Female ; Hemophilia A ; complications ; Humans ; Middle Aged ; Myasthenia Gravis ; complications

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

OBJECTIVETo explore the clinical significance of serum anti-ryanodine receptor (RyR) antibody in the diagnosis of myasthenia gravis (MG).

METHODSThe crude sarcoplasmic reticulum was prepared from rabbit skeletal muscle, and then purified by differential centrifugation to produce the antigen. The serum anti-RyR antibody levels in 74 patients with MG (including 21 patients with comorbidic thymomas) were determined with ELISA.

RESULTSWestern blot demonstrated the presence of RyR in purified crude sarcoplasmic reticulum. The positive rate of anti-RyR antibody was significantly higher in MG patients who had comorbidic thymoma compared with those who had no such comorbidity (P < 0.01). Also, the positive rate was closely correlated with the severity of MG.

CONCLUSIONSerum anti-RyR antibody test is helpful in the diagnosis of MG associated with thymoma and can be used to judge the outcome of MG.

Autoantibodies ; blood ; Humans ; Myasthenia Gravis ; blood ; complications ; immunology ; Ryanodine Receptor Calcium Release Channel ; immunology ; Thymoma ; complications ; Thymus Neoplasms ; complications

A 65-year-old man who had been experiencing diplopia in front and down gaze for 15 days visited our hospital. Hypertropia was noted in the patient's left eye, and limitation of depression was found in the adduction, primary gaze, and abduction. Brain magnetic resonance imaging showed no remarkable findings. Two weeks after the first visit, the patient complained of ptosis in the left eye. An ice test was performed and the ptosis was resolved after the test. Then, anti-acetylcholine receptor binding antibody levels were checked and found to be slightly elevated. We prescribed methylprednisolone per os 24 mg for 2 weeks, and his symptoms improved after the 2-week treatment. Five weeks after his first visit, the patient showed an ortho result in the alternate prism cover test and normal ocular movements. This may be the first case in which ocular myasthenia gravis presented as double depressor palsy, and in such cases, the possibility of ocular myasthenia gravis should be considered to rule out double depressor palsy.
Aged ; Diagnosis, Differential ; Diplopia/*diagnosis/*etiology ; Humans ; Male ; Myasthenia Gravis/*complications/*diagnosis ; Ocular Motility Disorders/diagnosis/etiology ; Strabismus/diagnosis/etiology

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

OBJECTIVETo explore the clinical characteristics and outcome of myasthenia gravis with and without thymoma after operation.

METHODSTwo hundred and forty-three patients with myasthenia gravis surgically treated in our department from 1978 to 2003 were studied retrospectively. The clinical characteristics, complication, remission and survival were compared between myasthenia gravis with and without thymoma.

RESULTSThe patients of myasthenia gravis with thymoma were significantly older (t = 6.138, P = 0.000), had shorter duration of symptom (t = 3.783, P = 0.000), and also had higher myasthenia crisis rates after operation (chi(2) = 64.77, P = 0.000) than those of myasthenia gravis without thymoma. No differences of Osserman classification was found between the two groups (chi(2) = 7.678, P = 0.104). The complete remission rates and partial remission rates of myasthenia gravis with thymoma were significantly lower than those of myasthenia gravis without thymoma at 1 and 3 years (P = 0.049, P = 0.000; P = 0.015, P = 0.010), but no differences at 5 year (P = 0.457; P = 0.699). The survival rates of MG with thymoma were lower than that of MG without thymoma (Log-rank = 18.58, P = 0.000).

CONCLUSIONSThe clinical characteristics are different between myasthenia gravis with and without thymoma. The remission of symptom of myasthenia gravis with thymoma is worse than that of myasthenia gravis without thymoma in the near future, but is similar in the long future. The death rates of MG with thymoma is significantly higher than that of MG without thymoma.

Adolescent ; Adult ; Aged ; Child ; Child, Preschool ; Female ; Humans ; Male ; Middle Aged ; Myasthenia Gravis ; mortality ; surgery ; Retrospective Studies ; Survival Rate ; Thymectomy ; Thymoma ; complications ; Thymus Neoplasms ; complications ; Treatment Outcome

BACKGROUND: Thymoma is frequently associated with myasthenia gravis (MG). However, whether MG is a factor for the outcome of patients with thymoma following complete thymectomy remains unknown. The aim of this study is to investigate the effect of thymoma with MG prognostic factors. METHODS: A retrospective analysis of The Chinese Alliance for Research in Thymomas (ChART) database within 1992-2012 complete cases 875 cases, 20 years follow-up data analysis thymic tumor tissue type credits and MG, Masaoka staging and prognosis, postoperative adjuvant therapy and relationship with the prognosis of surgical removal of the way. RESULTS: Thymic tumor tissue type credit has correlation with MG, difference was statistically significant (χ²=24.908, P<0.001). MG: incidence of B2 type (58/178, 32.58%) > B3 type (65/239, 27.20%) > B1 (27/132, 20.45%) > AB (43/267, 16.10%) > type A, 10.17% (6/59), Masaoka stage has no correlation with MG (χ²=0.365, P=1.365). Survival analysis showed that the WHO classification, Masaoka stage associated with prognosis (P<0.05), and whether the merger MG (χ²=0.113, P=0.736), postoperative adjuvant radiotherapy (χ²=0.380, P=0.538) has nothing to do with the prognosis, postoperative adjuvant chemotherapy is associated with poor prognosis (χ²=14.417, P<0.001). Whether has nothing to do with the prognosis of the thymus resection (χ²=1.548, P=1.548), whether the whole correlated with the curative effect of thymus excision with MG (χ²=24.695, P<0.001). CONCLUSIONS Thymoma patients with MG and extended thymectomy have no correlation with prognosis. Extended thymectomy can improve the effect of MG patients.
Adolescent ; Adult ; Aged ; Aged, 80 and over ; Female ; Humans ; Male ; Middle Aged ; Myasthenia Gravis ; complications ; diagnosis ; surgery ; Prognosis ; Retrospective Studies ; Survival Analysis ; Thymoma ; complications ; Young Adult

Myasthenia gravis (MG) is often complicated by respiratory failure, known as a myasthenic crisis. However, most of the patients who develop respiratory symptoms do so during the late course of disease and have other neurological signs and symptoms. However, in some patients respiratory failure is the initial presenting symptom. We report the case of a 68-year-old woman with MG who presented with isolated respiratory failure as her first presenting symptom. As illustrated by this case, it is important to consider neuromuscular disorders in cases of unexplained respiratory failure.
Acute Disease ; Aged ; Electromyography ; Female ; Humans ; Myasthenia Gravis/*complications/*diagnosis ; Pulmonary Atelectasis/etiology/radiography ; Respiratory Insufficiency/*etiology/*radiography ; Tomography, Spiral Computed

BACKGROUNDThymectomy is considered the most effective treatment in patients with myasthenia gravis. This study aimed to explore the predictors of postoperative myasthenic crisis in patients with myasthenia gravis after thymectomy.

METHODSClinical records of 243 patients with myasthenia gravis who underwent thymectomy were reviewed retrospectively. The following factors were analyzed in relation to the occurrence of myasthenic crisis after thymectomy: gender, age, duration of symptoms, Osserman stage, history of myasthenic crisis, concomitant diseases, preoperative pyridostigmine dose, preoperative steroid therapy, operation approach, operation time, presence of thymoma, major postoperative complications.

RESULTSForty-four patients experienced postoperative myasthenic crisis during the first month after thymectomy. Univariate analysis revealed that Osserman stage (RR = 0.0976, P = 0.000), history of myasthenic crisis (RR = 0.2309, P = 0.012), preoperative pyridostigmine dose (RR = 0.4349, P = 0.016), thymoma (RR = 0.0606, P = 0.000), and major postoperative complications (RR = 0.1094, P = 0.000) were significantly related to postoperative myasthenic crisis. Multivariate Logistic regression analysis showed that Osserman stage (IIb + III + IV) (RR = 0.0953, P = 0.000), thymoma (RR = 0.0294, P = 0.000), and major postoperative complications (RR = 0.0424, P = 0.000) independently predict postoperative myasthenic crisis.

CONCLUSIONOsserman stage (IIb + IIIb + IV), thymoma and major postoperative complications are independent predictors of postoperative myasthenic crisis in patients with myasthenia gravis who underwent thymectomy.

Adolescent ; Adult ; Aged ; Child ; Female ; Humans ; Male ; Middle Aged ; Myasthenia Gravis ; surgery ; Postoperative Complications ; Thymectomy ; adverse effects ; Treatment Outcome ; Young Adult

OBJECTIVESTo study the diagnosis and treatment of thymoma and to assess prognosis factors.

METHODSThe clinical data on 116 patients with thymoma were collected. A retrospective analysis was performed by comparison of their survival rates computed by the actuarial method and rate of recurrence and metastasis.

RESULTSChest radiograph was used chiefly for the preoperative diagnosis of thymoma; myasthenia gravis (MG) (25.0%, 29/116) was the most common paraneoplastic disease. An extensive and radical resection was carried out to reduce the recurrence rate of thymoma with stage I and stage II (chi(2) = 4.941 P = 0.0219). The survival time was prolonged by postoperative radiotherapy and chemotherapy. A strong correlation was noted between the clinical stage and histologic subtype of M-H classification, by which the invasive behavior of thymoma was predicted (r = 0.385, P = 0.007). The 3-, 5-, and 10-year survival rates were 81.2%, 67.9% and 40.5%, respectively. Statistical analysis showed a significant negative correlation between stage and survival rate (r = -0.897, P = 0.0000).

CONCLUSIONThe prognosis of thymoma depends mainly on the histologic subtype, clinical stage and multimodality treatment rather than paraneoplastic diseases.

Adolescent ; Adult ; Aged ; Child ; Child, Preschool ; Female ; Humans ; Male ; Middle Aged ; Myasthenia Gravis ; etiology ; Prognosis ; Radiography ; Retrospective Studies ; Thymoma ; complications ; diagnosis ; diagnostic imaging ; therapy