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Myasthenia

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https://www.readbyqxmd.com/read/29778765/needle-in-a-haystack-post-thymectomy-new-onset-myasthenia-gravis
#1
EDITORIAL
Andrew Tang, Alejandro Bribriesco, Usman Ahmad
Myasthenia gravis can develop after resection of thymic tumors. Post-thymectomy myasthenia gravis can be heralded by elevated preoperative anti-acetylcholine-receptor antibodies. Thymic tissue in isolated mediastinal areas can be the culprit lesion and identified by PET SCAN. Re-resection in selected patients can lead to remission of this rare disease.
May 17, 2018: Seminars in Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/29776427/rheumatoid-meningitis-developed-in-patient-with-stable-rheumatoid-arthritis-and-myasthenia-gravis-detailed-analysis-of-intracranial-inflammation-using-flow-cytometry
#2
Miki Oono, Yoshimasa Fujita, Nobuaki Uchida, Ukichiro Kawai, Michiyo Fujita-Nakata, Megumi Nakanishi, Mitsuru Sanada, Shigemi Nagayama, Makoto Matsui
BACKGROUND: Rheumatoid meningitis (RM) is a rare disorder that often develops during a remission phase of rheumatoid arthritis (RA). This is the first study to demonstrate differences in regard to immunological disturbance between blood and cerebrospinal fluid (CSF) samples obtained from a patient with RM using flow cytometry. CASE PRESENTATION: A 36-year-old woman with RA and generalized myasthenia gravis (MG) developed RM during a remission phase. Although both RA and MG were stable and well controlled, she noticed fever, headache, and transient sensory disturbance...
May 18, 2018: Journal of Neuroinflammation
https://www.readbyqxmd.com/read/29773681/decreased-expression-of-circulating-aire-and-increased-tfh-tfr-cells-in-myasthenia-gravis-patients
#3
Sijia Zhao, Jiaqi Ding, Shengyuan Wang, Chuan Li, Peng Guo, Min Zhang, Zhuyi Li
Myasthenia gravis (MG) is a rare prototypical autoimmune disorder caused by antibodies (Ab) against postsynaptic membrane proteins. Most reports have investigated the role of Autoimmune regulator gene (Aire) in thymic tissue in machianism of MG initiation. So far, the expression of Aire in human peripheral blood cells(we call it circulating Aire expression in the following passage) has not been reported. Herein, we explore the expression of Aire in peripharal blood, circulating T follicular helper (cTfh) and T follicular regulatory (cTfr) cells in MG patients...
May 17, 2018: Bioscience Reports
https://www.readbyqxmd.com/read/29768973/myasthenia-gravis-appearing-after-thymectomy-heralding-recurrent-thymoma
#4
Paul A J Beckers, Rudy Mercelis, Stijn Heyman, Lies Verheuen, Patrick Lauwers, Jeroen M Hendriks, Paul E Van Schil
INTRODUCTION: Thirty to fifty percent of thymoma patients develop myasthenia gravis (MG). In 1.5-28% of cases, MG appears many years after removal of a thymoma. PATIENTS AND METHODS: We present a case report of a 72-year-old female who presented with MG four months after total thymectomy. RESULTS: A 72-year-old female patient presents with MG four months after total thymectomy. Imaging revealed a PET-positive nodule anterior to the superior vena cava...
May 17, 2018: Acta Chirurgica Belgica
https://www.readbyqxmd.com/read/29765992/serological-immunoglobulin-free-light-chain-profile-in-myasthenia-gravis-patients
#5
Umberto Basile, Mariapaola Marino, Cecilia Napodano, Krizia Pocino, Paolo Emilio Alboini, Francesca Gulli, Amelia Evoli, Carlo Provenzano, Emanuela Bartoccioni
Background: Serological levels of free immunoglobulin light chains (FLCs), produced in excess of heavy chains during synthesis of immunoglobulins by plasma cells, can be considered a direct marker of B cell activity in different systemic inflammatory-autoimmune conditions and may represent a useful predictor of rituximab (RTX) therapeutic efficacy, as reported for rheumatoid arthritis and systemic lupus erythematosus. Myasthenia gravis (MG) is an autoimmune disease of the neuromuscular junction with antibodies (abs) targeting the acetylcholine receptor (AChR) or the muscle-specific tyrosine kinase (MuSK), inducing muscle weakness and excessive fatigability...
2018: Journal of Immunology Research
https://www.readbyqxmd.com/read/29761126/seronegative-antibody-mediated-neurology-after-immune-checkpoint-inhibitors
#6
Robert Wilson, David A Menassa, Alexander J Davies, Sophia Michael, Joanna Hester, Wilhelm Kuker, Graham P Collins, Judith Cossins, David Beeson, Neil Steven, Paul Maddison, Simon Rinaldi, Saiju Jacob, Sarosh R Irani
Checkpoint inhibitor medications have revolutionized oncology practice, but frequently induce immune-related adverse events. During autoimmune neurology practice over 20 months, we prospectively identified four patients with likely antibody-mediated neurological diseases after checkpoint inhibitors: longitudinally extensive transverse myelitis, Guillain-Barré syndrome, and myasthenia gravis. All patients shared three characteristics: symptoms commenced 4 weeks after drug administration, responses to conventional immunotherapies were excellent, and autoantibodies traditionally associated with their syndrome were absent...
May 2018: Annals of Clinical and Translational Neurology
https://www.readbyqxmd.com/read/29759142/heterogeneity-of-auto-antibodies-against-nachr-in-myasthenic-serum-and-their-pathogenic-roles-in-experimental-autoimmune-myasthenia-gravis
#7
Ryuichi Nakamura, Tomohiro Makino, Takeshi Hanada, Maki Terakawa, Kazuhiro Nagahira, Jyoji Yamate, Hirokazu Shiraishi, Masakatsu Motomura
Many myasthenia gravis (MG) patients have auto-antibodies against the nicotinic acetylcholine receptor (nAChR), and monoclonal antibodies against the main immunogenic region (MIR) of nAChR can induce experimental autoimmune MG (EAMG). We investigated whether Fab fragment of MIR antibody (Fab35) could block the pathogenicity of polyclonal antibodies. Fab35 partially inhibited nAChR downmodulation, blocked EAMG serum-induced binding of polyclonal antibodies and complement deposition in vitro. Moreover, Fab35 did not ameliorate the EAMG serum-induced EAMG phenotype in rats...
July 15, 2018: Journal of Neuroimmunology
https://www.readbyqxmd.com/read/29748118/diaphragmatic-dysfunction-as-the-presenting-symptom-in-neuromuscular-disorders-a-retrospective-longitudinal-study-of-etiology-and-outcome-in-30-german-patients
#8
Matthias Türk, Irina Weber, Gernot Vogt-Ladner, Rolf Schröder, Martin Winterholler
Diaphragmatic dysfunction is well-known in advanced stages of neuromuscular disorders. However, data on its presence as the presenting symptom in neuromuscular disorders is scarce. The goal of this retrospective longitudinal study was to evaluate the etiology and clinical outcome in patients, in whom uni- or bilateral diaphragmatic dysfunction was primarily diagnosed, before a specific neuromuscular disease was found. Patients with critical illness neuropathy/myopathy were excluded from this study. Analysis of the medical records of two tertiary referral centers for patients with neuromuscular diseases identified 30 corresponding patients with diaphragmatic dysfunction (17 unilateral; 13 bilateral)...
April 9, 2018: Neuromuscular Disorders: NMD
https://www.readbyqxmd.com/read/29746944/increased-risk-of-autoimmune-diseases-in-dengue-patients-a-population-based-cohort-study
#9
Hao-Ming Li, Ying-Kai Huang, Yuan-Chih Su, Chia-Hung Kao
OBJECTIVE: To investigate the risk of autoimmune diseases in dengue patients. METHODS: We conducted a population-based cohort study by the Taiwan National Health Insurance Research Database, including a total of 12506 newly diagnosed dengue patients and 112554 control subjects between 2000 and 2010, matched by gender, age, income, urbanization, and comorbidities. Both cohorts were followed for a 3-year period to examine the incidence of autoimmune diseases. A Cox proportional hazards regression analysis was applied to calculate the risk of autoimmune diseases between both groups...
May 7, 2018: Journal of Infection
https://www.readbyqxmd.com/read/29743585/evaluating-the-temperature-effects-of-ice-and-heat-tests-on-ptosis-due-to-myasthenia-gravis
#10
Emmanuel Marinos, Katherine Buzzard, Clare L Fraser, Stephen Reddel
AIMS: The aims of this study are as follows: (1) to describe a standardised methodology for the ice test, and where necessary a heat test; (2) to determine the effects of local cooling vs. heating on ptosis in myasthenia gravis (MG) patients. METHODS: Twenty-three MG patients with ptosis were identified from a tertiary referral neuroimmunology clinic, seventeen with significant ptosis ≥2 mm. Latex party balloons were filled with ice and cold tap water for the ice test, or with water heated to 45 °C for the heat test...
May 10, 2018: Eye
https://www.readbyqxmd.com/read/29742813/mutated-cancer-autoantigen-implicated-cause-of-paraneoplastic-myasthenia-gravis
#11
Anastasia Zekeridou, Guy E Griesmann, Vanda A Lennon
INTRODUCTION: Anti-tumor immune responses are postulated to initiate paraneoplastic neurological disorders when proteins normally restricted to neural cells are expressed as oncoproteins. Mutated oncopeptides could bypass self-tolerant T-cells to activate cytotoxic effector T-lymphocytes and requisite helper T-lymphocytes to stimulate autoantibody production by B-lymphocytes. METHODS: We investigated muscle-type nicotinic acetylcholine receptor (AChR) antigen expression at transcriptional and protein levels in a small-cell lung cancer line (SCLC) established from a patient with AChR-IgG-positive myasthenia gravis...
May 9, 2018: Muscle & Nerve
https://www.readbyqxmd.com/read/29742795/rituximab-in-refractory-myasthenia-gravis-extended-prospective-study-results
#12
Grayson Beecher, Dustin Anderson, Zaeem A Siddiqi
INTRODUCTION: Rituximab appears beneficial in treatment-refractory myasthenia gravis (MG), however, prospective, long-term durability data is lacking. METHODS: In this prospective, open-label study of rituximab in refractory MG, 22 patients (10 AChR, 9 MuSK, 3 seronegative) received rituximab at baseline, with repeat cycles driven by clinical worsening. Manual muscle testing (MMT) scores and CD19/CD20+ B cell counts were serially monitored. RESULTS: At mean follow-up of 28...
May 9, 2018: Muscle & Nerve
https://www.readbyqxmd.com/read/29736936/hla-and-musk-positive-myasthenia-gravis-a-systemic-review-and-meta-analysis
#13
Y Hong, H-F Li, F Romi, G O Skeie, N E Gilhus
OBJECTIVES: Myasthenia gravis (MG) represents a spectrum of clinical subtypes with differences in disease mechanisms and treatment response. MG with muscle-specific tyrosine kinase (MuSK) antibodies accounts for 1%-10% of all MG patients. We conducted a meta-analysis to evaluate the association between HLA genes and MuSK-MG susceptibility. SUBJECTS AND METHODS: Studies were searched in Pubmed, EMBASE database and other sources between 2001 and 2018. Genotype, allele and haplotype frequencies of HLA loci in MuSK-MG patients and healthy controls were extracted from each included study...
May 7, 2018: Acta Neurologica Scandinavica
https://www.readbyqxmd.com/read/29732733/oral-immunosuppressive-treatment-of-myasthenia-gravis-in-denmark-a-nationwide-drug-utilization-study-1996-2013
#14
Emil Greve Pedersen, Jesper Hallas, Anton Pottegård, Stine Munk Hald, Poul Erik Hyldgaard Jensen, David Gaist
Although immunosuppressants in the treatment of myasthenia have been available for several decades, population-based studies describing drug utilization in myasthenia patients are scarce. We aimed in this study to describe the treatment of myasthenia in Denmark in more recent years with emphasis on use of oral immunosuppressant agents. We identified a nationwide cohort of incident myasthenia patients in Denmark from 1996 to 2013 and tracked their use of drugs over the entire period using data from nationwide registers...
May 6, 2018: Basic & Clinical Pharmacology & Toxicology
https://www.readbyqxmd.com/read/29730659/myxedema-coma-due-to-hashimoto-thyroiditis-a-rare-but-real-presentation-of-failure-to-thrive-in-infancy
#15
Ryan A Heksch, Rohan K Henry
BACKGROUND: Hashimoto thyroiditis (HT) is uncommon in infancy, and myxedema coma (MC) is even less common. While prior reports have documented these entities separately, to our knowledge, MC in combination with HT has not been reported before in this age group. METHODS/RESULTS: A 10-month-old female presented with ptosis, lethargy, dysphagia, and failure to thrive (FTT). She developed hypotension, bradycardia, hypothermia, and apnea requiring intubation. Initial thyroid-stimulating hormone was 422 μIU/mL, and free thyroxine was < 0...
May 4, 2018: Hormone Research in Pædiatrics
https://www.readbyqxmd.com/read/29725917/pain-headache-and-other-non-motor-symptoms-in-myasthenia-gravis
#16
REVIEW
Olivia Tong, Leslie Delfiner, Steven Herskovitz
PURPOSE OF REVIEW: Myasthenia gravis (MG) is traditionally conceptualized as a disease with purely motor manifestations. This paper reviews the supporting evidence and pathophysiology of non-motor symptoms in MG, including pain, headache, special sense and autonomic dysfunction, sleep disturbance, and cognitive and psychosocial issues. RECENT FINDINGS: Work in this area has been limited. Recent studies have identified bodily pain and headache as common complaints in patients with MG...
May 3, 2018: Current Pain and Headache Reports
https://www.readbyqxmd.com/read/29720797/the-spectrum-of-autonomic-dysfunction-in-myasthenic-crisis
#17
Rohit Ninan Benjamin, Sanjith Aaron, Ajith Sivadasan, Suresh Devasahayam, Amalan Sebastin, Mathew Alexander
Background: Autoimmune autonomic dysfunction is described in Myasthenia Gravis. In myasthenic crisis, the spectrum of autonomic dysfunction is hitherto uncharacterized. Objective: The objective of this study is to describe the spectrum of autonomic dysfunction in myasthenic crises using the composite autonomic symptom scale 31 (COMPASS 31) autonomic symptom questionnaire and power spectral analysis of heart rate variability (HRV), which is a simple way of estimating general autonomic dysfunction...
January 2018: Annals of Indian Academy of Neurology
https://www.readbyqxmd.com/read/29718870/ctla-4-methylation-regulates-the-pathogenesis-of-myasthenia-gravis-and-the-expression-of-related-cytokines
#18
Ti-Kun Fang, Cheng-Jun Yan, Juan Du
BACKGROUND: Myasthenia gravis (MG) is a progressive autoimmune disease that occurs as a result of the failure of neuromuscular transmission and is characterized by muscle weakness. There has been evidence on the correlations between the genetic predisposition of cytotoxic T lymphocyte and the antigen-4 (CTLA-4) and MG. Thus, the present study was conducted to study is designed to examine the effects of CTLA-4 methylation on the pathogenesis of MG and the expressions of related cytokines...
May 2018: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29713553/acquired-amegakaryocytic-thrombocytopenia-and-pure-red-cell-aplasia-in-thymoma
#19
Sumit Dahal, Eliza Sharma, Suyash Dahal, Binav Shrestha, Bikash Bhattarai
Association of thymoma with myasthenia gravis, pure red cell aplasia, and aplastic anemia is well documented. However, thymoma complicated by acquired amegakaryocytic thrombocytopenia (AAMT) is rarely reported. Here, we present a case of a 60-year-old male with past medical history of recurrent invasive thymoma who presented with cough and blood in sputum. He was found to have severe normocytic normochromic anemia and thrombocytopenia that did not improve with intravenous steroids or multiple transfusions of red cells and platelets...
2018: Case Reports in Hematology
https://www.readbyqxmd.com/read/29709757/rapid-recovery-of-spontaneous-spinal-epidural-hematoma-without-surgical-treatment-a-case-reportand-literature-review
#20
Shu Baozhang, Fengyang Geng, Jiyue Wang, Zhiti Zhang, Chigang Du
BACKGROUND: Spontaneousspinal epidural hematoma (SSEH) is a relatively uncommon yet potentially disabling neurological emergency.The classical presentation includes a severe acute attack, sometimes radiating pain at the back,interscapular, or neckareas, followed by neurological deficits. The main treatment is surgical,and self-healing cases are rare. CASE DESCRIPTION: A 17-year-old female was admitted to the department of neurosurgery with neck pain, myasthenia of the limbs, and difficulty moving...
April 27, 2018: World Neurosurgery
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