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Myasthenic crisis

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https://www.readbyqxmd.com/read/28636535/the-correlation-of-neutrophil-to-lymphocyte-ratio-with-the-presence-and-activity-of-myasthenia-gravis
#1
De-Hao Yang, Mei-Zi Qian, Mao-Mao Wei, Jia Li, Meng-Meng Yu, Xue-Mian Lu, Hong Yang, Hai Lin, Xiang Li, Jun-Yan Zhu, Xu Zhang
Though the pathogenesis of myasthenia gravis (MG) is not fully understood, the role of inflammation has been well appreciated in the development of MG. We aimed to investigate the role of neutrophil-to-lymphocyte ratio (NLR) in MG patients and the relationship between the NLR and the activity of the disease. A total number of 172 MG patients and 207 healthy controls (HC) were enrolled in this study. The MG patients were divided into tertiles according to NLR (low NLR < 1.58, n = 57; intermediate NLR 1.58-2...
June 16, 2017: Oncotarget
https://www.readbyqxmd.com/read/28635482/diagnosis-and-management-of-myasthenia-gravis
#2
Christopher Barber
Myasthenia gravis is a rare long-term neurological condition that is characterised by fluctuating skeletal muscle weakness and fatigue, as well as respiratory difficulties. It is both an acquired autoimmune disease and a chronic neuromuscular disorder. Because of its rarity, myasthenia gravis is relatively unknown and may be unfamiliar to many nurses. While there are various types of myasthenia, this article focuses on myasthenia gravis, exploring its symptoms, diagnosis and treatment, and examining the nurse's role in managing the condition...
June 21, 2017: Nursing Standard
https://www.readbyqxmd.com/read/28579974/tracheoesophageal-fistula-caused-by-tracheostomy-in-a-patient-with-myasthenia-gravis-after-a-myasthenic-crisis
#3
Chen Jiaxin, Li Jingjing, Zhu Kai, Zhou Zhou, Liu Weibin, Wang Haiyan, Feng Huiyu
A 57-year-old woman with myasthenia gravis (MG), who had experienced a myasthenic crisis, complained of coughing while drinking. At first, this appeared to be a sequela of the myasthenic crisis. However, after further investigation, the problem was identified as a tracheoesophageal fistula, a complication of tracheostomy. Here, we describe this special case in the hope that we can improve diagnostic accuracy by providing a reminder for other physicians to consider the differences between MG and tracheoesophageal fistula...
2017: Frontiers in Neurology
https://www.readbyqxmd.com/read/28540092/anti-musk-positive-myasthenic-crisis-in-a-7-year-old-female
#4
Harrison J Matthews, Apisadaporn Thambundit, Brandon R Allen
A seven-year-old African American female with anti-MuSK-positive Juvenile Myasthenia Gravis collapsed while at school from progressively worsening weakness and dyspnea. On initial emergency department presentation, she required 15 liters per minute of supplemental oxygen to maintain oxygen saturation above 92%. Initial pulmonary function tests and venous blood gas led to the decision to place her on noninvasive positive pressure ventilation (NPPV) with BiPAP in the emergency department. Due to worsening hypercarbia, she later required mechanical intubation in the PICU and underwent IVIG therapy followed by plasmapheresis in order to achieve a stable discharge from the hospital...
2017: Case Reports in Emergency Medicine
https://www.readbyqxmd.com/read/28516329/screening-for-lipoprotein-receptor-related-protein-4-agrin-and-titin-antibodies-and-exploring-the-autoimmune-spectrum-in-myasthenia-gravis
#5
Isabell Cordts, Nicolas Bodart, Kathi Hartmann, Katerina Karagiorgou, John S Tzartos, Lin Mei, Jens Reimann, Philip Van Damme, Michael H Rivner, Alain Vigneron, Joachim Weis, Jörg B Schulz, Socrates J Tzartos, Kristl G Claeys
In autoimmune myasthenia gravis (MG), the identification of antibodies and characterization of serological subgroups is of great importance for diagnosis and management of the disease. Our aims were to study the frequency of antibodies against lipoprotein-related protein 4 (LRP4), agrin, and titin using the most recent techniques, and to characterize corresponding clinical features and autoimmune diseases (AID) in 100 MG-patients. The antibody frequencies in the 55 AChR-antibody positive patients were 7% LRP4, 5% agrin, 53% titin, and in the 45 AChR-antibody negative patients 2% MuSK, 2% LRP4, 2% agrin, and 27% titin...
June 2017: Journal of Neurology
https://www.readbyqxmd.com/read/28505981/severe-exacerbation-of-myasthenia-gravis-associated-with-checkpoint-inhibitor-immunotherapy
#6
Dana S Cooper, Matthew N Meriggioli, Philip D Bonomi, Rabia Malik
Monoclonal antibodies that target either PD-1 or PD-L1 have recently been approved for treatment of advanced non-small cell lung cancer. These antibodies are immune checkpoint inhibitors which have been shown to exacerbate Myasthenia Gravis (MG) and other autoimmune diseases. While effective in preventing tumor cells from evading immune attack, immune checkpoint inhibitors such as nivolumab, an antibody directed against the programmed cell death protein-1 (PD-1) receptor located on T-cells, may also cause immune dysregulation and could cause or potentiate pre-existing autoimmune conditions...
2017: Journal of Neuromuscular Diseases
https://www.readbyqxmd.com/read/28488612/analysis-of-mortality-and-related-factors-in-2195-adult-myasthenia-gravis-patients-in-a-10-year-follow-up-study
#7
Chanchan Liu, Qiong Wang, Zhandong Qiu, Jing Lin, Bo Chen, Yue Li, Mengcui Gui, Min Zhang, Mingshan Yang, Wei Wang, Bitao Bu
OBJECTIVE: To analyze the mortality and potential risk factors for death in myasthenia gravis (MG) patients. MATERIALS AND METHODS: A total of 2195 adult patients with MG (aged older than 18 years) diagnosed during the period between 2003 and 2013 were followed-up and retrospectively reviewed. RESULTS: During the 10-year follow-up, 129 patients died and the total mortality rate was 5.88%. The risk factors associated with MG-related deaths were duration of the disease, occurrence of myasthenic crisis, severity of disease that included the Myasthenia Gravis Foundation of America (MGFA) grade III and IV at onset, elevation of acetylcholine receptor antibody (AchR-abs) titers, presence of thymic pathology, and failure of administrating immunosuppressants (P < 0...
May 2017: Neurology India
https://www.readbyqxmd.com/read/28457757/juvenile-myasthenia-gravis-in-norway-clinical-characteristics-treatment-and-long-term-outcome-in-a-nationwide-population-based-cohort
#8
T H Popperud, M I Boldingh, M Rasmussen, E Kerty
BACKGROUND: This study aimed to characterize juvenile myasthenia gravis in a national population-based cohort in Norway, and to evaluate long-term outcome and potential differences correlated with prepubertal versus postpubertal disease onset. PATIENTS AND METHODS: Patients with onset of myasthenia gravis aged ≤18 years were identified through multiple strategies. Retrospective clinical data were collected by means of medical charts. All patients had an updated clinical examination...
April 20, 2017: European Journal of Paediatric Neurology: EJPN
https://www.readbyqxmd.com/read/28382612/clinical-profile-and-outcome-of-myasthenic-crisis-in-central-taiwan
#9
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/28381664/therapeutic-potential-of-docetaxel-plus-cisplatin-chemotherapy-for-myasthenia-gravis-patients-with-metastatic-thymoma
#10
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/28360447/botulism-outbreak-in-a-family-after-ingestion-of-locally-produced-cheese
#11
Shahram Rafie, Shokrollah Salmanzadeh, Asieh Mehramiri, Adel Nejati
Botulism is one of the most important foodborne diseases and is caused by Clostridium botulinum toxin. The main manifestations are flaccid muscle paralysis and cranial nerve palsies. Botulism is an essential health problem because of its high mortality. The diagnosis of botulism, especially in sporadic cases, is a medical challenge and a high clinical suspicion is necessary for early recognition. So, every physician should be familiar with its signs and symptoms for early detection and treatment. We describe a family with dysphasia and acute paralysis after the ingestion of locally made cheese...
March 2017: Iranian Journal of Medical Sciences
https://www.readbyqxmd.com/read/28278141/metastatic-thymoma-associated-myasthenia-gravis-favorable-response-to-steroid-pulse-therapy-plus-immunosuppressive-agent
#12
Guoyan Qi, Peng Liu, Huimin Dong, Shanshan Gu, Hongxia Yang, Yinping Xue
BACKGROUND Our study retrospectively reviewed the therapeutic effect of steroid pulse therapy in combination with an immunosuppressive agent in myasthenia gravis (MG) patients with metastatic thymoma. MATERIAL AND METHODS MG patients with metastatic thymoma that underwent methylprednisolone pulse therapy plus cyclophosphamide were retrospectively analyzed. Patients initially received methylprednisolone pulse therapy followed by oral methylprednisolone. Cyclophosphamide was prescribed simultaneously at the beginning of treatment...
March 9, 2017: Medical Science Monitor: International Medical Journal of Experimental and Clinical Research
https://www.readbyqxmd.com/read/28214292/prescription-profile-of-pyridostigmine-use-in-a-population-of-patients-with-myasthenia-gravis
#13
Jorge Enrique Machado-Alba, Luis Felipe Calvo-Torres, Andrés Gaviria-Mendoza, César Augusto Mejía-Vélez
INTRODUCTION: We determined the pyridostigmine prescription pattern in a population of patients with myasthenia gravis. METHODS: A descriptive cross-sectional study was done using a prescription database of 3.5 million individuals from which patients diagnosed with myasthenia gravis (MG) and for whom pyridostigmine was prescribed. RESULTS: A total of 306 outpatients with MG were found, and 258 were receiving pyridostigmine, mean age, 53.0±18...
February 18, 2017: Muscle & Nerve
https://www.readbyqxmd.com/read/28174404/-two-cases-of-invasive-thymoma-with-taste-disorder
#14
Tatsuya Katayama, Shinji Hirai
Two 50s female patients with the taste disorder of sweet taste loss and stage IV a type B2 invasive thymoma underwent surgery at our hospital. One patient with myasthenia gravis (MG) developed postoperative myasthenic crisis and recovered by the treatment with plasma apheresis and steroid pulse therapy. Her taste disorder fully recovered together with her MG symptom. The taste disorder of the other patient without MG had persisted for 3 years after the surgery. The taste disorder of sweet taste loss was reported as one of non-motor symptoms caused by MG-related autoimmune mechanisms associated with thymoma, improving with the therapy for MG...
February 2017: Kyobu Geka. the Japanese Journal of Thoracic Surgery
https://www.readbyqxmd.com/read/28166107/challenges-in-the-anesthetic-management-for-a-robotic-thymectomy-in-a-patient-with-myasthenia-gravis-a-case-report
#15
Susan M Martinelli, Bilal D Lateef, Jason M Long, David Y Huang, Ameeta Karmarkar, Brian P Barrick
We describe the case of a patient with myasthenia gravis undergoing a robotic-assisted thymectomy complicated by postoperative myasthenic crisis, with a focus on the anesthetic considerations specific to this case. Because myasthenia gravis is an autoimmune disease affecting acetylcholine receptors, caution must be taken with the use of neuromuscular blockade and reversal. Utilizing a robotic-assisted surgical approach makes anesthetic management challenging given the dangers of patient movement while the robot is docked, lung isolation, extubation criteria, and postoperative disposition...
February 3, 2017: A & A Case Reports
https://www.readbyqxmd.com/read/28109638/myasthenia-triggered-by-immune-checkpoint-inhibitors-new-case-and-literature-review
#16
Natalia L Gonzalez, Araya Puwanant, Angela Lu, Stanley M Marks, Saša A Živković
Immune checkpoint molecules are potent regulators of immunologic homeostasis that prevent the development of autoimmunity while maintaining self-tolerance. Inhibitors of immune checkpoint molecules are used as immunotherapy in the treatment of melanoma and different types of refractory cancer, and can trigger various autoimmune complications including myositis and myasthenia gravis. We describe a case of generalized myasthenia gravis induced by pembrolizumab and review 11 other cases. Five patients also had elevated serum CK levels ranging from 1200 to 8729 IU/L, and biopsy showed myositis in one...
March 2017: Neuromuscular Disorders: NMD
https://www.readbyqxmd.com/read/27907966/getting-rid-of-weakness-in-the-icu-an-updated-approach-to-the-acute-management-of-myasthenia-gravis-and-guillain-barr%C3%A3-syndrome
#17
Alexis A Lizarraga, Karlo J Lizarraga, Michael Benatar
After prompt diagnosis, severe myasthenia gravis and Guillain-Barré syndrome (GBS) usually require management in the intensive care unit. In the myasthenic patient, recognition of precipitating factors is paramount, and frequent monitoring of bulbar, upper airway, and/or respiratory muscle strength is needed to identify impending myasthenic crisis. Noninvasive ventilation can be attempted prior to intubation and mechanical ventilation in the setting of respiratory failure. Cholinesterase inhibitors should be discontinued, but resumed prior to extubation, and steroid dosage could be increased once the airway is secured...
December 2016: Seminars in Neurology
https://www.readbyqxmd.com/read/27856933/predictors-of-extubation-outcomes-following-myasthenic-crisis
#18
Zhenguo Liu, Shiyuan Yao, Qian Zhou, Zhensheng Deng, Jianyong Zou, Huiyu Feng, Hua Zhu, Chao Cheng
Objective Myasthenic crisis (MC) is considered the most severe adverse event in patients with myasthenia gravis. The present retrospective study was performed to evaluate the predictors of clinical outcomes in patients with MC. Methods The medical charts of 33 patients (19 women, 14 men) with 76 MC attacks from 2002 to 2014 were retrospectively reviewed. Early extubation (≤7 days) and prolonged ventilation (>15 days) during the MC were used to assess patient outcomes. Results Among the 33 patients, 24 (72...
December 2016: Journal of International Medical Research
https://www.readbyqxmd.com/read/27781146/prevalence-and-impact-of-autoimmune-thyroid-disease-on-myasthenia-gravis-course
#19
Justyna Kubiszewska, Beata Szyluk, Piotr Szczudlik, Zbigniew Bartoszewicz, Małgorzata Dutkiewicz, Maksymilian Bielecki, Tomasz Bednarczuk, Anna Kostera-Pruszczyk
OBJECTIVES: Autoimmune thyroid diseases (ATDs) frequently accompany myasthenia gravis (MG) and may influence its course. We aimed to determine the association and impact of ATD with early- (<50 years), late-onset MG, or thymoma-MG. MATERIALS AND METHODS: Prevalence of ATD was measured in a cross-sectional study of 343 consecutive patients with MG (236 F, 107 M) aged 4-89 years; 83.8% were seropositive, in 2.9%, anti-MuSK antibodies were detected. Concentrations of antithyroid peroxidase antibodies, antithyroglobulin antibodies, antithyrotropin receptor antibodies, and TSH level were measured in all patients...
October 2016: Brain and Behavior
https://www.readbyqxmd.com/read/27744133/repetitive-nerve-stimulation-often-fails-to-detect-abnormal-decrement-in-acute-severe-generalized-myasthenia-gravis
#20
Maarika Liik, Anna Rostedt Punga
OBJECTIVE: We assessed the diagnostic pattern of repetitive nerve stimulation (RNS) test and concentric electrode (CNE) jitter analysis between patients with generalized myasthenia gravis (GMG) with acute versus slow onset. METHODS: All examinations that established the diagnosis of GMG at the department of Clinical Neurophysiology, Uppsala University Hospital, were retrospectively analyzed from January 2012 to December 2014. Patients were grouped according to disease duration at neurophysiological evaluation: acute onset (<4weeks) or slow onset (⩾4weeks)...
November 2016: Clinical Neurophysiology: Official Journal of the International Federation of Clinical Neurophysiology
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