immunoglobulins regularly for several years and the second patient had been treated both with different oral immune suppressants and immunoglobulins before testing a low dose of rituximab without significant clinical effect...
March 16, 2017: Neuromuscular Disorders: NMD
https://www.readbyqxmd.com/read/28431604/a-reappraisal-of-diagnostic-tests-for-myasthenia-gravis-in-a-large-asian-cohort
#2
Yew Long Lo, Raymond P Najjar, Kelvin Y Teo, Sharon L Tow, Jing Liang Loo, Dan Milea
BACKGROUND: Myasthenia gravis (MG) is a chronic autoimmune neuromuscular disease characterized by weakness of bodily skeletal muscles. Office-based diagnostic tests such as repetitive nerve stimulation (RNS), single fiber electromyography (SFEMG), and the ice test, are used to refine the differential clinical diagnosis of this disease. Evaluating the clinical sensitivity and specificity of these tests, however, may be confounded by lack of a gold standard, non-blinding, incorporation bias, use of non-representative populations and retrospective data...
May 15, 2017: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/28429750/correlations-of-tnf-%C3%AE-gene-promoter-polymorphisms-with-the-risk-of-thymoma-associated-myasthenia-gravis-in-a-northern-chinese-han-population
#3
H-W Yang, P Lei, Y-C Xie, Z-L Han, D Li, S-H Wang, Z-L Sun
This study was performed with the aim to investigate the correlations of tumor necrosis factor-alpha (TNF-α) gene promoter polymorphisms with the risk of thymoma-associated myasthenia gravis (T-MG) in a northern Chinese Han population. Between June 2005 and June 2015, 305 MG patients (150 males and 155 females, MG group) and 293 healthy volunteers (negative control (NC) group) were enrolled in this study. Among the MG patients, there were 121 patients with thymoma-associated MG (T-MG group) and 184 without T-MG (NT-MG group)...
April 21, 2017: Cancer Gene Therapy
https://www.readbyqxmd.com/read/28429039/-true-thymic-hyperplasia-differential-diagnosis-of-thymic-mass-lesions-in-neonates-and-children
#4
C-A Weis, B Märkl, T Schuster, K Vollert, P Ströbel, A Marx
Reactive and neoplastic thymic pathologies are the main considerations in the case of masses in the anterior and middle part of the mediastinum, while neurogenic tumors are predominant in the posterior mediastinum (which are not dealt with here). In neonates and infants, the commonest pathologies in the anterior mediastinum comprise germ cell tumors (mainly teratomas), congenital thymic cysts and true thymic hyperplasia (TTH). In toddlers, teratomas, yolk sac tumors and cysts predominate. In children over 5 years of age, lymphomas are the commonest mass lesions whereas thymomas and thymic carcinomas are rare...
April 20, 2017: Der Pathologe
https://www.readbyqxmd.com/read/28428630/hinge-deleted-igg4-blocker-therapy-for-acetylcholine-receptor-myasthenia-gravis-in-rhesus-monkeys
#5
Mario Losen, Aran F Labrijn, Vivianne H van Kranen-Mastenbroek, Maarten L Janmaat, Krista G Haanstra, Frank J Beurskens, Tom Vink, Margreet Jonker, Bert A 't Hart, Marina Mané-Damas, Peter C Molenaar, Pilar Martinez-Martinez, Eline van der Esch, Janine Schuurman, Marc H de Baets, Paul W H I Parren
Autoantibodies against ion channels are the cause of numerous neurologic autoimmune disorders. Frequently, such pathogenic autoantibodies have a restricted epitope-specificity. In such cases, competing antibody formats devoid of pathogenic effector functions (blocker antibodies) have the potential to treat disease by displacing autoantibodies from their target. Here, we have used a model of the neuromuscular autoimmune disease myasthenia gravis in rhesus monkeys (Macaca mulatta) to test the therapeutic potential of a new blocker antibody: MG was induced by passive transfer of pathogenic acetylcholine receptor-specific monoclonal antibody IgG1-637...
April 20, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28424453/comorbid-human-immunodeficiency-virus-hiv-and-muscle-specific-kinase-musk-myasthenia-gravis-a-case-report-and-literature-review
#6
Michael Sherpa, Ravi K Metai, Viki Kumar, Tinu Hirachan, Kawser U Ahmed, Sharon J Atkinson
BACKGROUND HIV infections with concomitant immunologically-mediated disorders have been frequently described but there has been little research on the association between HIV and myasthenia gravis. MuSK myasthenia gravis coexisting with HIV is an even a rarer entity and can occur as a part of immune restoration disease. We report the case of a patient with asymptomatic HIV infection who presented with new-onset MuSK myasthenia gravis. CASE REPORT A 44-year-old African-American woman with HIV since 2004 and on highly active antiretroviral therapy (HAART) presented to the ED with complains of double vision and difficulty swallowing for 2 weeks...
April 20, 2017: American Journal of Case Reports
https://www.readbyqxmd.com/read/28421357/incidence-of-autoimmune-diseases-in-patients-with-scabies-a-nationwide-population-based-study-in-taiwan
#7
Jui-Ming Liu, Feng-Hsiang Chiu, Chien-Yu Lin, Fung-Wei Chang, Ren-Jun Hsu
Scabies is a commonly occurring infectious immune-mediated inflammatory skin disease. Immune-mediated inflammatory processes are also observed in autoimmune diseases. There have been very few previous studies; however, that have investigated the possible association between scabies and autoimmune diseases. To address this research gap, we conducted a nationwide population-based cohort study that included a total of 4481 scabies patients and 16,559 control subjects matched by gender, age, insured region, urbanization and income...
April 18, 2017: Rheumatology International
https://www.readbyqxmd.com/read/28420327/clinical-predictors-for-the-prognosis-of-myasthenia-gravis
#8
Lili Wang, Yun Zhang, Maolin He
BACKGROUND: Clinical predictors for myasthenia gravis relapse and ocular myasthenia gravis secondary generalization during the first two years after disease onset remain incompletely identified. This study attempts to investigate the clinical predictors for the prognosis of Myasthenia Gravis. METHODS: Eighty three patients with myasthenia gravis were concluded in this study. Baseline characteristics were analyzed as predictors. RESULTS: Relapse of myasthenia gravis developed in 26 patients (34%)...
April 19, 2017: BMC Neurology
https://www.readbyqxmd.com/read/28416933/thymectomy-in-myasthenia-gravis
#9
REVIEW
Yener Aydin, Ali Bilal Ulas, Vahit Mutlu, Abdurrahim Colak, Atilla Eroglu
In recent years, thymectomy has become a widespread procedure in the treatment of myasthenia gravis (MG). Likelihood of remission was highest in preoperative mild disease classification (Osserman classification 1, 2A). In absence of thymoma or hyperplasia, there was no relationship between age and gender in remission with thymectomy. In MG treatment, randomized trials that compare conservative treatment with thymectomy have started, recently. As with non-randomized trials, remission with thymectomy in MG treatment was better than conservative treatment with only medication...
February 2017: Eurasian Journal of Medicine
https://www.readbyqxmd.com/read/28415886/post-operative-delayed-ambulation-after-thymectomy-is-associated-withpre-operative-six-minute-walk-distance
#10
Kazuhiro Hayashi, Koichi Fukumoto, Kohei Yokoi, Motoki Nagaya, Takayuki Inoue, Satoru Ito, Hiroki Nakajima, Keiko Hattori, Izumi Kadono, Yoshihiro Nishida
OBJECTIVE: Delayed post-operative ambulation is a risk of post-operative complications and increases overall healthcare costs. We investigated pre-operative and intraoperative variables associated with delayed ambulation in patients who underwent thymectomy. METHODS: A total of 57 consecutive patients undergoing thymectomy were included in this study. Pre-operative functional exercise capacity was evaluated by six-minute walk distance. Ambulation was considered to be delayed if the patient could not walk the ward on post-operative day 1...
April 17, 2017: Disability and Rehabilitation
https://www.readbyqxmd.com/read/28414153/inflammatory-myopathy-associated-with-myasthenia-gravis-with-and-without-thymic-pathology-report-of-four-cases-and-literature-review
#11
REVIEW
Ernestina Santos, Ester Coutinho, Ana Martins da Silva, António Marinho, Carlos Vasconcelos, Ricardo Taipa, Manuel Melo Pires, Guilherme Gonçalves, Carlos Lopes, Maria Isabel Leite
INTRODUCTION: the association of myasthenia gravis (MG) and inflammatory myopathy is rare and often only one of the diseases is diagnosed. Thymus pathology may be in the origin of such disease association. METHODS: we described four patients with both MG and inflammatory myopathy. RESULTS: these cases correspond to 2.3% of our MG cohort. Case 1: MG, polymyositis and thymolipoma; case 2: MG and necrotizing myopathy without thymic pathology on a background of scleroderma, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, telangiectasia (CREST); case 3: MG and dermatomyositis without thymic pathology; case 4: MG and dermatomyositis with type C thymoma...
April 13, 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/28413704/diversity-in-mental-fatigue-and-social-profile-of-patients-with-myasthenia-gravis-in-two-different-northern-european-countries
#12
COMPARATIVE STUDY
Liis Sabre, Elisabet Westerberg, Maarika Liik, Anna R Punga
Self-estimated health can be used for comparison of different diseases between countries. It is important to elaborate on whether disparities in self-estimated health are due to disease-specific parameters or socioeconomic differences. In this study, we aimed at evaluating clinical and social similarities and differences in myasthenia gravis (MG) patients between comparable regions in two Baltic Sea countries, Estonia and Sweden. METHODS: This cross-sectional study included southern counties in Sweden and Estonia of comparable size...
April 2017: Brain and Behavior
https://www.readbyqxmd.com/read/28405609/preconditioned-mesenchymal-stem-cells-treat-myasthenia-gravis-in-a-humanized-preclinical-model
#13
Muriel Sudres, Marie Maurer, Marieke Robinet, Jacky Bismuth, Frédérique Truffault, Diane Girard, Nadine Dragin, Mohamed Attia, Elie Fadel, Nicola Santelmo, Camille Sicsic, Talma Brenner, Sonia Berrih-Aknin
Myasthenia gravis (MG) with anti-acetylcholine receptor (AChR) Abs is an autoimmune disease characterized by severe defects in immune regulation and thymic inflammation. Because mesenchymal stem cells (MSCs) display immunomodulatory features, we investigated whether and how in vitro-preconditioned human MSCs (cMSCs) could treat MG disease. We developed a new humanized preclinical model by subcutaneously grafting thymic MG fragments into immunodeficient NSG mice (NSG-MG model). Ninety percent of the animals displayed human anti-AChR Abs in the serum, and 50% of the animals displayed MG-like symptoms that correlated with the loss of AChR at the muscle endplates...
April 6, 2017: JCI Insight
https://www.readbyqxmd.com/read/28401300/the-role-of-thymectomy-in-the-treatment-of-juvenile-myasthenia-gravis-a-systematic-review
#14
Arin L Madenci, George Z Li, Brent R Weil, David Zurakowski, Peter B Kang, Christopher B Weldon
BACKGROUND: The role of thymectomy in the treatment of juvenile myasthenia gravis (JMG) is poorly defined. The objective of this systematic review was to evaluate the effect of thymectomy on survival, disease severity, and peri-operative complications for patients with JMG. METHODS: A search of MEDLINE, EMBASE, and the Cochrane Library (1/1/2000-3/1/2016) identified all English language, human studies of thymectomy for JMG. The population was patients with JMG age ≤18 years who underwent thymectomy (comparator group was unexposed to thymectomy)...
April 11, 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/28390639/a-woman-in-her-70s-with-a-history-of-myasthenia-gravis-complains-of-shortness-of-breath
#15
Craig Fryman, Sahar Ahmad
No abstract text is available yet for this article.
April 2017: Chest
https://www.readbyqxmd.com/read/28382612/clinical-profile-and-outcome-of-myasthenic-crisis-in-central-taiwan
#16
Yi-Sin Wong, Cheung-Ter Ong, Sheng-Feng Sung, Chi-Shun Wu, Yung-Chu Hsu, Yu-Hsiang Su, Ling-Chien Hung
BACKGROUND: Myasthenia gravis (MG) is an autoimmune disease caused by antibodies to acetylcholine receptors of the skeletal muscle. Myasthenic crisis (MC) is a complication observed during both early and late stage MG cases. In this study, we examined current treatments and three years outcomes in patients with MG and MC. We also investigated the impact of thymectomy and systemic lupus erythematosus (SLE) in patients with MG and MC. METHODS: In this retrospective study, we reviewed the medical records of all patients admitted to one teaching hospital between January 2006 and December 2014 and identified those for whom discharge diagnosis included the International Classification of Diseases, ninth revision (ICD-9) codes corresponding to MG (358...
December 15, 2016: Acta Neurologica Taiwanica
https://www.readbyqxmd.com/read/28382272/pure-red-cell-aplasia-associated-with-good-syndrome
#17
Masayuki Okui, Takashi Yamamichi, Ayaka Asakawa, Masahiko Harada, Hirotoshi Horio
Pure red cell aplasia (PRCA) and hypogammaglobulinemia are paraneoplastic syndromes that are rarer than myasthenia gravis in patients with thymoma. Good syndrome coexisting with PRCA is an extremely rare pathology. We report the case of a 50-year-old man with thymoma and PRCA associated with Good syndrome who achieved complete PRCA remission after thymectomy and postoperative immunosuppressive therapy, and provide a review of the pertinent literature.
April 2017: Korean Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/28381664/therapeutic-potential-of-docetaxel-plus-cisplatin-chemotherapy-for-myasthenia-gravis-patients-with-metastatic-thymoma
#18
Guoyan Qi, Peng Liu, Huimin Dong, Shanshan Gu, Hongxia Yang, Yinping Xue
The prognosis of myasthenia gravis (MG) in association with invasive or metastatic thymoma is usually worse, and therapeutic options are quite limited. Here, we retrospectively reported the therapeutic effect of docetaxel plus cisplatin (docetaxel/cisplatin) chemotherapy in 7 MG patients with metastatic thymoma. Previously, all patients underwent thymectomy at the first onset of thymoma. After the metastasis of thymoma, none of the patients received thymectomy due to unresectable conditions after surgeon's evaluation for great risk of myasthenic crisis (n = 5) or patients' refusal (n = 2)...
April 2017: Tohoku Journal of Experimental Medicine
https://www.readbyqxmd.com/read/28379106/detectable-voice-change-with-the-edrophonium-test-in-laryngeal-myasthenia-gravis
#19
Koichi Tsunoda, Yoko Fujimaki, Yoko Morita
A case of laryngeal myasthenia gravis in a 65-year-old woman presenting with hoarseness as the sole symptom is reported. Voice spectrography was performed before and after injection of intravenous edrophonium. There was a marked improvement in the patient's voice after the administration of edrophonium, which was confirmed by the changes seen on the sound spectrogram. This was the only objective indication of a diagnosis of myasthenia gravis. No thymoma was seen on chest X-ray and the patient was negative for anti-acetylcholine receptor antibodies...
January 1, 2017: Journal of International Medical Research
https://www.readbyqxmd.com/read/28375749/immunization-with-recombinantly-expressed-lrp4-induces-experimental-autoimmune-myasthenia-gravis-in-c57bl-6-mice
#20
Canan Ulusoy, Filiz Çavuş, Vuslat Yılmaz, Erdem Tüzün
BACKGROUND: Myasthenia gravis (MG) is an autoimmune disease of the neuromuscular junction (NMJ), characterized with muscle weakness. While MG develops due to acetylcholine receptor (AChR) antibodies in most patients, antibodies to muscle-specific receptor tyrosine kinase (MuSK) or low-density lipoprotein receptor-related protein 4 (LRP4) may also be identified. Experimental autoimmune myasthenia gravis (EAMG) has been previously induced by both LRP4 immunization and passive transfer of LRP4 antibodies...
April 4, 2017: Immunological Investigations
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