• Title/Summary/Keyword: Myasthenia gravis

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A Study on Surgical Treatment of Thymoma -Analysis of 18 Cases- (흉선종의 외과적 치료에 대한 연구 -18례 분석-)

  • 이형렬
    • Journal of Chest Surgery
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    • v.27 no.4
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    • pp.303-309
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    • 1994
  • Eighteen patients with thymoma have undergone surgical treatment at Pusan National university Hospital from 1983 to 1993. The mean age of the patients was 47.3$\pm$13.3 years old and male to female ratio was 1.6 : 1. Eleven of the patients[61.1%] with thymoma had myasthenia gravis; 12 cases[66.7%] were encapsulated and 6[33.3%] invasive. Operation was complete resection in 14[77.8%] of the patients, partial resection with residual tumor in 3[16.7%], and simple biopsy in one[5.5%]. Adjuvant radiotherapy was administered in 5 of 6 invasive thymoma. Postoperatively one patient died for excessive bleeding and sepsis. A tumor relapse was observed in one patient who had invasive thymoma and underwent partial resection followed by postoperative radiotherapy. The overall 5-year survival rate was 88.2$\pm$ 7. 9%.The presence of myasthenia gravis is not considered as an adverse factor for survival but it contributes to early discovery of associated thymoma.

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Video-Assisted Thoracoscopic Thymectomy (흉강내시경을 이용한 흉선 절제술)

  • Jang, Taek-Hui;Baek, Hui-Jong
    • Journal of Chest Surgery
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    • v.28 no.1
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    • pp.78-80
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    • 1995
  • We report the thoracoscopic resection of thymus and pericardial fat tissue in a patient who was in the setting of late severe myasthenia gravis[Osserman`s Group II-C-2 and previous tracheostomy state. The patient was 33-year-old female. She had been supported with mechanical ventilator for 88 days and suffered from recurrent pneumonia. Our first aim was to weaning her from mechanical ventilator. Traditional methods such as median sternotomy or transcervial thymectomy or transsternal sternotomy were difficult because of the anticipating complications of mediastinitis or morbidity, especially chest pain, following thoracotomy. We could wean her from the mechanical ventilator at postoperative 9 days. So, we concluded that video-assisted thoracoscopic thymectomy is a useful alternative tool in this case.

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Surgical Treatment of Myasthenia Gravis (중증 근무력증의 외과적 치료)

  • 손영상
    • Journal of Chest Surgery
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    • v.21 no.3
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    • pp.454-461
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    • 1988
  • Myasthenia gravis is a functional neuromuscular disorder with characteristic voluntary muscle weakness. The role of thymus in pathogenesis of this disorder has become apparent that thymectomy in treatment has gained acceptance. Between January 1976 and June 1987, twenty patients underwent thymectomy for myasthenia gravis at the Department of Thoracic and Cardiovascular Surgery, College of Medicine, Korea University. A clinical study that is focused on the factors affecting the operative results was analyzed and the following results were obtained. Among the 20 patients, male to female ratio was 8:12 and the age of onset was ranged from 3 years to 67 years. The chief complaints in order of frequency were as follows; ocular symptoms such as ptosis and diplopia[7 cases], general weakness[4 cases], swallowing difficulty[3 cases], dyspnea[3 cases], dysphasia[1 case], headache[1 case] and dizziness[1 case]. The severity of disease was classified by modified Osserman`s method that Group IIa was 8 cases, Group IIb; 7 cases, Group IIc; 3 cases and Group I; 2 cases. In histopathology of thymus, the most frequent finding was hyperplasia[11 cases] followed by thymoma[4 cases], normal tissue[3 cases] and malignant` thymoma[2 cases]. There were two cases of postoperative complications; one case was wound infection and the other was mediastinitis. One case of malignant thymoma died due to respiratory failure with pulmonary metastasis. There was 16 cases[80%] of improvement after thymectomy as follows; complete remission was 4 cases[20%], marked improvement was 9 cases[45%] and subjective improvement only was 3 cases[15%]. The effect of severity and duration of disease on operative result has statistically significant. The effect of thymus histopathology on operative result was not statistically significant. But there were comparable results between thymoma cases and non-thymoma cases.

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A Case of Acquired Myasthenia Gravis in German Shepherd Dog (개에서 발생한 후천성 중증근육무력증 일례)

  • Lee Sang-Gwan;Hoh Woo-Pil;Kim Youn-Ju;Kim Tae-Wan;Yoo Jong-Hyun;Eom Ki-Dong;Oh Tae-Ho;Lee Keun-Woo
    • Journal of Veterinary Clinics
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    • v.22 no.4
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    • pp.392-395
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    • 2005
  • A three-year old intact female German shepherd dog weighing 32kg was presented with the history of regurgitation and vomiting. Physical examination revealed a thin body condition, hoarse voice, hypersalivation, and pelvic limb weakness. In radiograph, meagaesophagus was identified. The neostigmine challenge test was positive. The titer of acetylcholine receptor (AChR) antibody was 1.58 nmol/L (reference range, <0.6nmol). Therefore, the dog was diagnosed to be suffering with chronic generalized form of acquired myasthenia gravis and followed by treatment with pyridostigmine bromide, 1mg/kg, q12 hrs. Treatment improved the pelvic limb weakness, however intermittent regurgitation and vomiting persisted. This resulted in aspiration pneumonia f3r which antibiotics were administered However, the dog was euthanized due to poor management and owner's request.

Coexistence of Myasthenia Gravis and Systemic Lupus Erythematosus (중증 근무력증과 전신성 홍반성 낭창의 동반 발생)

  • Heo, Jae-Hyeok;Min, Ju-Hong;Cho, Joong-Yang;Kim, Nam-Hee;Lee, Kwang-Woo
    • Annals of Clinical Neurophysiology
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    • v.7 no.2
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    • pp.93-96
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    • 2005
  • Background: Myasthenia gravis (MG) and systemic lupus erythematosus (SLE) are well recognized to coexist and have some similarities in immunologic, clinical and serologic findings. Despite several reports of the association with autoantibodies and thymectomy in these disorders, the pathomechanism of coexistence remains to be elucidated. Objective: We aimed to investigate the relationship of MG and SLE through overall features of patients with both disorders;: clinical, laboratory, and electrophysiological findings. Materials and Methods: We reviewed the medical records of 6 consecutive patients with MG and SLE (2 men, 4 women, ages 17-51, mean 30.5 years, Seoul National University Hospital, from 1998 to 2005). Results: Three patients who developed SLE first, had ocular type of MG and 2 were children showing much severe and recurrent SLE features and only 1 patient had thymic hyperplasia. The other 3 developed MG first and they were generalized type and none underwent thymectomy. In addition, the development of MG or SLE was not coincident with remission or improvement of another disorder. Conclusion: The coexistence of SLE and MG may support the hypothesis of two different antibody populations modulated by thymus in the opposite extremesThis report suggests that the systemic and extensive autoimmune response in preceding MG or SLE may effect the development of the other disorder followed, while. the coexistence of two disorders cannot be explained by the hypothesis of two different antibody populations modulated by thymus in the opposite extremes The role of thymectomy and the theorectical subsequent effect on the development of SLE have been debated with controversy. However, SLE occurred without thymectomy in MG and these disorders did not develop in the quiescent period of another disorder. Therefore, the other pathomechanism for the coexistence of MG and SLE should be elucidated.

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A Clinical Study of 52 Patients with Myasthenia Gravis Syndrome (중증 근무력증 52례에 대한 임상적 고찰)

  • Park, Gun-Ju;Hah, Jung-Sang;Lee, Jun;Do, Hyun-Cheol;Park, Seung-Kweun;Suh, Sang-Dug;Kee, Byung-Soo;Kim, Jin-Kook
    • Journal of Yeungnam Medical Science
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    • v.13 no.1
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    • pp.86-96
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    • 1996
  • The authors experienced 52 patients with myasthenia gravis who were diagnosed at the Department of Neurology, Yeungnarn University Hospital from August 1985 to January 1996. The following results were obtained through diagnostic evaluation and treatment. 1. The ratio of male to female was 1:1.7 and the most prevalent age group was second decade. 2. The most common initial presentation symptom was ocular(71.2%) and the peak incidence group was stage I (69.3%) according to the modified Osserman's classification. 3. In 16 patients(30.8%), it took more than a year to diagnose due to symptoms which were relapsed and remitting. 4. Of 52 patients, 2 cases were associated with thyroid disease(3.8%) and 2 with insulin-dependent diabetes mellitus(3.8%). 5. All of those who received anticholinesterase and corticosteroid therapy were improved with the exception of 5 cases which were improved after thymectomy and/or plasmapheresis. 6. Spontaneous remission was occured in 2 cases(3.8%) of ocular myasthenia gravis. And 1 case of group II. was cured through thymectomy and long-term corticosteroid therapy.

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Robot-assisted Thymectomy with the 'da Vinci' Surgical System in a Patient with Myasthenia Gravis -A case report- (중증 근무력증 환자에서 da Vinci 로봇을 이용한 흉선절제술 -1예 보고-)

  • Yi, Jung-Hoon;Jeong, Sang-Seok;Woo, Jong-Soo;Cho, Gwang-Jo;Bang, Jung-Hee;Choi, Pill-Jo;Park, Kwon-Jae
    • Journal of Chest Surgery
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    • v.43 no.5
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    • pp.557-561
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    • 2010
  • In the treatment of myasthenia gravis, thymectomy is generally accepted as the standard of therapy. For thymectomy, there have been various conventional open approaches including sternal splitting, but recently minimally invasive approaches have been increasingly applied. A 28-year-old man presenting with weakness of both hands and fatigability was diagnosed as having myasthenia gravis with thymic hyperplasia. He underwent a robot-assisted thymectomy with the 'da Vinci' surgical system. Through the right thoracic cavity, two thirds of the thymic gland was dissected, and the remainder was resected through the left; these procedures took, respectively, 1 hour and 30 minutes. The patient was discharged on the 8th postoperative day without complications. The minimally invasive approach with the 'da Vinci' surgical system is emerging as a popular choice and various advantages have been reported. Here we report the first successful case of robot-assisted thymectomy.

In vitro Selection of the 2'-Fluoro-2'-Deoxyribonucleotide Decoy RNA Inhibitor of Myasthenic Autoantibodies

  • Seo, Hwa-Seon;Lee, Seong-Wook
    • Journal of Microbiology and Biotechnology
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    • v.10 no.5
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    • pp.707-713
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    • 2000
  • Myasthenia gravis (MG) is caused mainly by autoantibodies directed against acetylcholine receptors located in the postsynaptic muscle cell membrane. Using in vitro selection techniques, we isolated an RNA containing 2'-fluoro pyrimidines that can specifically and avidly ($K_d$ ~25 nM) bind rat monoclonal antibody called mAb198, which recognizes the main immunogenic region on the acetylcholine receptors. This RNA can act as a very effective decoy and block mAb198 binding to the receptors in vitro. Furthermore, this RNA decoy can prevent the antigenic modulation of the acetylcholine receptor caused by mAb198 in human muscle cell cultures with and $IC_{50} $of approximately $2.4{\mu}M$. These results indicate that the RNA selected in this study is a more potent decly inhibitor of myashthenic antibodies than the previously identified RNA with 2'-amino pyrimidines [11]. Moreover, this RNA cross-reacts with autoantibodies from patients with MG and can protect human cells from the effects of these antibodies. These observations have important implications for developing an antigen-specific treatment of autoimmune diseases including MG, which is based on decoy RNAs selected in vitro.

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A Clinical Analysis of Thymoma (흉선종의 임상적 고찰)

  • 이헌재
    • Journal of Chest Surgery
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    • v.23 no.4
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    • pp.715-719
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    • 1990
  • Thymoma is defined as a neoplasm of the epithelialreticular framework cells of the thymus. The treatment of choice and the prognosis for patients with thymoma are still controversial subjects due to lack of a uniform histological classification and standardized criteria for typing thymic tumors. Between June 1985 and May 1989, eight patients underwent thymomectomy at the Department of Thoracic and Cardiovascular Surgery, Korea University Medical Center. A clinical analysis was performed and the following result was obtained. In histologic cell type, epithelial type was 2 cases, lymphocytic type 3 cases, and mixed type was 3 cases. The clinical stage was classified by Masaoka’s classification that Stage I was 4 cases, Stage II; 2 cases, Stage III; 2 cases and Stage IV was none. Myasthenia Gravis was associated with thymoma in 5 patients. In 8 patients, thymomectomy were performed. Adjuvant radiotherapy was performed in one patient with Stage I due to suspicious tumor remnant and in Stage II, III patients routinely. Combined chemotherapy was performed in one patient with Stage III due to local recurrence. We concluded that the most important prognostic factor of thymoma is extent of tumor invasion. And the presence of Myasthenia Gravis is no more prognostic factor. Surgical resection of thymoma is treatment of choice, If local invasion or distant dissemination is present, postoperative radiotherapy and /or combined chemotherapy is recommended.

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Twenty-one-year follow-up of variable onset MELAS syndrome with heteroplasmic nt3243A>G mtDNA mutation: A case report

  • Song, Wung Joo;Lee, Yoon Jin;Kang, Joon Won;Chang, Mea Young;Song, Kyu Sang;Kang, Dae Young;Kim, Sook Za
    • Journal of Genetic Medicine
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    • v.16 no.1
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    • pp.31-38
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    • 2019
  • Mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome is a maternally inherited mitochondrial disorder of which m.3243A>G is the most commonly associated mutation, resulting in an inability to meet the energy requirements of various organs. MELAS poses a diagnostic challenge owing to its multiple organ involvement and great clinical variability due to its heteroplasmic nature. We report three cases from a family who were initially misdiagnosed with myasthenia gravis or undiagnosed. Although there is no optimal consensus treatment approach for patients with MELAS because of the disease's heterogeneity, our 21-year-long therapy regimen of ${\text\tiny{L}}-arginine$, ${\text\tiny{L}}-carnitine$, and coenzyme Q10 supplementation combined with dietary management appeared to provide noticeable protection from the symptoms and complications. Prompt early diagnosis is important, as optimal multidisciplinary management and early intervention may improve outcomes.