scholarly journals Thymoma resection in a patient with a history of extended thymectomy for myasthenia gravis without thymoma

2016 ◽  
Vol 30 (2) ◽  
pp. 214-217
Author(s):  
Yasuhiro Otsuki ◽  
Kotaro Kameyama ◽  
Tomoaki Matsuoka ◽  
Kenji Kimura ◽  
Norihito Okumura
Keyword(s):  
Myasthenia Gravis ◽  
Extended Thymectomy ◽  
History Of

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 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 Predictive factors of myasthenic crisis after extended thymectomy for patients with myasthenia gravis

2015 ◽  
Vol 48 (5) ◽  
pp. 705-709 ◽  
Author(s):  
Takeshi Ando ◽  
Mitsugu Omasa ◽  
Takayuki Kondo ◽  
Tetsu Yamada ◽  
Masaaki Sato ◽  
...  
Keyword(s):  
Myasthenia Gravis ◽  
Predictive Factors ◽  
Myasthenic Crisis ◽  
Extended Thymectomy

Sudden Onset of Diplopia and a Skeletal Muscle Antibody

2021 ◽  
pp. 160-162
Author(s):  
John R. Mills
Keyword(s):  
Computed Tomography ◽  
Myasthenia Gravis ◽  
Patent Foramen Ovale ◽  
Time Course ◽  
Vision Loss ◽  
Sudden Onset ◽  
Foramen Ovale ◽  
Clinical Disorder ◽  
History Of ◽  
The Right

A 62-year-old man with a history of migraine came to the emergency department with sudden onset of horizontal diplopia and, subsequently, bilateral ptosis. He noted feeling unsteady when walking. He reported that the diplopia worsened throughout the day. He had a history of hepatitis C infection. He had some vision loss in his left eye, which was thought to relate to a retinopathy. He disclosed that he had a history of cold feet and had notably high arches. He had a pacemaker because of syncope attributed to sick sinus syndrome. Computed tomography angiography of the head and neck were ruled negative for intracranial stenosis, occlusions, or aneurysms. Computed tomography of the head indicated a tiny lacunar infarct in the right caudate head. Magnetic resonance imaging of the brain identified a tiny, periaqueductal, enhancing abnormality in the right midbrain that was thought to be likely ischemic, but there was some concern for a demyelinating or inflammatory lesion. Cerebrospinal fluid evaluation indicated an increased protein concentration. Serologic evaluation for myasthenia gravis striational antibodies were positive at a titer of 1:240. Serum protein studies indicated the presence of polyclonal hypergammaglobulinemia. Myasthenia gravis was effectively ruled out. Given the hyperacute time course, the patient’s clinical disorder was most probably explained by an ischemic stroke that affected the oculomotor nuclei regions causing ptosis and ophthalmoparesis. On follow-up, the patient was discovered to have a patent foramen ovale. Whether the patent foramen ovale was a contributing factor to the stroke is uncertain. The recurrence rate in this setting is thought to be low relative to other causes of stroke. Ultimately it was decided to not close the patent foramen ovale and to maintain the patient on clopidogrel and adult low-dose aspirin. The onset of diplopia is typically sudden, but this occurs exclusively with vascular pathologic processes. Diplopia that appears intermittently with diurnal variation suggests the possibility of a neuromuscular junction disease such as myasthenia gravis.

scholarly journals Response to eculizumab in patients with myasthenia gravis recently treated with chronic IVIg: a subgroup analysis of REGAIN and its open-label extension study

2020 ◽  
Vol 13 ◽  
pp. 175628642091178 ◽  
Author(s):  
Saiju Jacob ◽  
Hiroyuki Murai ◽  
Kimiaki Utsugisawa ◽  
Richard J. Nowak ◽  
Heinz Wiendl ◽  
...  
Keyword(s):  
Myasthenia Gravis ◽  
Subgroup Analysis ◽  
Phase Iii ◽  
Ivig Treatment ◽  
Open Label ◽  
Assessment Scores ◽  
Open Label Extension ◽  
Similar Patient ◽  
Antiacetylcholine Receptor Antibody ◽  
History Of

Background: In the phase III eculizumab for refractory generalized myasthenia gravis REGAIN study [ClinicalTrials.gov identifier: NCT01997229] and its open-label extension (OLE) [ClinicalTrials.gov identifier: NCT02301624], patients with treatment-refractory antiacetylcholine receptor antibody-positive generalized myasthenia gravis had clinically meaningful improvements with eculizumab versus placebo. This subgroup analysis evaluated data from patients with a recent history of chronic intravenous immunoglobulin (IVIg) use before study entry. Methods: The subgroup comprised patients who had received IVIg at least four times in 1 year, with at least one IVIg treatment cycle during the 6 months before the first REGAIN study dose. Data from REGAIN and the OLE were analyzed. Response to eculizumab versus placebo was assessed using four validated, disease-specific measures. Incidences of exacerbations and safety endpoints were recorded. Results: The subgroup had similar patient and disease characteristics as the overall REGAIN population. Clinical assessments showed sustained eculizumab efficacy during REGAIN and the OLE over 18 months. Patients receiving placebo in REGAIN experienced rapid improvements in assessment scores when treated with eculizumab in the OLE. There was a lower rate of disease exacerbations with eculizumab than with placebo during REGAIN, and eculizumab was well tolerated. Conclusion: Eculizumab treatment, compared with placebo, results in meaningful clinical improvements and fewer disease exacerbations for patients who previously received chronic IVIg. Trial registration: REGAIN [ClinicalTrials.gov identifier: NCT01997229]; REGAIN open-label extension [ClinicalTrials.gov identifier: NCT02301624].

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