Twenty-one myasthenic patients were treated with high-dose daily prednisone regimen at Seoul National University Hospital from May 1983 to January 1985. Observations in relation to dosage, drug schedule, clinical responses, and sideeffects led us to following conclusions concerning the management of myasthenia gravis with steroid. In addition, factors influencing the result were considered. 1) Among 21 patients, twenty(95%) showed clinical improvement of variable degrees. 2) Significant improvement could be expected especially in male patients, in older patients, and in those with duration of myasthenia gravis less than 6 months prior to treatment. Performance of thymectomy or thymothymectomy, thymic pathology, and clinical grade at the time of therapy were not considered to affect the outcome. 3) Ten cases (48%) suffered from initial exacerbations, most of which occurred within the first four days of treatment. Steroid-induced crises developed in four cases with preexisting brittle or severe generalized myasthenia. 4) Period taken to show the initial response and the maximum improvement varied widely but majority of them occurred within 15 days and 70 days respectively. 5) Off-day weakness during the alternate-day schedule required special cautions and, if persitent, immediate return to daily schedule was desirable. 6) Thymectomy prior to steroid treatment was not always necessary but, when both regimens were scheduled together, preparation therapy with steroid thought to be more favorable. 7) Aside from initial exacerbations side-effects during the treatment were not remarkable, all of which disappeared with reduction of dosage.
Appointments and Schedules ; Humans ; Male ; Myasthenia Gravis* ; Pathology ; Prednisone ; Seoul ; Thymectomy

Myasthenia gravis and pemphigus are both considered to have an autoimmune basis. Although immunological and clinical studies have been performed on large numbers of patients with myasthenia gravis, the coexistence of myasthenia gravis and pemphigus foliaceus has rarely been described. We recently have the opportunity to study a 33-year-old female patient having both of these autoimmune diseases confirmed by various diagnostic methods. This rare coexistence of myasthenia gravis and pemphigus foliaceus has not been previously documented in Korea.
Adult ; Case Report ; Female ; Human ; Myasthenia Gravis/*complications/immunology/pathology ; Pemphigus/*complications/immunology/pathology

Ocular myasthenia gravis(OMG)is an autoimmune disease caused by neuromuscular junction transmission disorders and manifested mainly as fluctuating blepharoptosis and diplopia,with the extraocular muscles as the main involveed sites.While the pathogenesis of OMG remains unclear,some antibodies,complements,and cytokines may be the contributing factors.The diagnosis and treatment of OMG have been defined in recent years.This article reviews the pathogenesis,diagnosis,and treatment of OMG.
Antibodies ; Complement System Proteins ; Cytokines ; Humans ; Myasthenia Gravis ; diagnosis ; pathology ; therapy ; Oculomotor Muscles ; pathology

Myasthenia gravis is a chronic disease characterized by skeletal muscle weakness. The etiology of myasthenia gravis is not clarified but recently it has been suggested that it is an autoimmune disease. Myasthenia gravis is not difficult to diagnose but the therapy remains as an assignment. The author observed in 10 cases of myasthenia gravis attending the eye department of B.N.U. hospital for 5 years from 1967 to 1971, and review of the literature of recent yeary related to myasthenia gravis, particulary its pathogenesis, clinical course, pathology, physiology, diagnosis, treatment, and prognosis were discussed.
Autoimmune Diseases ; Chronic Disease ; Diagnosis ; Muscle, Skeletal ; Myasthenia Gravis* ; Pathology ; Physiology ; Prognosis

Myasthenia gravis is a chronic disease characterized by voluntary muscle weakness and fatigue. Myasthenia gravis was first described by Thomas Willis in 1672. The etiology of myasthenia gravis is not clarified but recently it has been suggested that it is an autoimmune disease. Ocular myasthenia gravis is characterized clinically by blepharoptosis and external ophthalmopleia. The illness has a tendency to exacerbation and spontaneous remission. The authors observed a case of severe ocular myasthenia gravis which had developed total external ophthalmoplegia. And we reviewed the literature of recent years related to myasthenia gravis, particulary for its etiology, clinical course, pathology, diagnosi, treatment, and prognosis.
Autoimmune Diseases ; Blepharoptosis ; Chronic Disease ; Fatigue ; Muscle, Skeletal ; Myasthenia Gravis* ; Ophthalmoplegia* ; Pathology ; Prognosis ; Remission, Spontaneous

Pure red cell aplasia (PRCA) and hypogammaglobulinemia are paraneoplastic syndromes that are rarer than myasthenia gravis in patients with thymoma. Good syndrome coexisting with PRCA is an extremely rare pathology. We report the case of a 50-year-old man with thymoma and PRCA associated with Good syndrome who achieved complete PRCA remission after thymectomy and postoperative immunosuppressive therapy, and provide a review of the pertinent literature.
Agammaglobulinemia ; Humans ; Middle Aged ; Myasthenia Gravis ; Paraneoplastic Syndromes ; Pathology ; Red-Cell Aplasia, Pure* ; Thymectomy ; Thymoma

BACKGROUND: Thymectomy was known as an effective treatment modality of myasthenia gravis. In this paper, we analyzed the result of thymectomy and the factors affecting the postoperative symptom improvement. MATERIAL AND METHOD: We analyzed the medical records of 50 patients who received the thymectomy for myasthenia gravis from January 1997 to December 2001. RESULT: 39 patients showed symptom improvement. The effect ofthymectomy as a treatment is 78%. There was no statistically significant correlation between postoperative improvement and Sex, Age, the Weight of thymic tissue, preoperative symptom duration, and preoperative mestinon dosage. However, the thymic pathology and low grade preoperative symptoms were affecting the postoperative prognosis. CONCLUSION: Thymic hyperplasia showed good prognosis compared to thymoma. Low grade preoperative symptoms (Group I or IIA) also showed good prognosis. So, early thymectomy is recommendable for the good treatment results of myasthenia gravis.
Humans ; Medical Records ; Myasthenia Gravis ; Pathology ; Prognosis ; Pyridostigmine Bromide ; Thymectomy* ; Thymoma ; Thymus Hyperplasia

Adolescent ; Adult ; Aged ; Child ; Child, Preschool ; Female ; Humans ; Male ; Middle Aged ; Myasthenia Gravis ; diagnosis ; pathology ; Phenotype ; Prognosis ; Young Adult

OBJECTIVETo determine whether the clinical and pathologic characteristics and prognoses of myasthenia gravis (MG) patients below 15 years differ from those patients over 50 years after thymectomy.

METHODSWe reviewed the registry material of 30 pediatric and 32 elderly MG patients after thymectomy, including their age, sex, clinical classification, pathological types, and prognoses. The Chi-square test or Wilcoxon's rank-sum test was used to determine the statistical differences between the children and elderly groups.

RESULTSNo significant difference was seen in sex distribution between the two groups. (Chi-square test, P=0.625), but there were differences in clinical classification: more type I was observed in the pediatric group than in the elderly group, but more type II or III was seen in the elderly group (Wilcoxon's rank-sum test, P<0.001). As to pathological types, the pediatric group was also significantly different from the elderly group (Chi-square test, P<0.01). All of the patients (100%) in the pediatric group had thymus hyperplasia, but in the elderly group more than half (56.26%) were found to have thymoma (benign or malignant). The prognoses after thymectomy were better in the pediatric group than in the elderly group (Wilcoxon's rank-sum test, P<0.001).

CONCLUSIONSBecause the prognoses are generally better than those of the elderly patients, we should be careful when operating on pediatric patients of ocular type. The elderly patients tend to receive more aggressive treatment because of more severe generalized types often associated with thymoma and poor prognoses. Both pediatric and elderly patients are seldom associated with other autoimmune disease.

Adolescent ; Aged ; Child ; Child, Preschool ; Female ; Humans ; Male ; Middle Aged ; Myasthenia Gravis ; mortality ; pathology ; surgery ; Prognosis ; Thymectomy

Adult ; Antigens, CD1 ; metabolism ; Female ; Histiocytosis, Langerhans-Cell ; complications ; metabolism ; pathology ; surgery ; Humans ; Mediastinoscopy ; Myasthenia Gravis ; complications ; metabolism ; pathology ; surgery ; S100 Proteins ; metabolism ; Thymus Gland ; metabolism ; pathology ; surgery