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https://www.readbyqxmd.com/read/28820310/orbital-mantle-cell-lymphoma-presenting-as-myasthenia-gravis
#1
Justin Karlin, Travis Peck, Karyn Prenshaw, Craig A Portell, Maria Kirzhner
A 69-year-old man, previously treated with pyridostigmine for myasthenia gravis (manifesting as ptosis and diplopia) was evaluated for several concomitant bilateral anterior orbital masses. Imaging revealed 3 discrete, solid masses within and around the orbits. An incisional biopsy demonstrated atypical lymphocytes positive for CD20 and Cyclin-D1, consistent with mantle cell lymphoma. The patient received induction chemotherapy with a rituximab-based regimen. He experienced resolution of his diplopia and ptosis after one cycle of chemotherapy and achieved complete remission of the orbital masses and myasthenia symptoms after 6 cycles...
August 18, 2017: Orbit
https://www.readbyqxmd.com/read/28814461/subcutaneous-immunoglobulin-in-myasthenia-gravis-exacerbation-a-prospective-open-label-trial
#2
Grayson Beecher, Dustin Anderson, Zaeem A Siddiqi
OBJECTIVE: To investigate the efficacy, tolerability, and safety of subcutaneous immunoglobulin (SCIg) in patients with mild to moderate myasthenia gravis (MG) exacerbation. METHODS: We performed a prospective, open-label, phase 3 trial in patients with MG aged 18 years or older and mild to moderate worsening (transition from Myasthenia Gravis Foundation of America class I to II/III or class II to III), treated with SCIg (2 g/kg), self-administered over 4 weeks...
August 16, 2017: Neurology
https://www.readbyqxmd.com/read/28813773/a-case-of-ivig-induced-aseptic-chemical-meningitis
#3
Anish Patel, Kalyan Chakravarthy Potu, Tamera Sturm
Intravenous immunoglobulin (IVIG) is a commonly used and generally well-tolerated medication. Common side effects include flu-like symptoms such as fevers, headaches, myalgia, fatigue, and nausea. One of the more rare side effects is aseptic meningitis, with a reported incidence rate of around 0.067 percent of all IVIG infusions. In this paper, we describe a 47-year-old female patient with a history of myasthenia gravis who presented with a headache, neck pain, and neck stiffness while undergoing IVIG infusions for a myasthenia crisis...
March 2017: South Dakota Medicine: the Journal of the South Dakota State Medical Association
https://www.readbyqxmd.com/read/28803488/clinical-outcome-and-predictive-factors-of-postoperative-myasthenic-crisis-in-173-thymomatous-myasthenia-gravis-patients-myasthenic-crisis-in-thymomatous-patients
#4
Yan Li, Haiyan Wang, Pei Chen, Zhenguang Chen, Chunhua Su, Chuanming Luo, Huiyu Feng, Weibin Liu
PURPOSE: Thymectomy is the first-line therapy for thymomatous myasthenia gravis patients. The aim of this study is to explore the clinical outcome and predictors of postoperative myasthenic crisis (POMC) in these patients. METHOD: Clinical data of 173 thymomatous myasthenia gravis patients undergoing thymectomy from January 2000 to March 2013 were retrospectively reviewed. Variables potentially affecting the occurrence of POMC were evaluated using binary logistic regression analysis...
August 14, 2017: International Journal of Neuroscience
https://www.readbyqxmd.com/read/28801669/balance-between-estrogens-and-proinflammatory-cytokines-regulates-chemokine-production-involved-in-thymic-germinal-center-formation
#5
Nadine Dragin, Patrice Nancy, José Villegas, Régine Roussin, Rozen Le Panse, Sonia Berrih-Aknin
The early-onset form of Myasthenia Gravis (MG) is prevalent in women and associates with ectopic germinal centers (GCs) development and inflammation in the thymus. we aimed to investigate the contribution of estrogens in the molecular processes involved in thymic GCs formation. We examined expression of genes involved in anti-acetylcholine receptor (AChR) response in MG, MHC class II and α-AChR subunit as well as chemokines involved in GC development (CXCL13, CCL21and CXCL12). In resting conditions, estrogens have strong regulatory effects on thymic epithelial cells (TECs), inducing a decreased protein expression of the above molecules...
August 11, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28801338/rituximab-as-treatment-for-anti-musk-myasthenia-gravis-multicenter-blinded-prospective-review
#6
Michael K Hehir, Lisa D Hobson-Webb, Michael Benatar, Carolina Barnett, Nicholas J Silvestri, James F Howard, Diantha Howard, Amy Visser, Brian A Crum, Richard Nowak, Rachel Beekman, Aditya Kumar, Katherine Ruzhansky, I-Hweii Amy Chen, Michael T Pulley, Shannon M LaBoy, Melissa A Fellman, Shane M Greene, Mamatha Pasnoor, Ted M Burns
OBJECTIVE: To evaluate the efficacy of rituximab in treatment of anti-muscle-specific kinase (MuSK) myasthenia gravis (MG). METHODS: This was a multicenter, blinded, prospective review, comparing anti-MuSK-positive patients with MG treated with rituximab to those not treated with rituximab. The primary clinical endpoint was the Myasthenia Gravis Status and Treatment Intensity (MGSTI), a novel outcome that combines the Myasthenia Gravis Foundation of America (MGFA) postintervention status (PIS) and the number and dosages of other immunosuppressant therapies used...
August 11, 2017: Neurology
https://www.readbyqxmd.com/read/28798720/commentary-amyotrophic-lateral-sclerosis-and-myasthenia-gravis-overlap-syndrome-a-review-of-two-cases-and-the-associated-literature
#7
COMMENT
Ian Paul Johnson, Patrizia Longone
No abstract text is available yet for this article.
2017: Frontiers in Neurology
https://www.readbyqxmd.com/read/28796382/physician-and-self-assessed-myasthenia-gravis-activities-of-daily-living-score
#8
Hye Lim Lee, Ju-Hong Min, Jin Myoung Seok, Eun Bin Cho, Hye Jin Cho, Yong-Dae Kim, Byoung Joon Kim
INTRODUCTION: The myasthenia gravis-activities of daily living profile (MG-ADL) scale is simple. We aimed to validate this scale in the Korean language and to compare physician- and self-assessed MG-ADL scores (pMG-ADL-K and sMG-ADL-K). METHODS: pMG-ADL-K and sMG-ADL-K and MG composite (MGC) scores were obtained from patients. The correlation between pMG-ADL-K and MGC and the relationship between pMG-ADL-K and sMG-ADL-K were assessed using Cronbach's alpha and Spearman's coefficient...
August 10, 2017: Muscle & Nerve
https://www.readbyqxmd.com/read/28796013/electroconvulsive-therapy-for-depression-comorbid-with-myasthenia-gravis-a-case-report-and-review-of-the-literature
#9
Mark B Warren, Scott Elder, Norman P Litchfield
OBJECTIVES: Myasthenia gravis (MG) is a rare but well-described autoimmune disease, which is sometimes comorbid with psychiatric illness. There have been several case reports describing the use of electroconvulsive therapy (ECT) for the treatment of core psychopathology in the context of MG. We sought to review the available published data on ECT in MG and add another case example to the literature. METHODS: We performed a PubMed search for relevant articles or case reports in English describing ECT in MG and summarized findings...
August 9, 2017: Journal of ECT
https://www.readbyqxmd.com/read/28790248/-concomitant-operations-for-thoracic-aortic-aneurysm-and-myasthenia-gravis-report-of-a-case
#10
Hayato Konishi, Takahiro Katsumata, Yoshikazu Motohashi, Hiroaki Uchida, Eiki Woo, Tomoyasu Sasaki, Shigetoshi Mieno, Hideki Ozawa, Masahiro Daimon, Shintaro Nemoto
A 77-year-old man, who had been under medical treatment for myasthenia gravis without thymoma, was diagnosed with aortic arch aneurysm. He underwent total aortic arch replacement and total resection of the thymus through median sternotomy. His symptoms relating to myasthenia gravis dramatically disappeared after the surgery. The serum anti-acetyl chorine receptor antibody decreased from 2.7 to 0.7 nmol/l (N<0.2) with the reduction of oral predonisolone from 12.5 to 5 mg/day at 4 years after the surgery. The concomitant operations significantly improved his quality of life...
August 2017: Kyobu Geka. the Japanese Journal of Thoracic Surgery
https://www.readbyqxmd.com/read/28789841/responsiveness-to-low-dose-rituximab-in-refractory-generalized-myasthenia-gravis
#11
Sisi Jing, Yang Song, Jie Song, Song Pang, Chao Quan, Lei Zhou, Yuyuan Huang, Jiahong Lu, Jianying Xi, Chongbo Zhao
We aim to investigate the effect of a low dose of rituximab (RTX) in improving the clinical symptoms of refractory generalized myasthenia gravis (MG). Eight patients with refractory generalized MG were treated with a low dose of 600mg RTX. Patients were evaluated by serial clinical scales, flow cytometry of peripheral blood B, T and NK cells, immunoglobulin, complement levels and antibody titer. The quantitative MG score (QMGS), manual muscle testing (MMT), MG-related activities of daily living (MG-ADL) and MG-specific quality-of-life (QOL) were recorded at baseline as well as 1, 3, and 6months after RTX infusion...
May 30, 2017: Journal of Neuroimmunology
https://www.readbyqxmd.com/read/28789662/predictive-factors-of-prolonged-mechanical-ventilation-overall-survival-and-quality-of-life-in-patients-with-post-thymectomy-myasthenic-crisis
#12
Kun-Kun Li, Kai Qian, Yong-Geng Feng, Wei Guo, Qun-You Tan, Bo Deng
BACKGROUND: Thymectomy is the primary approach for the treatment of myasthenia gravis (MG). This retrospective study aimed to identify the clinical and demographical features that may impact the duration of mechanical ventilation (DMV), the long-term survival, and the quality of life (QOL) in patients with post-thymectomy myasthenic crisis (PTMC). METHODS: We reviewed the patients who suffered from PTMC from June 2008 to November 2015. Cox proportional hazard regression analysis was used to identify potential prognostic factors that may impact DMV and long-term survival...
August 8, 2017: World Journal of Surgical Oncology
https://www.readbyqxmd.com/read/28782655/on-the-role-of-igg4-in-inflammatory-conditions-lessons-for-igg4-related-disease
#13
REVIEW
David C Trampert, Lowiek M Hubers, Stan F J van de Graaf, Ulrich Beuers
The pathophysiology of immunoglobulin G4-related disease (IgG4-RD) and its most common manifestations, IgG4-associated (sclerosing) cholangitis and autoimmune pancreatitis, remains largely unknown, but IgG4 is presumably involved. IgG4 is a promiscuous antibody, which could be directly pathogenic, fulfill a protective role, or could just be a fortuitous marker of an aberrant inflammatory response. IgG4 antibodies possess exclusive structural and functional characteristics suggesting anti-inflammatory and tolerance-inducing effects...
August 3, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/28780706/validity-reliability-and-sensitivity-to-change-of-the-myasthenia-gravis-activities-of-daily-living-profile-in-a-sample-of-italian-myasthenic-patients
#14
Alberto Raggi, Carlo Antozzi, Fulvio Baggi, Matilde Leonardi, Lorenzo Maggi, Renato Mantegazza
The purpose of this study is to report on the validity, reliability, and sensitivity of the myasthenia gravis activities of daily living profile (MG-ADL) in a sample of Italian patients. Patients with myasthenia gravis (MG) completed a protocol that included the MG-ADL, the WHO Disability Assessment Schedule (WHODAS 2.0), the Besta Neurological Institute rating scale for myasthenia gravis, and the MG-composite. Cronbach's alpha was used to test reliability, Spearman's correlation and intra-class correlation coefficient (ICC) to test short-term test-retest, Kruskal-Wallis test to assess differences in MG-ADL between patients with different disease severity, and Wilcoxon signed-rank test to assess sensitivity to change...
August 5, 2017: Neurological Sciences
https://www.readbyqxmd.com/read/28780294/pathophysiology-and-immunological-profile-of-myasthenia-gravis-and-its-subgroups
#15
REVIEW
Fredrik Romi, Yu Hong, Nils Erik Gilhus
Myasthenia gravis (MG) is an autoimmune antibody-mediated disease characterized by muscle weakness and fatigability. It is believed that the initial steps triggering humoral immunity in MG take place inside thymic tissue and thymoma. The immune response against one or several epitopes expressed on thymic tissue cells spills over to neuromuscular junction components sharing the same epitope causing humoral autoimmunity and antibody production. The main cause of MG is acetylcholine receptor antibodies. However, many other neuromuscular junction membrane protein targets, intracellular and extracellular proteins are suggested to participate in MG pathophysiology...
August 3, 2017: Current Opinion in Immunology
https://www.readbyqxmd.com/read/28768265/prompt-response-to-prednisone-predicts-benign-course-in-musk-mg
#16
Ozlem Gungor-Tuncer, Vuslat Yilmaz, Alper Toker, Guher Saruhan-Direskeneli, Yesim Gulsen-Parman, Piraye Oflazer-Serdaroglu, Feza Deymeer
BACKGROUND: The difficult course of patients with myasthenia gravis (MG) with anti-muscle-specific tyrosine kinase antibodies (MuSK) has been emphasized. However, no clear information is available on patients who have a benign course. METHODS: This study was aimed at comparing patients with favorable (minimal manifestations [MM] or better) and unfavorable outcomes to determine whether excellent response to corticosteroid (CS) treatment within 3 months (good response-3 months) has any predictive effect on the prognosis...
July 28, 2017: European Neurology
https://www.readbyqxmd.com/read/28765176/transient-clinical-improvement-of-a-mitochondrial-neurogastrointestinal-encephalomyopathy-like-syndrome-after-allogeneic-haematopoietic-stem-cell-transplantation
#17
Malcolm Kevin Baker, Clara Maria Schutte, Neelay Ranchhod, David Brittain, J E van Rensburg
Mitochondrial neurogastrointestinal encephalopathy (MNGIE), usually an autosomal-recessive inherited condition, causes gastrointestinal dysmotility, ophthalmoplegia, ptosis, leukoencephalopathy and neuropathy. The chromosome 22 disorder, due to mutations in the nuclear gene TYMP encoding thymidine phosphorylase (TP), leads to the accumulation of thymidine and deoxyuridine, with mitochondrial dysfunction.This report describes a patient with an MNGIE-like syndrome with a heterozygous TYMP mutation who showed marked, but transient improvement postallogeneic haematopoietic stem cell transplantation (HSCT)...
August 1, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28765064/treatment-of-epilepsy-in-patients-with-myasthenia-gravis-is-really-harder-than-it-looks
#18
Paulo José Lorenzoni, Renata Dal-Prá Ducci, Tallulah Spina Tensini, Giuliano Dalledone, Claudia Suemi Kamoi Kay, Luciano de Paola, Lineu Cesar Werneck, Rosana Herminia Scola, Carlos Silvado
The relationship between myasthenia gravis (MG) and epilepsy has been rarely reported. As consequence, there are no specific guidelines for the management of these conditions when they mutually occur. We reported on three patients in whom epilepsy and MG are coexisting, but in different clinical settings. Two patients were treated with antiepileptic drugs which improved their symptoms. One patient has controlled the seizures after a successful anterior temporal lobectomy with no appreciable consequences to her MG...
July 29, 2017: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
https://www.readbyqxmd.com/read/28763880/-clinical-significance-of-serum-resistin-in-patients-with-generalized-myasthenia-gravis
#19
N Zhao, D Q Zhang, L J Zhang, L N Yang, L M Li, Y Qi, J Wang, L Yang
Objective: To detect the serum resistin levels in patients with generalized myasthenia gravis (GMG) and evaluate the clinical values of resistin. Methods: We detected the serum resistin levels in 58 patients with GMG and 58 healthy controls (HC) from January 2013 to December 2015 in Tianjin medical university general hospital.Then we analyzed the correlation of the serum resistin levels with the clinical features. Results: The serum resistin levels in patients with GMG, (8.26±4.27) ng/ml, was significantly higher than in HC, (4...
July 18, 2017: Zhonghua Yi Xue za Zhi [Chinese medical journal]
https://www.readbyqxmd.com/read/28761633/oculopharyngeal-muscular-dystrophy-misdiagnosed-as-myasthenia-gravis-case-report-and-review-of-literature
#20
Omid Aryani, Mohammadreza Akbari, Masoud Aghsaei-Fard, Arash Mirmohammad-Sadeghi, Samira Yadegari
No abstract text is available yet for this article.
April 4, 2017: Iranian Journal of Neurology
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