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Dario Amore, Roberto Scaramuzzi, Davide Di Natale, Carlo Curcio
The advantages of thymectomy as part of the treatment of myasthenia gravis has been demonstrated repeatedly in the literature. Both single-institution and multi-institution trials have shown robotic thymectomy to be safe, feasible and associated with better early clinical outcomes than the trans-sternal approach. Most reports have also documented the superiority of robotic technology in the dissection of the superior mediastinum over conventional thoracoscopy, thanks to instruments with more degrees of movement and freedom...
2018: Journal of Visualized Surgery
Marco Morsch, Dario A Protti, Delfine Cheng, Filip Braet, Roger S Chung, Stephen W Reddel, William D Phillips
Cannabinoids exert dynamic control over many physiological processes including memory formation, cognition and pain perception. In the central nervous system endocannabinoids mediate negative feedback of quantal transmitter release following postsynaptic depolarization. The influence of cannabinoids in the peripheral nervous system is less clear and might have broad implications for the therapeutic application of cannabinoids. We report a novel cannabinoid effect upon the mouse neuromuscular synapse: acutely increasing synaptic vesicle volume and raising the quantal amplitudes...
March 16, 2018: Scientific Reports
Vasiliki Zouvelou, Efstratios Karavasilis, Georgios Velonakis
No abstract text is available yet for this article.
February 26, 2018: Neuromuscular Disorders: NMD
Xin Huang, Yingkai Li, Huiyu Feng, Pei Chen, Weibin Liu
Objectives: To describe the clinical profile, clinical outcomes and factors that may affect the outcome of juvenile myasthenia gravis (JMG) patients in southern China. Methods: We reviewed information relating to JMG patients treated and evaluated at the First Affiliated Hospital, Sun Yat-sen University, between 1998 and 2015. The study involved 327 JMG patients who had been followed up for ≥1 year. Results: Overall, 77.4% patients showed initial symptoms in the prepubertal period (<12 years)...
2018: Frontiers in Neurology
Carles Galdeano, Nicolas Coquelle, Monika Cieslikiewicz-Bouet, Manuela Bartolini, Belén Pérez, M Victòria Clos, Israel Silman, Ludovic Jean, Jacques-Philippe Colletier, Pierre-Yves Renard, Diego Muñoz-Torrero
Symptomatic treatment of myasthenia gravis is based on the use of peripherally-acting acetylcholinesterase (AChE) inhibitors that, in some cases, must be discontinued due to the occurrence of a number of side-effects. Thus, new AChE inhibitors are being developed and investigated for their potential use against this disease. Here, we have explored two alternative approaches to get access to peripherally-acting AChE inhibitors as new agents against myasthenia gravis, by structural modification of the brain permeable anti-Alzheimer AChE inhibitors tacrine, 6-chlorotacrine, and huprine Y...
March 11, 2018: Molecules: a Journal of Synthetic Chemistry and Natural Product Chemistry
L J Li, Y Z Guan, F Lü, Y W Song, X J Xu, Y Jiang, O Wang, W B Xia, X P Xing, M Li
Objective: To investigate the glucose and lipid metabolic disorders in patients with myasthenia gravis (MG) without glucocorticoid therapy, and the relationships between insulin, insulin resistance, muscle strength, serum levels of osteocalcin, 25-hydroxy vitamin D (25OHD) and glucose and lipid metabolism. Methods: A total of 102 MG patients [(40±11) years old, 43 males and 59 females] without glucocorticoid treatment were enrolled in this cross-sectional study. Height, weight and the handgrip of dominant hands were measured...
February 27, 2018: Zhonghua Yi Xue za Zhi [Chinese medical journal]
Sujata P Thawani, Thomas H Brannagan, Benjamin Lebwohl, Peter H R Green, Jonas F Ludvigsson
BACKGROUND: Case reports suggest there may be an association between celiac disease (CD) and myasthenia gravis (MG). METHODS: We identified 29,086 individuals with CD in Sweden from 1969 to 2008. We compared these individuals with 144,480 matched controls. Hazard ratios (HRs) for future MG (identified through ICD codes) were estimated using Cox regression. RESULTS: During 326,376 person-years of follow-up in CD patients, there were 7 MG cases (21/million person-years) compared to 22 MG cases in controls during 1,642,273 years of follow-up (14/million person-years) corresponding to a HR of 1...
March 12, 2018: BMC Neurology
Sohita Dhillon
The article Eculizumab: A Review in Generalized Myasthenia Gravis, written by Sohita Dhillon, was originally published Online First without open access.
March 9, 2018: Drugs
Tommaso Claudio Mineo, Alessandro Tamburrini, Orazio Schillaci, Vincenzo Ambrogi
BACKGROUND: Patients with thymoma and without clinical or electromyographical myasthenic signs may occasionally develop myasthenia several years after thymectomy. Hereby, we investigated the predictors and the evolution of this peculiar disease. METHODS: We performed a retrospective analysis in 104 consecutive patients undergoing thymectomy between 1987 and 2013 for thymoma without clinical nor electromyographic signs of myasthenia gravis. Predictors of post-thymectomy onset of myasthenia gravis were investigated with univariale time-to-disease analysis...
March 6, 2018: Seminars in Thoracic and Cardiovascular Surgery
W C Li, J J Ma, Z B Zhang
No abstract text is available yet for this article.
March 2, 2018: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
Z M Liu, F Fang, C H Ding, W H Zhang, J Deng, C H Chen, X Wang, J Liu, Z Li, X L Jia, J S Zeng, S Y Qian
Objective: To investigate the clinical and genetic features of congenital myasthenia syndrome with episodic apnea (CMS-EA) caused by gene mutation of choline acetyltransferase (CHAT) Methods: The clinical data of 2 patients with congenital myasthenia syndrome were collected, and both were diagnosed from 2013 to 2015 in Beijing Children's Hospital, Capital Medical University. The clinical features and gene mutation characteristics were analyzed, and the patients were followed-up for therapeutic efficacy. Results: The two patients (case 1 and case 2) had the onset soon after birth and at 3 months after birth respectively...
March 2, 2018: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
Kee Hong Park, Patrick Waters, Mark Woodhall, Bethan Lang, Thomas Smith, Jung-Joon Sung, Kwang-Kuk Kim, Young-Min Lim, Jee-Eun Kim, Byung-Jo Kim, Jin-Sung Park, Jeong-Geon Lim, Dae-Seong Kim, Ohyun Kwon, Eun Hee Sohn, Jong Seok Bae, Byung-Nam Yoon, Nam-Hee Kim, Suk-Won Ahn, Jeeyoung Oh, Hyung Jun Park, Kyong Jin Shin, Yoon-Ho Hong
Acquired myasthenia gravis (MG) is a prototype autoimmune disease of the neuromuscular junction, caused in most patients by autoantibodies to the muscle nicotinic acetylcholine receptor (AChR). There seem to be ethnic and regional differences in the frequency and clinical features of MG seronegative for the AChR antibody. This study aimed to describe the autoantibody profiles and clinical features of Korean patients with generalized MG seronegative for the AChR antibody. A total of 62 patients with a high index of clinical suspicion of seronegative generalized MG were identified from 18 centers, and we examined their sera for antibodies to clustered AChR, muscle-specific tyrosine kinase (MuSK), and low-density lipoprotein receptor-related protein 4 (LRP4) by cell-based assays (CBA) and to MuSK by radioimmunoprecipitation assay (RIPA)...
2018: PloS One
Takuya Onuki, Sho Ueda, Shinichi Otsu, Takahiro Yanagihara, Naoki Kawakami, Masatoshi Yamaoka, Masaharu Inagaki
A 34-year-old man was diagnosed with thymoma, which was evaluated preoperatively as stage II or III, with myasthenia gravis (MG). The size of the tumor was 70 × 44 × 80 mm. No invasion to neighboring organs was observed. Prednisolone was prescribed for stabilization of MG. However, a myasthenic crisis (MC) occurred, and intensive care, including emergent endobronchial intubation followed by artificial ventilation, pulse steroid therapy, high-dose intravenous immunoglobulin, and tacrolimus hydrate, was initiated...
March 7, 2018: Annals of Thoracic and Cardiovascular Surgery
Marzieh Tavakol, Seyed Alireza Mahdaviani, Mir Reza Ghaemi, Mohammad Vaezi, Atosa Dorudinia, Hamidreza Jamaati, Ali Akbar Velayati
Good's syndrome, the adult onset hypogammaglobulinemia associated with thymoma has been explained about six decades ago. It generally presents with recurrent infections and several paraneoplastic syndromes including myasthenia gravis, pure red cell aplasia, connective tissue disorders, superior vena cava, Horner's syndrome, lichen planus and inflammatory bowel disease. Lack of B cell, dysfunction of T cell, CD4+ T cell lymphopenia, reversed CD4/CD8+ T cell ratio, autoantibodies against Th17 related cytokines have been respected as the pathogenesis of the immune dysregulation this syndrome...
February 2018: Iranian Journal of Allergy, Asthma, and Immunology
Carl Johan Molin, Liis Sabre, Cleo-Aron Weis, Tanel Punga, Anna Rostedt Punga
Objective: The aim of the study was to analyze the effect of thymectomy on the proposed disease-specific microRNA (miRNA) biomarkers miR-150-5p and miR-21-5p in patients from the prospective randomized trial of thymectomy in myasthenia gravis (MGTX trial) and to evaluate the longitudinal changes in clinical patterns compared with these miRNA levels. Methods: Serum samples were obtained from 80 patients with MG who were included in the MGTX trial. Thirty-eight patients were randomized to thymectomy plus prednisone treatment, and 42 patients were randomized to prednisone treatment...
May 2018: Neurology® Neuroimmunology & Neuroinflammation
Man Amanat, Mona Salehi, Nima Rezaei
Psoriasis used to be known as a skin disorder; however, it can now be considered as a systemic disease with the involvement of multiple organs. Neurological and psychiatric disorders are some of the associated problems that can be observed in patients with psoriasis. Stroke, multiple sclerosis, seizure, migraine, restless leg syndrome, Parkinson's disease, Guillain-Barré syndrome, and myasthenia gravis are the reported neurological diseases, while depression, bipolar mood disorder, anxiety, psychosis, cognitive impairment, personality disorders, sexual disorders, sleep disturbance, and eating disorders are the recognized psychiatric presentations in patients with psoriasis...
March 6, 2018: Reviews in the Neurosciences
Nakul Katyal, Raghav Govindarajan
Pure ocular presentation of Lambert-Eaton syndrome is not a common phenomenon. Such presentation poses significant diagnostic challenges and requires conscientious evaluation. In this review, we have described a case of a patient with pure ocular weakness, initially diagnosed as seronegative ocular myasthenia which on further evaluation was found to have ocular Lambert-Eaton myasthenic syndrome (LEMS).
December 31, 2017: Curēus
Yasuhiro Hijikata, Masahisa Katsuno, Keisuke Suzuki, Atsushi Hashizume, Amane Araki, Shinichiro Yamada, Tomonori Inagaki, Daisuke Ito, Akihiro Hirakawa, Fumie Kinoshita, Masahiko Gosho, Gen Sobue
BACKGROUND: Although spinal and bulbar muscular atrophy (SBMA) has been classified as a motor neuron disease, several reports have indicated the primary involvement of skeletal muscle in the pathogenesis of this devastating disease. Recent studies reported decreased intramuscular creatine levels in skeletal muscles in both patients with SBMA and transgenic mouse models of SBMA, which appears to contribute to muscle weakness. OBJECTIVE: The present study aimed to examine the efficacy and safety of oral creatine supplementation to improve motor function in patients with SBMA...
March 5, 2018: JMIR Research Protocols
Nermin Gorkem Sirin, Elif Kocasoy Orhan, Hacer Durmus, Feza Deymeer, Mehmet Baris Baslo
INTRODUCTION: The aim of this study was to define the diagnostic accuracy of concentric needle (CN)-jitter in newly diagnosed myasthenia gravis (MG) patients and to compare CN-jitter with repetitive nerve stimulation. METHODS: In 30 MG patients, repetitive nerve stimulation in 4 muscles (orbicularis oculi, nasalis, trapezius and abductor digiti minimi) and CN-jitter of extensor digitorum (ED) and frontalis muscles were evaluated. RESULTS: Twenty-eight of 30 patients (93%) had high jitter in at least one muscle...
February 25, 2018: Neurophysiologie Clinique, Clinical Neurophysiology
Ling Li, Haisong Yang, Jian Li, Yunli Yu, Fan Wang, Xianghui Zhu, Guicheng Liu
RATIONALE: Idiopathic hypoparathyroidism (IHP) is a rare endocrine condition, which is frequently represented by neuropsychiatric disorders. Hence, the misdiagnosis rate of the disease is rather high, especially for neurologists. PATIENT CONCERNS: We reported a case of misdiagnosed, atypical IHP. In addition, the literature on IHP and the misdiagnosis published in China in the past 2 decades has been reviewed and summarized. DIAGNOSES: Blood testing confirmed that parathyroid hormone (PTH) = 0 pg/mL and the final diagnosis was IHP...
March 2018: Medicine (Baltimore)
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