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PubMed1975

Myasthenic decrement and myasthenic myopathy. A study on the effects of thymectomy.

Motor unit action potentials, M responses to repetitive nerve stimulation, and anticholinesterase tests were investigated in 12 myasthenic patients before and after thymectomy. In six of them the endarterial acetylcholine test was also carried out. Responsiveness to ACTH or to prednisone treatment was evaluated before and after thymectomy. The typical myasthenic presynaptic disorders were improved by thymectomy, while signs of myasthenic myopathy (according to Rowland's definition) were apparently increased. This process of 'functional myopathophanerosis' is discussed and explained in terms of a previous presynaptic disorder blocking the voluntary recruitment threshold of those motor units which are most affected at both presynaptic and postsynaptic level.

Acetylcholine↗
PubMed1996

Thymus in myasthenia gravis: comparison of CT and pathologic findings and clinical outcome after thymectomy.

PURPOSE: To correlate computed tomographic (CT) appearance of the thymus with results from histologic examination of thymic tissue and clinical outcome in patients with generalized myasthenia gravis who underwent thymectomy. MATERIALS AND METHODS: Forty-five patients with myasthenia gravis underwent CT of the thorax and thymectomy. Findings at clinical follow-up were available in all patients. RESULTS: Twenty-six patients had normal CT findings, seven had a diffusely enlarged thymus, and 12 had a focal mass. The results of histologic examination showed that 16 of 26 patients with normal CT findings had normal thymic tissue and 10 had lymphoid follicular hyperplasia; all seven patients with an enlarged thymus had lymphoid hyperplasia. Five of 12 patients with a focal mass at CT had lymphoid hyperplasia, and seven had thymoma. Clinical improvement following thymectomy was observed in 27 (93%) of 29 patients with lymphoid hyperplasia or thymoma and 11 (69%) of 16 patients with normal histologic examination (P < .03, chi(2) test). CONCLUSION: The presence of an enlarged thymus or a focal mass in patients with myasthenia gravis indicates lymphoid hyperplasia or thymoma. However, CT is of limited value in distinguishing lymphoid follicular hyperplasia from a normal thymus or thymoma and in predicting clinical outcome.

Adolescent↗
PubMed2001

Factors influencing improvement and remission rates after thymectomy for myasthenia gravis.

BACKGROUND: Thymectomy has become an accepted option in the treatment of myasthenia gravis (MG). However, the optimal selection of patients for surgery remains controversial. OBJECTIVE: The objective of the study is to define factors influencing improvement and remission rates after thymectomy for MG. METHODS: We retrospectively reviewed our experience in the surgical management of patients with MG over the last 20 years, and analyzed if patient's age, sex, preoperative Osserman Stage, thymic histology, duration of symptoms, or radiological findings influenced outcome. RESULTS: There were 25 women and 10 men. Most of them presented with Osserman stage IIA (n = 12) and stage IIB (n = 17). Hyperplasia of the thymus was observed in 15 patients (43%), thymoma in 12 (34%), and atrophic or normal thymus in 8 (23%). Computed tomography (CT) had a positive predictive value of 91% in detecting thymoma and of 80% in detecting thymic hyperplasia. Over a mean follow-up of 96 months, MG improved markedly in 26 patients. Postoperative improvement was significantly greater in patients with advanced MG (stage IIB, III and IV), whereas a higher rate of remission occurred in patients with mild MG (stage I and IIA), and in those with thymic hyperplasia. No relation was observed between outcome and age, sex, duration of symptoms and abnormal CT findings. CONCLUSIONS: The beneficial effects of thymectomy are characterized by postoperative clinical improvement in most patients with moderate to severe preoperative myasthenic involvement and by a higher remission rate in patients with thymic hyperplasia and/or mild myasthenic symptoms.

Adolescent↗
PubMed2003

Predictors of outcome for myasthenia gravis after thymectomy.

The aim of this study was to assess the clinical outcome of patients with myasthenia gravis treated with maximal thymectomy and to identify prognostic variables that predict the outcome. Over 15 years, from 1986 to 2001, we collected data on 100 patients with myasthenia gravis who underwent maximal thymectomy and retrospectively reviewed their outcome. Women comprised 63% and the median age was 25 years (range, 4 to 61). The median duration of the disease was 26 months (range, 1 to 240). According to the Osserman classification, there were seven patients in class I, 31 in class II, 47 in class III, and 15 in class IV. In non thymomatous patients (93 patients), complete remission rate progressively increased from 37.4% to 58.2% and 75% at 3, 10 and 15 years of follow-up respectively. These findings suggest that the complete remission rate is prone to increase with time after maximal thymectomy. The total benefit rate achieved was estimated to be 86% while 14% did not improve at a mean follow-up period of 7.6 years (range, 8 to 180 months). Univariate analysis (p < 0.05) showed that age, thymic histology and ectopic thymic tissue are significant prognostic factors for outcome.

Adolescent↗
PubMed2006

Thoracoscopic thymectomy in a previous sternotomy.

A video assisted thoracoscopic surgery (VATS) thymectomy is a viable alternative to conventional open thymectomy in myasthenia gravis (MG). A previous operation in the same anatomical region is generally considered a relative contraindication to any minimally invasive approach in the same area. Few cases of VATS on previously operated chests have been reported. We report a case of a VATS thymectomy in a patient who had undergone two previous sternotomies for cardiac disease.

Female↗
PubMed2000

Continuous neostigmine infusion in post-thymectomy juvenile myasthenic crisis.

A 10-year-old boy with myasthenia gravis had severe post-thymectomy myasthenic crisis necessitating reintroduction of mechanical ventilation on the 5th day after thymectomy. He did not respond to therapy with oral pyridostigmine, corticosteroids, or high-dose intravenous immunoglobulin. Finally, in addition to the usual supportive care, he was treated successfully with continuous intravenous infusion of neostigmine. Continuous infusion of neostigmine was used for the first time in post-thymectomy myasthenic crisis in a child to the best of our knowledge.

Child↗
PubMed1990

Thymectomy in juvenile myasthenia gravis.

The long-term results of thymectomy in 24 children with generalized myasthenia gravis are reviewed. Sixteen had complete remission and another seven were improved. This compares favorably with reported spontaneous remission rates of 30%. Because of the low morbidity in recent reports and the possibility that early thymectomy is more beneficial, we recommend thymectomy at the onset of juvenile generalized myasthenia gravis.

Adolescent↗
PubMed1995

Appearance of systemic lupus erythematosus after thymectomy: four case reports and review of the literature.

The appearance of systemic lupus erythematosus (SLE) after thymectomy (or thymomectomy) is presented in four patients together with a comparative review of additional reports found in a Medline search for the years 1966-94 in the English and French literature. Fourteen women and two men of average age of 39 years (range 11-66 years) at presentation, developed SLE after thymectomy (11 patients) or thymomectomy (five patients). Half developed SLE within 3 years after surgery (range 3 months to 18 years). The most common SLE manifestation was polyarthritis occurring in 15 of 16 patients either at presentation or during the first year. Other frequent manifestations included skin rashes, fever, cytopenias and pleuritis. Two rare manifestations of SLE, optic neuritis and transverse myelitis, were reported in two patients. Thymic hormone activity was measured in one patient and was undetectable compared with normal controls. HLA studies in eight patients showed the combination of A1, B8 in four. In conclusion, the appearance of SLE after thymectomy or thymomectomy appears to be more than a coincidence. It may provide insights into the pathogenesis of SLE.

Adolescent↗
PubMed1997

Primary antiphospholipid syndrome emerging following thymectomy for myasthenia gravis: additional evidence for the kaleidoscope of autoimmunity.

The etiology of autoimmune diseases is multifactorial. In many of them the stimulation by a specific autoantigen is claimed to be responsible for the initiation of the disease. Alternatively, an autoimmune state may be induced by a pure dysregulation of the immune system. Such is the case in which severe systemic lupus erythematosus (SLE) is induced in young patients with myasthenia gravis following thymectomy. We have referred to this set of events as the 'kaleidoscope of autoimmunity'. Herewith, we would like to present another example of the kaleidoscope phenomenon, namely: the emergence of a full blown clinical presentation of the primary antiphospholipid syndrome (APS-recurrent thromboembolic phenomena, repeated fetal loss with high titers of anti-cardiolipid antibodies) in a 32 y old female with myasthenia gravis, two years following thymectomy. Thymectomy in myasthenic patients may be associated with the emergence of new autoimmune conditions such as SLE and APS, pointing to the importance of immune dysregulation in the induction of these autoimmune diseases.

Abortion, Habitual↗
PubMed1975

Studies in myasthenia gravis. Pyridostigmine-C14 metabolism after thymectomy.

Pyridostigmine-carbon 14 (P-C14) excretion studies in myasthenia gravis patients who had had thymectomies failed to produce any significant difference from results observed in myasthenic patients who had not had thymectomies. Thus, change in P-C14 metabolism cannot help explain decreased anticholinesterase requirements and electromyographic changes observed in some patients following thymectomy.

Adult↗
PubMed1976

Electrophysiologic evaluations of thymectomy in myasthenia gravis. Preliminary findings.

Electric testing was performed in 106 myasthenia gravis patients before and after transcervical thymectomy. Twenty-nine were followed for 3 to 24 months. Results were correlated with thymic pathology, duration of disease, age at operation, and follow-up clinical status. Electric improvement was significantly greater in patients without thymic germinal centers or with only rare to occasional germinal centers, in patients operated on within 1 year after onset of symptoms, and in patients under age 30. Electric improvement immediately after thymectomy heralded later clinical improvement in those patients without germinal centers or with rare to occasional germinal centers. Electric-clinical correlations were excellent in patients with longer follow-up. Serial electric testing provides an objective evaluation of the patients' clinical status post-thymectomy.

Adult↗
PubMed1977

Effective treatment of infantile myasthenia gravis by combined prednisone and thymectomy.

Myasthenia gravis developed in two children at 2 and 3 years of age respectively. Minimal improvement followed chronic oral administration of cholinesterase inhibitors. Patient 1 had ptosis and ophthalmoplegia but no clinical or electromyographic involvement of muscles of the extremities, although a quadriceps muscle biopsy revealed lymphorrhages. Patient 2 had progressive generalized myasthenia for 3 1/2 years. Both children were given a 3-month course of prednisone followed by thymectomy. They both are in remission, 12 and 8 months after thymectomy, with only minimal residual ocular weakness, but this weakness is much more responsive to anticholinesterase drugs than before thymectomy. Long-term administration of steroids, with the attendant complication of growth retardation, is avoided.

Age Factors↗
PubMed1979

Cellular immune response to acetylcholine receptor in myasthenia gravis: II. Thymectomy and corticosteroids.

We studied 11 patients with myasthenia gravis who demonstrated a cellular immune response to acetylcholine receptor (AChR) of the electric organ of Torpedo marmorata. After thymectomy, there was a marked decrease in the patients' lymphocyte reactivity to AChR. The mean reduction of the stimulation index (SI) was 50%, but the response to the nonspecific mitogen phytohemagglutinin (PHA) was not affected. In six cases, the lymphocyte response was measured at intervals up to 22 months after thymectomy; in all six, the immune response to AChR remained decreased. In some cases, the response continued to decrease, even to normal values. The effect of corticosteroid treatment was tested in other patients. The cellular immune response to AChR was significantly lower in treated patients (mean SI, 1.64 +/- 0.25) than in untreated controls (mean SI, 2.41 +/- 0.38), with no significant difference in the response to PHA. These data suggest that a decrease in the cellular immune response to AChR may be one mechanism by which thymectomy and corticosteroids are therapeutic in myasthenia.

Acetylcholine↗
PubMed1982

Reoperation after transcervical thymectomy for myasthenia gravis.

Among 137 thymectomized patients with nonthymomatous myasthenia gravis (MG), six were reoperations. Thymectomy had initially been performed by the transcervical approach, but was ineffective. Extended thymectomy was then performed by median sternotomy. The reoperations revealed residual thymus (average weight, 19 gm) in all cases. Postoperative courses were favorable in three patients and unfavorable in three others. The latter had received high-dose steroid therapy before the second operation. We conclude that total thymectomy by the transcervical approach is almost impossible, and that reoperation may accelerate improvement in some cases.

Adult↗
PubMed1983

Increased circulation of T lymphocytes bearing surface thymosin alpha 1 in patients with myasthenia gravis: effect of thymectomy.

We studied the interaction of the thymic hormone thymosin alpha 1 with peripheral blood B and T lymphocytes in patients with myasthenia gravis (MG), using antibodies against thymosin alpha 1 in an immunofluorescence technique. Eleven of 16 patients with symptomatic MG had an increased number of T lymphocytes bearing surface thymosin alpha 1 (T alpha 1); 5 patients with asymptomatic disease had normal levels of T alpha 1. In six young adults with symptomatic MG who subsequently responded to thymectomy, the number of T alpha 1 cells returned to normal 1 month after thymectomy. Because levels of T alpha 1 correlated with symptoms and thymosin alpha 1 specifically recruits helper T cells, our findings suggest that T alpha 1 may play an immunoregulatory role in the pathogenesis of MG. Determination of T alpha 1 levels may prove to be helpful in assessing residual thymic activity after thymectomy.

Fluorescent Antibody Technique↗
PubMed1983

Postoperative pericarditis following thymectomy for myasthenia gravis. A prospective study.

Eight consecutive patients who underwent thymectomy for the treatment of myasthenia gravis were evaluated prospectively for the development of postoperative pericarditis. In four of the eight patients (50 percent) pericarditis developed within 48 hours after thymectomy. All four had a three-component pericardial friction rub, two of the four patients had a new postoperative pericardial effusion by echocardiography, and in two typical ECG diffuse concave ST segment elevation and evolutionary ST and T wave changes developed. In the four patients with postoperative pericarditis, a malignant thymoma adherent to the pericardium was found in two, while two patients had a normal thymus histology with no adherence to the pericardium. Thus, postoperative pericarditis was found in both of the patients with a thymoma and in two of six patients without a thymoma. This study demonstrates that a high incidence of pericarditis occurs following thymectomy for the treatment of myasthenia gravis.

Adolescent↗
PubMed1990

[Thymectomy and immunosuppression in myasthenia gravis: a prospective study].

Between 1982 and 1988 24 women and 6 men with myasthenia gravis were included in a prospective and standardized diagnostic and therapeutic protocol. Age varied between 10 and 74 years (34 +/- 16, mean +/- standard deviation). Three patients with the ocular form were treated with pyridostigmine. Four patients with the generalized form and advanced age received steroids and/or azathioprine. Twenty-three patients with the generalized form underwent thymectomy followed by prednisone and/or azathioprine. One patient died after complex thymectomy for invasive thymoma. Another died soon after admission in myasthenic/cholinergic crisis. Two other patients had minor complications of thymectomy. Of 19 patients followed for 1-60 (mean 24) months, 11 (58%) are in remission, one of them without any medication.

Adolescent↗
PubMed1995

Plasmapheresis as preparatory method for thymectomy in myasthenia gravis.

To study the effects of plasmapheresis in preparation for thymectomy, two groups of 40 patients were selected from a sample of 286 patients with myasthenia gravis examined by the first author Group 1 included patients (15 male and 25 female; age range 8-64 yrs) who underwent thymectomy without previous plasmapheresis, whereas patients in group 2 (17 male and 23 female; age range 11-61 yrs) were thymectomized after plasmapheresis. We required patients to have a minimum follow-up period of 12 months to be included in the study. A clinical evaluation protocol composed of 76 items was developed for the study. We found significant improvement in respiratory function and muscular strength in patients thymetomized after plasmapheresis. Furthermore, the combined treatment reduced cost and length of hospital stay. Therefore, we conclude that plasmapheresis should be considered as a coadjuvant to thymectomy in the treatment of myasthenia gravis.

Adolescent↗