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 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 Clinical Features, Diagnosis and Management of Patients with Suspicion of Fascioliasis in Kohgiluyeh and Boyer-Ahmad Province, Southwestern Iran

2020 ◽  
Author(s):  
Abdolali MOSHFE ◽  
Arash ARIA ◽  
Najme ERFANI ◽  
Ali JAMSHIDI ◽  
Bahador SARKARI ◽  
...  
Keyword(s):  
Clinical Features ◽  
Clinical Symptoms ◽  
Epigastric Pain ◽  
Serological Test ◽  
Demographic Data ◽  
Clinical Signs ◽  
Signs And Symptoms ◽  
Diagnosis And Management ◽  
History Of ◽  
Clinical Signs And Symptoms

Background: In the current study, we described the epidemiological features, clinical presentation, diagnosis and management of patients with suspicion of fascioliasis in Kohgiluyeh and Boyer-Ahmad Province in southwest of Iran.    Methods: Overall, 56 patients with suspicion of fascioliasis, based on their clinical signs and symptoms that referred to Clinic of Internal Medicine in Yasuj city, from 2014 to 2016 were enrolled. Demographic data, history of eating aquatic local plants, the chief complains, and laboratory findings were recorded for each patient. Stool samples were obtained from each case for detection of Fasciola eggs. Moreover, blood samples were taken from each patient and evaluated for detection of anti-Fasciola antibodies by an indirect ELISA. Patients who defined as having fascioliasis were treated with triclabendazole and were followed for at least three months for clinical improvement. Results: Serological test was positive in 5 patients. Of these 5 cases, three cases had a history of ingesting raw aquatic vegetables. The main clinical signs and symptoms in positive cases were; abdominal pain (60%), epigastric pain (40%), anemia (60%), and dermal pruritus (20%). Hypereosinophilia was seen in all of 5 positive cases. No Fasciola egg was found in stool specimens of any of the patients. The fascioliasis cases were treated by triclabendazole and clinical symptoms disappeared in all of 5 cases. Conclusion: Our observation further confirmed Yasuj district as a human endemic area for fascioliasis in Iran. The study also highlighted the importance of clinical features together with eosinophilia, as key parameters, in the diagnosis of human fascioliasis. Clinicians need to be aware of this disease and should keep in mind fascioliasis when hypereosinophilia present in patients in such endemic areas.

scholarly journals MYASTHENIA GRAVIS

2016 ◽  
Vol 23 (10) ◽  
pp. 1258-1262
Author(s):  
Raheel Ahmed ◽  
Sunil Kumar ◽  
Mr. Awais ◽  
Atif Sitwat Hayat
Keyword(s):  
Myasthenia Gravis ◽  
Clinical Features ◽  
Tertiary Care ◽  
Clinical Manifestations ◽  
Neuromuscular Disorder ◽  
Care Hospital ◽  
Chi Square ◽  
Duration Of Symptoms ◽  
Chi Square Test ◽  
Test Of Significance

Objectives: To determine the frequency of different clinical features of myastheniagravis in patients presenting to a tertiary care hospital, Karachi. Study Design: Cross sectionalstudy. Setting: Neurology ward, JPMC, Karachi. Period: 23rd January 2013 to 22nd July 2013.Patients and Methods: A total of 71 diagnosed patients of Myasthenia Gravis (MG) betweenthe age 15 and 70 years were recruited. Structured questionnaire was used to collect the dataregarding most common clinical manifestations of MG. Data were entered and analyzed inSPSS version 17. Chi-Square test was used as test of significance. Results: Mean age + S.Dof patients was 34.11 ± 10.42 years. The mean ± SD of duration of symptoms among thesepatients was 5.23 ± 3.52 months. Most of the patients (21%) belong to age between 21 to30 years. Regarding clinical features in these patients of myasthenia gravis it was noted thatptosis and diplopia were most common symptoms, 62% and 54.9%, respectively. Conclusion:Myasthenia gravis, a chronic neuromuscular disorder leads to various degrees of neurologicdysfunction which manifest as different clinical features. The current study found that ocularsymptoms are commonest presenting features.

scholarly journals Contracturing Granulomatous Myositis with Recurrent Malignant Thymoma and Myasthenia Gravis: A Case Report

2021 ◽  
pp. 1-5
Author(s):  
Ching On Luk ◽  
Wai Ting Joyce Lo ◽  
Kitty Yan Kwok ◽  
Moon Ho Leung ◽  
Wing Chi Fong ◽  
...  
Keyword(s):  
Myasthenia Gravis ◽  
Clinical Features ◽  
Case Reports ◽  
Immunoglobulin A ◽  
Chemotherapy Treatment ◽  
Malignant Thymoma ◽  
History Of ◽  
Antibody Levels ◽  
Granulomatous Myositis ◽  
Anti Acetylcholine

Contracturing granulomatous myositis (CGM) is a very rare myopathic syndrome where patients present with slowly progressive muscle pain or weakness affecting mainly proximal muscles together with flexion contractures of the limbs. To date, there have only been several case reports of CGM, which have been reported to occur most commonly in association with sarcoidosis. We report a case of CGM associated with recurrent malignant thymoma and myasthenia gravis (MG). A gentleman with history of treated malignant thymoma presented with new onset proximal muscle weakness and pain that later evolved into contractures that persisted despite treatment with steroids and intravenous immunoglobulin. A malignancy workup revealed recurrence of his malignant thymoma warranting chemotherapy treatment. His contractures only resolved after addition of chemotherapy treatment. He later developed clinical features of MG 1 year after his initial elevation in Anti-acetylcholine (Anti-AChR) antibody levels. We postulate CGM to be a paraneoplastic phenomenon of underlying malignant thymoma. Treatment of thymoma together with immunosuppressive therapies led to clinical improvement of our patient’s CGM. The sole elevation of Anti-AChR levels without the accompanying clinical features does not equate to a MG diagnosis, but should raise the clinician’s vigilance for future development of the condition.

ITI with high-dose FIX and combined immunosuppressive therapy in a patient with severe haemophilia B and inhibitor

2009 ◽  
Vol 29 (02) ◽  
pp. 155-157 ◽  
Author(s):  
H. Hauch ◽  
J. Rischewski ◽  
U. Kordes ◽  
J. Schneppenheim ◽  
R. Schneppenheim ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Immunosuppressive Agents ◽  
Tolerance Induction ◽  
Intravenous Immunoglobulins ◽  
Factor Ix ◽  
High Dose ◽  
Allergic Reactions ◽  
Success Rates ◽  
Serious Infections ◽  
History Of

SummaryInhibitor development is a rare but serious event in hemophilia B patients. Management is hampered by the frequent occurrence of allergic reactions to factor IX, low success rates of current inhibitor elimination protocols and the risk of development of nephrotic syndrome. Single cases of immune tolerance induction (ITI) including immunosuppressive agents like mycophenolat mofetil (MMF) or rituximab have been reported. We present a case of successful inhibitor elimination with a combined immune-modulating therapy and high-dose factor IX (FIX). This boy had developed a FIX inhibitor at the age of 5 years and had a history of allergic reactions to FIX and to FEIBA→. Under on-demand treatment with recombinant activated FVII the inhibitor became undetectable but the boy suffered from multiple joint and muscle bleeds. At the age of 11.5 years ITI was attempted with a combination of rituximab, MMF, dexamethasone, intravenous immunoglobulins and high-dose FIX. The inhibitor did not reappear and FIX half-life normalized. No allergic reaction, no signs of nephrotic syndrome and no serious infections were observed.

The diagnosis and management of ocular myasthenia gravis

2019 ◽  
Vol 5 (1-2) ◽  
pp. 3-8
Author(s):  
Gordon Plant
Keyword(s):  
Myasthenia Gravis ◽  
Severe Form ◽  
Differential Diagnostics ◽  
Diagnosis And Management ◽  
Ocular Myasthenia ◽  
Clinical Presentations ◽  
Ocular Myasthenia Gravis

Ocular myasthenia is not the most severe form of myasthenia but it might cause certain difficulties to diagnose. In this article, we shall discuss the clinical presentations of ocular myasthenia, its differential diagnostics, examinations and management. Keywords: ocular myasthenia gravis, diagnosis, examination, management.

Myasthenia gravis miatt végzett három különböző típusú csecsemőmirigy-eltávolítás sebészeti és korai neurológiai eredményei

2015 ◽  
Vol 68 (6) ◽  
pp. 219-224
Author(s):  
Aurél Ottlakán ◽  
Tibor Géczi ◽  
Balázs Pécsy ◽  
Bernadett Borda ◽  
Judit Lantos ◽  
...  
Keyword(s):  
Myasthenia Gravis ◽  
Thoracoscopic Surgery ◽  
Extended Thymectomy ◽  
Video Assisted ◽  
Video Assisted Thoracoscopic Surgery

Absztrakt Célkitűzés: A myasthenia gravis (MG) kezelésében számos nyitott, illetve minimálisan invazív thymectomia ismert. A tanulmány ugyanazon intézeten belül a transsternalis (TS), illetve kétféle minimálisan invazív thymectomia (video-assisted thoracoscopic extended thymectomy – VATET; unilateral video-assisted thoracoscopic surgery – UL-VATS) eredményeit hasonlítja össze. Anyag és módszerek: Három különböző időintervallumban 71 betegnél történt thymectomia MG miatt (60 nő, 11 férfi): 23 transsternalis thymectomia (1995. január–2004. szeptember), 22 VATET (2004. szeptember – 2009. augusztus) és 26 UL-VATS thymectomia (2009. szeptember – 2011. december). Az eredmények értékelésénél a műtéti idő, MG-hez társuló neurológiai és a műtét utáni sebészi szövődmények, valamint az MG státuszában az egyéves utánkövetéskor észlelt neurológiai változások szerepeltek. Eredmények: Perioperatív mortalitás nem fordult elő. A műtéti idő 112, 211, 116 perc (p = 0,001), a kórházi napok száma: 8,9, 5,6 és 4 nap (p = 0,001) volt a TS-, VATET- és UL-VATS-csoportban. Az MG-hez kapcsolódó postoperativ neurológiai szövődmények 21,7%, 18,2% és 7,7% (p = 0,365) értékeket mutattak. A sebészi szövődmény 4,3%, 13,7%, 0% (p = 0,118) volt. Az MG tüneteinek javulása 91,3%, 94,7%, 87,5% (p = 0,712), míg komplett remisszió 13%, 10,5%, 11,5% (p = 0,917) volt a TS-, VATET- és UL-VATS-csoportokban. Következtetések: A műtéti idő, valamint a kórházban eltöltött napok száma UL-VATS esetében volt a legrövidebb. A kisebb sebészi beavatkozáshoz alacsonyabb sebészi, illetve MG-s neurológiai szövődmények társultak. Az MG-tünetek javulásában mindhárom módszernél kiváló eredményt értek el.

scholarly journals AB0450 PERIPHERAL MACROVASCULAR INVOLVEMENT IN SYSTEMIC SCLEROSIS AS COMPARED WITH HEALTHY CONTROLS: A SMALL COHORT STUDY BY COLOR AND SPECTRAL DOPPLER ULTRASONOGRAPHY

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1252.2-1252
Author(s):  
R. D’alessandro ◽  
E. Garcia Gonzales ◽  
P. Falsetti ◽  
C. Baldi ◽  
F. Bellisai ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Clinical Features ◽  
Doppler Ultrasonography ◽  
Power Doppler ◽  
Ulnar Artery ◽  
Artery Occlusion ◽  
Power Doppler Ultrasonography ◽  
Digital Ulcers ◽  
Healthy Controls ◽  
History Of

Background:Together with autoimmune-inflammation and fibrosis, microvasculopathy is a hallmark of SSc. However, also macrovascular changes may occur including peripheral proliferative vasculopathy. Whether this changes may represent a specific SSc marker with a predictive value remains a matter of debate.[1,2,3]Objectives:To study peripheral macrovascular involvement by color doppler ultrasound (CDUS) with spectral wave analysis (SWA) in a cohort of 40 SSc patients as compared to healthy controls. To further analyze any differences among the SSc population.Methods:Forty SSc patients and 36 healthy controls were examined by CDUS with SWA of both hands. Macrovascular involvement was assessed by measuring the resistivity index (RI) of distal ulnar and radial arteries. Examinations were performed with an Esaote MyLab Twice machine equipped with a linear 10-22 MHz probe. Ultrasound examination was carried out by two independent rheumatologists blinded to clinical conditions of the patients. Statistical analysis was performed by using MaxStat software.Results:The RI index resulted increased in the SSc cohort as compared with healthy controls (left ulnar RI 0.977 vs 0.715; right ulnar RI 0.996 vs 0.699; left radial RI 0.988 vs 0.706; right radial RI 0.999 vs 0.688; p<0.001). SSc patients with an increased RI in one artery were more probable to have an increased RI in the other vessels too (r 2 = 0.35; p<0.01). In addition, 8 out of 40 SSc patients presented left ulnar artery occlusion (UAO) and 7 out of 40 SSc patients presented right UAO, of which 6 presented bilateral UAO. Awaiting to enlarge the cohort for further analysis, descriptive data regarding increased RI at CDUS/SWA and clinical features, including years from onset of the disease, subtype of SSc, mRSS, history of digital ulcers, interstitial lung disease and PAH are described in Table 1.Conclusion:Peripheral macrovascular involvement was observed in SSc patients as compared with healthy controls. Further studies will determine whether this feature may have specificity for diagnosis/prognosis in SSc.References:[1]Lescoat A, Yelnik CM, Coiffier G et al. Ulnar Artery Occlusion and Severity Markers of Vasculopathy in Systemic Sclerosis: A Multicenter Cross-Sectional Study. Arthritis Rheumatol. 2019;71:983-990.[2]Lescoat A, Coiffier G, Rouil A et al. Vascular Evaluation of the Hand by Power Doppler Ultrasonography and New Predictive Markers of Ischemic Digital Ulcers in Systemic Sclerosis: Results of a Prospective Pilot Study. Arthritis Care Res (Hoboken). 2017;69:543-551.[3]Schioppo T, Orenti A, Boracchi P, De Lucia O, Murgo A, Ingegnoli F. Evidence of macro- and micro-angiopathy in scleroderma: An integrated approach combining 22-MHz power Doppler ultrasonography and video-capillaroscopy. Microvasc Res. 2019;122:125-130.Table 1.Main clinical features of the SSc cohort (n=40) studied by CDUS for macrovascular involvement.SSc cohort (n = 40)Years from onsetrange (35 y – 0 y)mean = 10.5 yAutoantibodiesACA 13/40Anti-TopoI 14/40Other 13/40mRSSrange (0 -30)mean = 3ILD17/40PAH7/40Capillaroscopy patternEarly 10/40Active 11/40Late 6/40History of digital ulcers16/40Left ulnar IR0.977Left radial IR0.988Right ulnar IR0.996Right radial IR0.999Disclosure of Interests:None declared.

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.

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