• Title/Summary/Keyword: Myasthenia gravis

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A Case of Myasthenia Gravis Diagnosed by Repetitive Hypoglossal Nerve Stimulation Test (반복 설하신경 자극검사로 진단한 중증근무력증 1예)

  • Jang, Il-Mi;Lee, Kyung-Bok;Roh, Hakjae;Ahn, Moo-Young;Yang, Kwang-Ik;Sung, Ki-Bum
    • Annals of Clinical Neurophysiology
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    • v.8 no.1
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    • pp.74-77
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    • 2006
  • Repetitive nerve stimulation is a simple and widely used technique to demonstrate neuromuscular transmission defect. A significant decremental response for repetitive hypoglossal nerve stimulation was obtained from the surface recordings in the tongue of a patient with dysarthria and dysphagia. Repetitive hypoglossal nerve stimulation test may be useful in diagnosis of myasthenia gravis with bulbar symptoms only. We utilized repetitive hypoglossal nerve stimulation with tongue recordings and diagnosed a case of myasthenia gravis.

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A Clinical Study of Management In Myasthenia Gravis (중증 근무력증 환자의 임상적 고찰)

  • Kim, Hun;Lee, Du-Yeon;Jo, Beom-Gu;Hong, Seung-Rok
    • Journal of Chest Surgery
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    • v.20 no.1
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    • pp.112-127
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    • 1987
  • Myasthenia gravis is a neuromuscular transmission function disorder characterized by fatigue and weakness of voluntary muscles. This muscular weakness is intensified by activity and stress, and improved by the use of anticholinesterase compounds. It was initially described by Erb in 1879 and later named myasthenia gravis by Jolly in 1895. Although the pathogenesis is Known to be an autoimmune related reduction in the number of available acetylcholine receptors at neuromuscular junctions, the role of thymus in myasthenia gravis is still unclear and under investigation. Thymectomy in the management of myasthenia gravis has become increasingly important since Dr. Blalock observed in 1939 that some patients with thymic tumors and myasthenia gravis improved following thymectomy. A clinical study of 102 cases of myasthenia gravis was performed at Yonsei University College of Medicine. Seoul, Korea from Jan. 1976 to Jun. 1986. In order to determine which factors are of prognostic significance, attention is focused upon pre-operative patient evaluation, problems in operative and post-operative care, and long-term follow-up observations. The results were as follows: 1. The sex distribution was 67 females and 35 males, the mean age of onset was 28.95*1.69 years, and the maximal incidence occurred between 21 and 40 years of age [56 cases: 54.9%]. 2. Clinical manifestations of ocular symptoms were seen to 70 patients [68.6%] extremities weakness in 33 [32.3%], bulbar weakness in 29 [28.4%], and dyspnea in 13 [12.7%]. 3. Study cases more than two thirds were classified as mild types [MG 1 and MG 11A] and 6 cases as grave [MG 1V] based on the modified Osserman`s classification system, 4. Thymectomy was performed in 19 cases which presented in severe myasthenia symptoms and showed no improvement with cholinergic drugs. Histologic examination of the excised thymus glands revealed no abnormalities in 4 cases, thymic hyperplasia in 5, benign thymoma in 5, and malignant thymoma in 5. 5. Immediate post-operative complications included 2 cases of pneumothorax which were treated by tube thoracostomies, there was no operative mortality. 6. The response to cholinergic drugs in 36 cases younger than 20 years old and in 27 cases older than 40 years was relatively poor, while that in 35 cases between the ages of 21 and 40 years old was good. 7. Thirty of 39 cases in groups IIB, III & IV improved markedly with medical or surgical management while only 16 of 59 cases in the mild groups [I and IIA] improved, almost all surgical cases improved in all categories. 8. There were 5 deaths. occurring between 7 months and 3 years 3 months of treatment of myasthenia gravis. The causes of death were myasthenic crisis in 2 cases, respiratory failure due to candidiasis & radiation pneumonitis in one case, cerebral hemorrhage due to high blood pressure in two case.

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Guillain-Barré Syndrome in a Patient with Myasthenia Gravis (중증근무력증 환자에서 발생한 길랑-바레 증후군 1례)

  • Seo, Soo Kun;Park, Moon Ho;Park, Beom Sick;Kim, Byung Jo;Park, Min Gyu;Park, Gun Woo;Lee, Dae Hie
    • Annals of Clinical Neurophysiology
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    • v.2 no.2
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    • pp.135-138
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    • 2000
  • Both myasthenia gravis and Guillain-$Barr{\acute{e}}$ syndrome are autoimmune disorder, but it is very rare that both of them occur together. A 53-year old woman with history of myathenia gravis complained of weakness of extremities, worsening progressively for several days. Electrophysiologic study showed findings of motor axonal neuropathy without sensory involvement. She became improved in aspect of clinical symtoms and electrophysiologic findings after infusion of immunoglobulin. This is a case of Guillain-$Barr{\acute{e}}$ syndrome in a patient with myasthenia gravis.

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Surgical treatment of myasthenia gravis: a report of 10 cases (중증 근무력증의 외과적 요법: 10례 보)

  • Jo, Dae-Yun;Kim, Yong-Jin;Kim, Ju-Hyeon
    • Journal of Chest Surgery
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    • v.17 no.2
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    • pp.292-298
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    • 1984
  • Myasthenia 8ravis is a disorder of neuromuscular function due to a reduction of available acetylcholine receptors at the neuromuscular junction. Typically, the muscle weakness is worse after effort and improved by rest. In 1939, Blalock and associated reported a case of patient with myasthenia gravis who was successfully treated by thymectomy. Since then, operation has become increasingly important in the management of this disease. From 1968 to 1983, 10 cases of myasthenia gravis were operated at the Department of Thoracic and Cardiovascular Surgery,College of Medicine, Seoul National University. 1.Among 10 cases, 4 were male and 6 were female. 2.Thymectomy was performed in 9 cases, and 1 case of malignant thymoma with deeply infiltration to adjacent structure was not resectable. 3.There was 1 operative mortality with respiratory failure. 4.During follow-up period, most of them showed symptomatic improvement, but complete remission was not noticed in any case.

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A Case of Generalized Myasthenia Gravis Combined with Psoriasis (전신성 건선이 병합된 중증 근무력증 1예)

  • Park, Jae Han;Seok, Jung Im;Lee, Dong Kuck
    • Annals of Clinical Neurophysiology
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    • v.8 no.2
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    • pp.203-205
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    • 2006
  • There have been several reports about coexistence of myasthenia and other autoimmune disease. Psoriasis is a papulosquamous disease defined by erythematous plaques with a silvery scale and a T-cell-mediated autoimmune disease. We report a case of a 49-year-old man with generalized myasthenia gravis (MG) superimposed by psoriasis. MG was diagnosed by clinical symptoms, increased acetylcholine receptor antibody titer and repetitive nerve stimulation test. Psoriasis was diagnosed by clinical manifestations and specific skin biopsy findings. MG and psoriasis are both autoimmune diseases. The coexistence of MG and psoriasis suggest a close connection of pathogenesis.

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Clinical Study of the Myasthenia Gravis (중증 근무력증의 임상적 고찰)

  • 지청현
    • Journal of Chest Surgery
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    • v.24 no.11
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    • pp.1125-1132
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    • 1991
  • Myasthenia gravis is a disorder of neuromuscular transmission which is characterized fatigue and weakness of the voluntary muscle. From 1985. 1 to 1991.6, 34 patients underwent managed including 12 patient thymectomy, at the kyunghee medical center. A clinical analysis was performed and following result was obtained. Among the 34 patients, male to female ratio was 11 : 23 and the age was ranged from 2 years to 63 years. Thymectomy was done in 12 case and 1 case of malignant thymoma was not resectable. There were 2 deaths after operation due to respiratory failure and 1 death dur to gradually progression. THe effect of thymectomy in mtasthenia graves was 66.7% (8cases). The prognostic factors were severity of the disease and histopathological findings. Others were not related to the prognosis of the mayasthenia gravis. The severity of the myasthenia gravis was classified by Osserman modified classification.

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Clinical study of Myasthenia Gravis associated with other autoimmune diseases (자가면역질환과 동반된 중증근무력증의 임상적 연구)

  • Kim, Byung Jo;Koh, Seong Beom;Park, Min Kyu;Park, Kun Woo;Lee, Dae Hie
    • Annals of Clinical Neurophysiology
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    • v.3 no.1
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    • pp.21-25
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    • 2001
  • Background & Object : Myasthenia gravis(MG) is an autoimmune disease due to binding of antibody to acetylcholine receptors on the muscle membrane. It is well known that other autoimmune diseases infrequently accompany myasthenia gravis. The aim of this study was to evaluate the clinical significance of associated autoimmune diseases(AAD) and compare prognosis between MG with AAD and MG without AAD. Method : A total of 65 MG patients(24 men and 41 women) were enrolled at this study. From the clinical records of these patients, we investigated the clinical characteristics and prognosis of MG with AAD and compared these data with those of MG without other such diseases. Results : AAD were found in 10 of 65 cases(15%). 9 cases of 10 MG with AAD were generalized MG type. The most common disease was thyroid disorder. The rate of AAD was higher in thymic abnormal patients. There was no significant remission rate difference between MG with AAD and MG without AAD, but the percentage of patients experienced crisis was higher in MG with AAD. Conclusion : The occurrence of AAD may suggest a more generalized autoimmune disturbance that could be associated with a less favorable prognosis.

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Thymectomy in Patients with Myasthenia Gravis (흉선절제로 치료한 중증 근무력증)

  • 조광현
    • Journal of Chest Surgery
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    • v.18 no.4
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    • pp.872-880
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    • 1985
  • Myasthenia gravis is a neuromuscular transmission disorder characterized by fatigue and weakness of voluntary muscles. Although the pathogenesis is known as reduction of available acetylcholine receptors at neuromuscular junctions by autoimmune attack, the thymic role in myasthenia gravis is still unclear and under investigation. But thymectomy in the management of myasthenia gravis has become increasingly important since the first successful operation with remission of symptoms in 1939 by Blalock. From January 1983 to June 1985, authors performed 17 thymectomies for patients with myasthenia gravis. Among them, 12 patients were free from thymoma [Croup A] and 5 were coupled with thymoma [Group B]. The results were as follows: 1] Sex distribution was 11 females and 6 males. Mean age of the patients was 32.2 year old. Sex and age distribution by the Group A and B are shown Table 1. 2] Clinical manifestations of ocular symptoms were seen in 5 patients [88.2%], extremity weakness in 13 patients, bulbar weakness in 12 patients and dyspnea in 6 patients. According to the Osserman`s classification, 5 patients were in group IIA, 6 in IIB and 6 in IIC. 3] Pre-operatively, all patients were positive response to the anti-cholinesterase test and 12 patients [92.3%] revealed positive findings in electromyography [EMC] which was done in 13 patients. 4] The postoperative complications were respiratory distress in 3 patients, myasthenic crisis in 2 patients and wound disruption in one patients. 5] Pathologic examination of the thymus showed hyperplasia in 10 patients [90%] and thymoma in 5 patients, of which 4 were mixed type with invasion to the adjacent tissues and one lymphocytic type without invasion. Normal thymus was noticed in only 2 patients. 6] In postoperative evaluations, among the 12 patients c free from thymoma [Group A], complete remission of symptoms was noticed in 3 patients and improvement in 7 patients. But among the 5 patients coupled with thymoma [Group B], only one patients showed improvement [Table 8]. Therefore, remission and clinical improvement were noticed in 11 patients [64.7%] of the all and complete remission was noticed in 3 patients [17.6%].

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A Case of Myasthenia Gravis Combined with Mediastinal Leiomyosarcoma and Stevens-Johnson Syndrome (종격 평활근육종 및 Stevens-Johnson증후군과 동반된 중증 근무력증 1예)

  • Lee, Dong-Kuck;Kweon, Young-Mi
    • Annals of Clinical Neurophysiology
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    • v.6 no.1
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    • pp.43-47
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    • 2004
  • We report a case of 36-year-old woman with myasthenia gravis (MG) combined with mediastinal leiomyosarcoma (LMS) and Stevens-Johnson syndrome (SJS). She was admitted to ICU with the symptoms of acute onset headache, diplopia, ptosis, dysphagia, general weakness, and respiratory difficulty for several days. Physical examination revealed tachypnea, decreased breath sounds and dullness to percussion in right lower chest. Neurologic examination showed ptosis, diplopia, decreased gag reflexes, and generalized proximal weakness. Laboratory studies revealed increased serum acetylcholine receptor antibodies and positive Tensilon test. Chest CT showed a huge mass in the right middle mediastium but no evidence of thymic enlargement. Mediastinal LMS was diagnosed by ultrasound-guided needle biopsy. The myasthenic symptoms were fluctuated in spite og intravenous immunoglobulin, plasmapheresis, and corticosteroid. During therapy, SJS developed. She died 4 months after the onset of the myasthenic symptoms despite the chemotherapy for LMS.

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Comparative Study for Maximal Thymectomy of Myasthenia Gravis (중증 근무력증에 대한 흉선조직의 공범위 절제술의 비교연구)

  • Park, Chang-Gwon;Jo, Chang-Hun
    • Journal of Chest Surgery
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    • v.25 no.10
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    • pp.1035-1040
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    • 1992
  • Myasthenia gravis is an autoimmune disease in which the thymus gland is thought to play a central role in the pathogenesis. Thymectomy is now standard therapy, and improvements in surgical techniques, anesthesia, and respiratory care and the use of plasmapheresis have markedly reduced the operative morbidity. We experienced 7 myasthenia gravis with maximal thymectomy from November 1989. As compared with the results of previous classic transsternal thymectomy group the 25 patients who were operated from June 1979 to December 1991[Group B], excellent surgical result was obtained in maximal thymectomy group[Group A]. During follow-up period that ranged from a month to 10.7 years[mean 2 years], the remission rate for the entire group was 15.6 percent and an additional 71.9 percent had improvement[87.5 percent benefited]. In Group A, the remission rate was 42.9 percent with 100 percent of the patients benefiting from operations. But in Group B, 8 percent had remission and a total of 80 percent benefited. In both Groups, there was no operative mortality but there was one case`s mortality in Group B during follow-up period.

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