scholarly journals A BRIEF HISTORY OF MYASTHENIA GRAVIS AND THYMECTOMY

2021 ◽  
Author(s):  
Bharath V
Keyword(s):  
Myasthenia Gravis ◽  
Surgical Techniques ◽  
Neuromuscular Disorder ◽  
History Of ◽  
Rational Management

Myasthenia gravis (MG) is a rare autoimmune neuromuscular disorder. Though MG was diagnosed four centuries ago, its rational management started in 1930s. In the present era, MG is managed by both medical means and surgical techniques. In this article, the concise history of MG and its management is being described.

scholarly journals Myasthenia Gravis and Thymectomy

2022 ◽  
Author(s):  
V Bharath
Keyword(s):  
Myasthenia Gravis ◽  
Clinical Features ◽  
Intravenous Immunoglobulins ◽  
Neuromuscular Disorder ◽  
Pharmacological Agents ◽  
Diagnosis And Management ◽  
Video Assisted ◽  
History Of ◽  
Rational Management ◽  
Robotic Thymectomy

AbstractMyasthenia gravis (MG) is a rare autoimmune neuromuscular disorder. Though MG was diagnosed four centuries ago, its rational management started in 1930s. In the present era, MG is managed by multimodality care including pharmacological agents, plasmapheresis, intravenous immunoglobulins, and surgical thymectomy. Thymectomy has evolved from open trans-sternal to video-assisted thoracoscopic and robotic thymectomy. In this article, the concise history of MG, its clinical features, diagnosis, and management are described.

scholarly journals Safety and effectiveness of eculizumab in Japanese patients with generalized myasthenia gravis: interim analysis of post-marketing surveillance

2021 ◽  
Vol 14 ◽  
pp. 175628642110019
Author(s):  
Hiroyuki Murai ◽  
Shigeaki Suzuki ◽  
Miki Hasebe ◽  
Yuji Fukamizu ◽  
Ema Rodrigues ◽  
...  
Keyword(s):  
Myasthenia Gravis ◽  
Interim Analysis ◽  
Japanese Patients ◽  
Phase Iii ◽  
High Dose ◽  
Complement Protein ◽  
Double Blind ◽  
Post Marketing Surveillance ◽  
Post Marketing ◽  
History Of

Background: Eculizumab, a humanized monoclonal antibody targeted to terminal complement protein C5, is approved in Japan for treatment of patients with anti-acetylcholine receptor antibody-positive (AChR+) generalized myasthenia gravis (gMG) whose symptoms are difficult to control with high-dose intravenous immunoglobulin (IVIg) therapy or plasmapheresis. Methods: This interim analysis of mandatory post-marketing surveillance in Japan assessed the safety and effectiveness of eculizumab at 26 weeks after treatment initiation in patients with AChR+ gMG. Results: Data were available for 40 adult patients in Japan [62.5% (25/40) female; mean age at eculizumab initiation, 51.0 years]. Fifteen patients had a history of thymoma. Six patients were excluded from the effectiveness analysis set due to participation in the open-label extension part of the phase III, randomized, double-blind, placebo-controlled REGAIN study [ClinicalTrials.gov identifier: NCT02301624]. After 26 weeks’ follow up, 32 patients (80%) were continuing eculizumab treatment. Adverse drug reactions were reported by seven patients [most frequently headache ( n = 3)]. One death was reported during eculizumab treatment (relationship unclear as determined by the treating physician) and there was one death 45 days after the last dose (considered unrelated). No meningococcal infections were reported. Mean (standard deviation) changes from baseline in Myasthenia Gravis-Activities of Daily Living (MG-ADL) and Quantitative Myasthenia Gravis (QMG) scores were −3.7 (2.61) ( n = 27) and −5.6 (3.50) ( n = 26), respectively, at 12 weeks, and −4.3 (2.72) ( n = 26) and −5.6 (4.02) ( n = 24), respectively, at 26 weeks. Improvements in MG-ADL and QMG scores were generally similar in patients with/without a history of thymoma. Frequency of IVIg use decreased following eculizumab initiation. Conclusion: In a real-world setting, eculizumab was effective and well tolerated for the treatment of AChR+ gMG in adult Japanese patients whose disease was refractory to IVIg or plasmapheresis. These findings are consistent with the efficacy and safety results from the global phase III REGAIN study of eculizumab.

Acute exudative paraneoplastic polymorphous vitelliform maculopathy in a patient with thymoma, myasthenia gravis, and polymyositis

2021 ◽  
pp. 112067212199404
Author(s):  
He Yu ◽  
Xinyu Ma ◽  
Nianting Tong ◽  
Zhanyu Zhou ◽  
Yu Zhang
Keyword(s):  
Myasthenia Gravis ◽  
Medical Center ◽  
Postoperative Radiotherapy ◽  
Clinical Manifestations ◽  
Subretinal Fluid ◽  
Metastatic Tumor ◽  
The Body ◽  
Pleural Thickening ◽  
History Of ◽  
Clinically Significant

Importance: This is the first reported case of acute exudative paraneoplastic polymorphous vitelliform maculopathy (AEPPVM) in a patient with thymoma, accompanied by myasthenia gravis (MG) and polymyositis. Objective: To examine the pathogenesis of ocular disease in a patient with yolk-like fundus lesions and thymoma, MG, and polymyositis throughout the body based on clinical manifestations, diagnosis, differential diagnosis, and genetic testing to determine the appropriate treatment course. Design, setting, and participants: We describe a 63-year-old woman who presented to our tertiary medical center with a 3-month history of reduced visual acuity in both eyes. Concurrent fundoscopy revealed a 2.0 × 1.7-mm, unifocal, yellow, round vitelliform lesion in the macular region, surrounded by multifocal, shallow, yellow-white pockets of subretinal fluid. The patient’s medical history included thymoma with thymectomy treatment, combined with pericardiectomy and postoperative radiotherapy (20 years prior), followed by a diagnosis of MG with suspect thymic association (15 years prior). Three years prior, the patient had been diagnosed with polymyositis related to paraneoplastic syndrome; 1 year prior, she had been examined for pleural thickening due to suspected metastatic tumor. Results: On her most recent follow-up visit at 3 months after initial diagnosis, the patient was stable with no clinically significant progression in ocular or systemic conditions.

scholarly journals Becker muscular dystrophy associated with sarcomeric hypertrophic cardiomyopathy in a paediatric patient: a case report

2019 ◽  
Vol 3 (3) ◽  
Author(s):  
Paola Dolader ◽  
Ella Field ◽  
Anna Sarkozy ◽  
Juan Pablo Kaski
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Muscular Dystrophy ◽  
Neuromuscular Disorders ◽  
Elevated Serum ◽  
Becker Muscular Dystrophy ◽  
Neuromuscular Disorder ◽  
Chain Gene ◽  
Septal Hypertrophy ◽  
History Of ◽  
Myocardial Involvement

Abstract Background  Becker muscular dystrophy (BMD) is a neuromuscular disorder associated with myocardial involvement. The most frequent presentation is dilated cardiomyopathy. There have been isolated reports of hypertrophic cardiomyopathy (HCM) in association with BMD, but it is unclear whether these patients had an additional aetiology. Case summary  A 10-year-old boy was diagnosed with BMD having presented with a history of muscular pain during exercise and elevated serum creatine kinase levels. A cardiac screening was arranged and the echocardiogram confirmed an asymmetric septal hypertrophy. Given the unusual finding of HCM in this patient with BMD, we performed genetic testing for HCM-causing mutations and identified a likely pathogenic variant in heterozygosis in the beta-myosin heavy chain gene. Discussion  This case highlights the importance of considering additional aetiologies of cardiac disease in the presence of infrequent phenotypic expressions in neuromuscular disorders.

Risk of reoperation and infection after percutaneous endoscopic lumbar discectomy and open lumbar discectomy

2021 ◽  
Vol 103-B (8) ◽  
pp. 1392-1399
Author(s):  
Tae Wook Kang ◽  
Si Young Park ◽  
Hoonji Oh ◽  
Soon Hyuck Lee ◽  
Jong Hoon Park ◽  
...  
Keyword(s):  
Lumbar Disc ◽  
Surgical Techniques ◽  
Lumbar Discectomy ◽  
Infection Rates ◽  
Percutaneous Endoscopic Lumbar Discectomy ◽  
Primary Endpoints ◽  
Registration Number ◽  
History Of ◽  
Endoscopic Lumbar Discectomy ◽  
Reoperation Rates

Aims Open discectomy (OD) is the standard operation for lumbar disc herniation (LDH). Percutaneous endoscopic lumbar discectomy (PELD), however, has shown similar outcomes to OD and there is increasing interest in this procedure. However despite improved surgical techniques and instrumentation, reoperation and infection rates continue and are reported to be between 6% and 24% and 0.7% and 16%, respectively. The objective of this study was to compare the rate of reoperation and infection within six months of patients being treated for LDH either by OD or PELD. Methods In this retrospective, nationwide cohort study, the Korean National Health Insurance database from 1 January 2007 to 31 December 2018 was reviewed. Data were extracted for patients who underwent OD or PELD for LDH without a history of having undergone either procedure during the preceding year. Individual patients were followed for six months through their encrypted unique resident registration number. The primary endpoints were rates of reoperation and infection during the follow-up period. Other risk factors for reoperation and infection were also evalulated. Results Out of 549,531 patients, 522,640 had undergone OD (95.11%) and 26,891 patients had undergone PELD (4.89%). Reoperation rates within six months were 2.28% in the OD group, and 5.38% in the PELD group. Infection rates were 1.18% in OD group and 0.83% in PELD group. The risk of reoperation was lower for patients with OD than for patients with PELD (adjusted hazard ratio (HR) 0.38). The risk of infection was higher for patients with OD than for patients undergoing PELD (HR, 1.325). Conclusion Compared with the OD group, the PELD group showed higher reoperation rates and lower infection rates. Cite this article: Bone Joint J 2021;103-B(8):1392–1399.

Spasmodic Torticollis Due to Neurovascular Compression of the Spinal Accessory Nerve by the Anteroinferior Cerebellar Artery: Case Report

Neurosurgery ◽  
2000 ◽  
Vol 47 (3) ◽  
pp. 768-772 ◽  
Author(s):  
Concetta Alafaci ◽  
Francesco M. Salpietro ◽  
Gaspare Montemagno ◽  
Giovanni Grasso ◽  
Francesco Tomasello
Keyword(s):  
Cranial Nerve ◽  
Neuromuscular Disorder ◽  
Spasmodic Torticollis ◽  
Accessory Nerve ◽  
Decompression Surgery ◽  
Neurovascular Compression ◽  
Cerebellar Artery ◽  
History Of ◽  
Microvascular Decompression Surgery ◽  
The Right

ABSTRACT OBJECTIVE AND IMPORTANCE Spasmodic torticollis is a neuromuscular disorder characterized by uncontrollable clonic and intermittently tonic spasm of the neck muscles. We report a case of spasmodic torticollis attributable to neurovascular compression of the right XIth cranial nerve by the right anteroinferior cerebellar artery (AICA). CLINICAL PRESENTATION A 72-year-old man with a 2-year history of right spasmodic torticollis underwent magnetic resonance imaging, which demonstrated compression of the right XIth cranial nerve by an abnormal descending loop of the right AICA. INTERVENTION The patient underwent microvascular decompression surgery. During surgery, it was confirmed that an abnormal loop of the right AICA was compressing the right accessory nerve. Compression was released by the interposition of muscle between the artery and the nerve. CONCLUSION The patient's postoperative course was uneventful, and his symptoms were fully relieved at the 2-year follow-up examination. This is the first reported case of spasmodic torticollis attributable to compression by the AICA; usually, the blood vessels involved are the vertebral artery and the posteroinferior cerebellar artery.

Azathioprine-Induced Pancytopenia and Septic Complications: A Probable Cause of Death

2017 ◽  
Vol 31 (5) ◽  
pp. 510-513 ◽  
Author(s):  
Bijoy K. Panda ◽  
Siddhi Umarje ◽  
Arundhati Diwan
Keyword(s):  
Chronic Kidney Disease ◽  
Septic Shock ◽  
Myasthenia Gravis ◽  
Blood Count ◽  
Neuromuscular Disorder ◽  
Suspected Drug ◽  
Septic Complications ◽  
Clinical Investigations ◽  
Severe Pancytopenia ◽  
Relationship Of

Azathioprine, an immunosuppressant which is widely used in the management of the autoimmune neuromuscular disorder. Myasthenia gravis is known to cause myelotoxicity. A 55-year-old male recently diagnosed with myasthenia gravis and chronic kidney disease was put on azathioprine (100 mg/d) along with pyridostigmine and prednisolone. When the treatment was initiated, the hematological reports revealed normal levels of blood count. However, approximately within 3 weeks of continuing the prescribed drugs, the patient was readmitted for complaints of loose watery stools, weakness, and giddiness. Clinical investigations revealed severe pancytopenia, suspecting to be related to azathioprine. The suspected drug (azathioprine) was withdrawn, and the management for pancytopenia was initiated. However, on the second day of hospitalization, the patient underwent cardiac arrest and septic shock which lead to death. Adverse drug reaction assessment revealed a plausible and causal relationship of azathioprine with pancytopenia and other adverse effects seen in this patient.

scholarly journals Analysis of electrooculogram (EOG) signals in studying myasthenia gravis

2021 ◽  
Author(s):  
Timothy Liang
Keyword(s):  
Myasthenia Gravis ◽  
Time Domain ◽  
Early Stage ◽  
Screening Method ◽  
Neuromuscular Disorder ◽  
Severe Form ◽  
Linear Discriminant ◽  
Rise Rate ◽  
Common Precursor ◽  
Average Rise

Myasthenia Gravis (MG) is a neuromuscular disorder that induces muscle weakness and fatigue which can be fatal. A common precursor for severe form of MG is ocular MG. In this thesis, we explored signal processing methodologies for early stage detection of MG using electrooculogram (EOG) signals. An EOG signal database consisting of 62 control and 16 MG (mild to moderate) subjects were analyzed for eye movement characteristics and EOG signal morphologies using time domain and wavelet domain techniques. A linear discriminant analysis (LDA) based classifier was used to quantify the ability of features in separating MG from control samples. Average overall classification accuracy achieved by the proposed method for the best time domain feature (average rise rate) and best wavelet feature (scale band energy) was 82.5% (P<0.01, AUC=0.887) and 83.8% (P<0.01, AUC=0.893), respectively. The obtained results suggest EOG based analysis is a viable, non-invasive alternative MG screening method.

scholarly journals Zytux in Refractory Myasthenia Gravis: A Multicenter, Open-Labeled, Clinical Trial Study of Effectiveness and Safety of a Rituximab Biosimilar

2021 ◽  
Vol 12 ◽  
Author(s):  
Farzad Fatehi ◽  
Kamyar Moradi ◽  
Ali Asghar Okhovat ◽  
Ghazaleh Shojatalab ◽  
Behnaz Sedighi ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Clinical Trial ◽  
Myasthenia Gravis ◽  
Clinical Status ◽  
Neuromuscular Disorder ◽  
Clinical Trial Registration ◽  
Prednisolone Dose ◽  
Immune Mediated ◽  
Post Infusion

Objectives: Myasthenia gravis (MG) is an immune-mediated neuromuscular disorder responsive to immunomodulatory treatments. 10–20% of MGs are not responsive to conventional first-line therapies. Here, we sought to investigate the efficacy and safety of rituximab therapy in the treatment of patients with refractory MG.Methods: In a 48-week, multicenter, open-labeled, prospective cohort setting, 34 participants with refractory MG were assigned to receive infusions of Zytux, which is a rituximab biosimilar, according to a validated protocol. Clinical, functional, and quality of life (QoL) measurements were recorded at baseline, and seven further visits using the Myasthenia Gravis Foundation of America (MGFA), Myasthenia Gravis Composite (MGC), Myasthenia Gravis Activities of Daily Living profile (MG-ADL), and Myasthenia Gravis Quality of Life (MGQoL-15) scales. Besides, the post-infusion side effects were systematically assessed throughout the study.Results: The correlation analysis performed by generalized estimating equations analysis represented a significant reduction of MGC, MG-ADL, and MGQoL-15 scores across the trial period. The subgroup analysis based on the patients' clinical status indicated a significant effect for the interaction between time and MGFA subtypes on MG-ADL score, MGC score, and pyridostigmine prednisolone dose, reflecting that the worse clinical condition was associated with a better response to rituximab. Finally, no serious adverse event was documented.Conclusions: Rituximab therapy could improve clinical, functional, and QoL in patients with refractory MG in a safe setting. Further investigations with larger sample size and a more extended follow-up period are warranted to confirm this finding.Clinical Trial Registration: The study was registered by the Iranian Registry of Clinical Trials (IRCT) (Code No: IRCT20150303021315N18).

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