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PubMedDate not supplied

Coexistence of primary biliary cirrhosis and myasthenia gravis: a case study.

We present a case that suggests a relationship between primary biliary cirrhosis and myasthenia gravis. A 43-year-old Japanese woman was admitted to the Nagoya City University Medical School, First Department of Internal Medicine with abnormal liver function in August 1991. She had had ptosis of the right eye since 1990. She had not been treated for liver disease. Ptosis of the right eye and hepatomegaly were present. Serum laboratory examinations revealed elevated biliary enzymes and IgM levels; tests were positive for antimitochondrial antibody and antiacetylcholine antibody. Liver histology revealed chronic non-suppurative destructive cholangitis and led to a diagnosis of primary biliary cirrhosis. The tensilon test was positive. Electromyography with repetitive motor nerve stimulation revealed a neuromuscular junction defect; i.e., the primary characteristic of myasthenia gravis. The patient was diagnosed with myasthenia gravis. Although the development of myasthenia gravis has previously been reported in patients with primary biliary cirrhosis during D-penicillamine administration, this is a very rare case of the coexistence of both diseases before such treatment.

Adult↗

PubMed1999

Extended thymectomy for myasthenia gravis in an octogenarian. A case report.

An 86-year-old woman with myasthenia gravis successfully underwent an extended thymectomy. The patient had a 2-year history of generalized myasthenia and had limited response to anticholinesterases and steroids. An extended thymectomy was successfully performed in the usual fashion. The postoperative course was uneventful except for an episode of psychosis probably due to postoperative steroid therapy. The steroid dosage was gradually reduced to 5 mg/day over seven months after the operation, during which she became fully asymptomatic. No symptoms due to myasthenia have surfaced for over 2 years after surgery. Although elderly patients are usually considered to be less responsive to an operation, thymectomy may sometimes be the treatment of choice for myasthenia gravis even in octogenarians.

Aged↗

PubMed1999

[Treatment of myasthenia gravis].

The strategy of myasthenia gravis has been progressed during recent 30 years. Extended thymectomy and alternate-day prednisolone administration remarkably improved therapeutic effect in myasthenia gravis. Besides of these therapies, plasmapheresis and immunoadsorption plasmapheresis extremely improved mortality in myasthenia gravis. 1. Corticosteroid therapy Usually initial doses of PSL is 20-30 mg every other day in the morning after thymectomy, and increased 5 mg every on-day. Maximum PSL doses is 80-100 mg alternate-day, then this doses will be maintained over 4 weeks. After these procedures, PSL is gyradually decreased and discontinued within 2-3 years. 2. Thymectomy Extended transsternal thymectomy is the most useful method, because many authors have reported that re-thymectomy reveals re-appearance of thymus. 3. Invasive thymoma Although this type of thymoma has been called malignant thymoma, it should be called as invasive thymoma since any thymoma can invade surrounding tissues for a long subclinical period. Strategy of treating invasive thymoma is PSL administration, radiation and chemotherapy. Chemotherapy should be performed depend on histopathological findings of thymoma. CHOP therapy is useful for the lymphocytic cell predominant type of thymoma, however CHOP + cis-platin therapy is useful for the epithelial cell predominant type. Treatment of myasthenia gravis has been improved, however it is still unclear that how to treat invasive thymoma, and how to prevent re-appearance of symptoms by decrease or discontinuity of PSL administration.

Adrenal Cortex Hormones↗

PubMed1998

[Thymectomy in the treatment of myasthenia gravis].

OBJECTIVES: To review the clinical course of patients with myasthenia gravis following thymectomy and to identify prognostic factors that may influence outcome. METHODS: We follow-up 91 patients with myasthenia gravis surgically treated with thymectomy through a median stereotomy from May, 1983 to October, 1993. The overall 5-year remission and improvement probabilities were obtained by Kaplan-Meier Method. RESULTS: 36 (39.6%) patients showed remission, 27 (29.7%) improvement, 14 (15.4%) no change and 14 (15.4%) worsening. The overall 5-year remission and improvement probabilities of patients were 64% and 88% respectively. CONCLUSIONS: Most patient's symptoms after thymectomy were progressively improved with time, which is probably attributable to thymectomy, and the natural history of myasthenia gravis. Age at surgery, sex, duration of clinical course and pathological findings may be important prognostic factors for patients with myasthenia gravis after thymectomy.

Adult↗

PubMed2000

[A case of myasthenia gravis, who developed minimal change nephrotic syndrome during immunosuppressive therapy after thymectomy].

Myasthenia gravis is caused by antibodies against acetylcholine receptors and is treated with inhibition or elimination of antibody production. We report a 58-year-old woman who had been suffering from myasthenia gravis and underwent thymectomy in July 1995. Her myasthenic symptoms improved with immunosuppressive treatment using corticosteroid(100 mg/day) and azathioprine(100 mg/day). However she presented edema with massive proteinuria(7.54 g/day) and was admitted to our hospital on July 1997. She was diagnosed as having nephrotic syndrome and a renal biopsy was performed. The histological findings showed minimal change nephrotic syndrome. After pulse therapy with methylprednisolone(1 g/day x 3 days) following oral administration of prednisolone(60 mg/day), proteinuria disappeared after one month. Nephrotic syndrome is a rare complication in patients with myasthenia gravis. The increase in lymphokine production caused by thymectomy may be closely associated with the occurrence of nephrotic syndrome in spite of intensive immunosuppressive treatment in the present case. In this report, we also summarized reported cases of minimal change nephrotic syndrome with thymoma and myasthenia gravis.

Azathioprine↗

PubMed2000

Normalization of elevated CD4-/CD8- (double-negative) T cells after thymectomy parallels clinical remission in myasthenia gravis associated with thymic hyperplasia but not thymoma.

T-cell-dependent B-cell help is likely to be of major importance in the pathogenesis of myasthenia gravis, but mechanisms provoking a pathological anti-acetylcholine receptor (AChR) response are poorly understood. We report on the dysregulation of recently identified CD4-/CD8- (double-negative) T cells (DN T cells), which have been shown to participate in immunoregulation and antibody augmentation. Compared with healthy controls, significantly increased frequencies of DN T cells were found in the blood of myasthenia gravis patients with lymphofollicular hyperplasia. After thymectomy, however, normalization in the number of these cells was seen in parallel with clinical improvement and reduction in anti-AChR antibody titers. The effect of thymectomy was observed irrespective of adjuvant treatment and held true for up to 4 years of follow-up. In marked contrast, frequencies similar to control values were found in myasthenia gravis patients with thymoma, with thymectomy having no further reducing effect. These data indicate that CD4-/CD8- T cells not only participate in the pathogenesis of myasthenia gravis but also correlate with disease activity and histological findings.

Adult↗

PubMedDate not supplied

[ORL manifestations of myasthenia gravis].

Myasthenia gravis is an illness of insidious onset whose first manifestation often is dysphagia. We report the case of a 25 year-old woman who consulted for nosebleed. On her first visit she referred progressive dysphagia, but the results of endoscopic digestive examination and indirect laryngoscopy were normal. The condition evolved until the appearance of weakness of the extremities suggested myasthenia. Myasthenia gravis was diagnosed by means of electromyography and determinations of antibodies against acetylcholine receptors. We reviewed the literature on the clinical manifestations, diagnostic procedures, and treatment of this condition. This case highlights the importance of suspecting myasthenia gravis in young women with dysphagia and a family or personal history of autoimmune disease in order to reach an early diagnosis and treatment.

Adult↗

PubMed2000

A study of myasthenia gravis in patients with and without thymoma.

This study was undertaken to compare the clinical, neurophysiological, radiological and prognostic features of myasthenia gravis with and without thymoma. 37 patients with myasthenia gravis (27 males, 10 females), with age range of 4.5 to 72 (mean 39) years, were managed at a tertiary care centre in India. Four patients were below 15 years of age and 6 above 55 years. Most of the patients were in stage II (34). There were 2 patients in stage III and 1 in stage I. 27 patients underwent thymectomy. Thymoma was detected in 10 cases. The decrement in patients with thymoma ranged between 11 and 62% (mean 27.9%) and nonthymoma group 10-75% (mean 28%). CT scan of thorax revealed mediastinal mass in 5 out of 10 cases of thymoma and 2 out of 27 patients without thymoma. Outcome of myasthenia gravis with thymoma was worse than without thymoma at 1 year followup. Severity of illness, extent of decrement, lack of facilitation, duration of illness and age of the patients were not related to the outcome. It is concluded the clinical and neurophysiological changes in myasthenia gravis with and without thymoma do not differ. However, patients with thymoma have a worse outcome.

Adult↗

PubMed2000

[Pulmonary adenocarcinoma in myasthenia gravis--autoimmunity and late development of malignancy].

We report a 75-year-old man with myasthenia gravis for many years, who was hospitalized because of cough, fever, and dyspnea. Chest x-ray revealed a bilateral pleural effusion. Adenocarcinomatous cells were found in the pleural fluid. Computerized tomography of the chest showed widespread pulmonary dissemination of the tumor. The relationship between myasthenia gravis, an autoimmune disease involving the motor end-plate, and malignancy (thymoma) has been widely recognized. Current literature documents few reports of lung malignancies with concurrent development of myasthenia gravis. A tentative explanation, based on current research, is provided for the possible role of myasthenia gravis and the late development of lung cancer. Moreover, a model for the autoimmune phenomenon and the development of late malignancies is provided with explicit explanations. It is important to search for occult, developing malignancies in newly diagnosed autoimmune diseases.

Adenocarcinoma↗

PubMed2001

[Therapy of myasthenia gravis].

The treatment of myasthenia gravis is a difficult entity. We especially discuss the immunosuppressive treatment with regard to the literature. In patients with thymoma all authors agree in thymectomy. In generalized myasthenia, even without thymoma, thymectomy should be performed in "younger" patients. In "elderly" patients thymectomy as treatment of choice is controversial. The basis of immunsuppressive therapy are corticosteroids, additionally azathioprine can be given considering the delay in response and the major side effects. As today, cyclophosphamide and cyclosporine A are too toxic for routine use, except third choice in therapy resistant patients as ultima ratio. The procedure of choice in myasthenic crisis is plasma exchange if the patient tolerates it. Alternatively intravenous immunoglobulin should be given. In pure ocular myasthenia treatment is controversial in the literature. In the last few years there is a tendency to treat this form early like generalized myasthenia, especially in young patients, to prevent generalization.

Humans↗

PubMedDate not supplied

[Myasthenia gravis associated with carcinoma of the thymus. A report of three cases].

INTRODUCTION: Although the association between benign neoplasias of the thymus and myasthenia gravis is well known, with greater predominance of squamous thymomas over other histological types, carcinoma of the thymus is only exceptionally associated with auto immune disorders. CASE REPORTS: We present three patients with myasthenia gravis associated with carcinoma of the thymus. They were three men aged between 38 and 73 years. Clinical features of myasthenia were generalized in two cases and ocular only in the third. Antireceptor antibodies to acetylcholine and anti striped muscle antibodies were present in all three patients. Repetitive stimulation was abnormal in two of the three cases. On CAT of the thorax there was a tumour of the thymus with the radiological characteristics of a benign thymoma in two of the patients and data suggesting an invasive thymoma in the third. In all three cases the diagnosis of carcinoma of the thymus was made on the post operative histological findings. CONCLUSIONS: The association of carcinoma of the thymus with myasthenia gravis in our series of cases was probably due to chance. We consider that this association is exceptional since the histological characteristics of carcinoma of the thymus do not favour the development of auto immunity.

Adult↗

PubMedDate not supplied

[Childhood myasthenia. Clinical picture and therapeutic results].

The paper contains an analysis of 37 cases of myasthenia beginning in childhood, including 3 cases of congenital myasthenia and 34 cases of infantile myasthenia. The cases of transient myasthenia of newborns, were excluded. The age of patients at the onset of the disease, diagnostic difficulties, clinical course and therapeutic results are discussed. Surgical treatment (thymectomy) was performed in 24 cases with severe clinical course and improvement was achieved in 17 cases (72%). In 13 cases conservative treatment was applied. The weights and the histological findings in exised thymus are presented.

Adolescent↗

PubMed2001

[Comparative characteristics of surgical treatment of thymoma with the clinical signs of myasthenia and without them].

Analysis of results of surgical treatment of 72 patients with thymoma, including 49 in combination with clinical signs of myasthenia and 23 without them, is presented. In group of patients with symptoms of myasthenia the women aged from 30 to 39 yrs prevailed and among those who had not--the men, aged from 40 to 49 yrs. Histologic investigation in 29 (59.1%) of patients with symptoms of myasthenia showed lymphocytic thymoma, in 10 (43.4%) of patients without signs of myasthenia--benign thymoma was revealed.

Adolescent↗

PubMed1999

[Long term effect of thymectomy for patients with myasthenia gravis].

OBJECTIVE: To evaluate the long term effect of thymectomy for patients with myasthenia gravis. METHODS: Patients with myasthenia gravis 4 - 54 months after thymectomy were followed up. RESULTS: 124 patients with myasthenia gravis were treated by thymectomy from April 1990 to August 1994. The mean age at the time of surgery was 29.2. According to the modified Osserman classification. 29 patients were type I, 23 type II a, 41 type II b and 11 type III. After thymectomy 82.7% patients had good response to the operation. 13.5% had no response and 1.9% were even worse. Of 104 patients followed up, no early death was found and late death occurred in 2. Type II patients and those with shorter preoperative course responded better. CONCLUSIONS: The surgical outcome is related to clinical classification of myasthenia gravis and the duration of preoperative period. Sex and pathologic type of thymus lesion seem to be irrelevant factors to the surgical outcome. To perform thymectomy or not should be very carefully weighed for patients younger than 10 years of age.

Adolescent↗

PubMed2002

Outcome of thymectomy in patients with Myasthenia Gravis.

OBJECTIVE: Myasthenia Gravis is a neuromuscular disorder characterized by weakness and fatigability of skeletal muscles. Thymectomy is now beneficial even for non-thymomatous patients. The aim of this paper is to evaluate the role of thymectomy as on the treatment options for myasthenia gravis. METHODS: Thirty patients underwent thymectomy in the period from April 1999 through to July 2000. They were collected from different teaching hospitals in Baghdad, Iraq. All these cases were studied conventionally including history, physical and neurological examination in addition to investigations with particular emphasis on connective tissue screen and thyroid function test. RESULTS: The age in this study ranged between 17-55 years. The preoperative duration ranged between 2-20 months and the postoperative follow up period ranged between 6 months-14 years. The most favorable results were obtained in patients without thymoma with short duration. CONCLUSION: Early thymectomy carries the best results in Myasthenia Gravis and every effort should be made to shorten the preoperative duration for both thymomatous and nonthymomatous patients. Patients with symptoms can be better controlled with anticholinesterase drugs than patients using steroid and cytotoxic drugs. Thymectomy is beneficial for most patients with Myasthenia Gravis and especially in those with benign folicular hyperplasia.

Adolescent↗

PubMedDate not supplied

[Myasthenia and thymoma in a seven year old girl].

INTRODUCTION: Myasthenia is an autoimmune disease, being generalized muscular weakness, with important participation of facial muscles, a prominent feature. Signs of muscular fatigue arise, worsened by exercise and alleviated by rest. Clinical symptoms are less marked before noon, and get worse as the day advances, through the afternoon and evening. A clear relationship between myasthenia and thymic abnormalities does exist, being glandular hyperplasia and tumours the commonest underlying pathologic findings. Initial treatment is based on anticholinesterase drugs and steroids. Non respondents should be treated with immunoglobulins, immunosuppresses, plasmapheresis and surgical removal of the thymus, according to the symptoms control. CASE REPORT: We present the case of a seven years old girl with generalized muscular weakness, worsening through the day, being the diagnosis of myasthenia confirmed by the high level of acetylcholine antireceptors antibodies and the neurophysiologic study. Imaging study of the mediastinum showed a thymic mass located in the right lobe. CONCLUSION: It is therefore most important to rule out these conditions when myasthenia is suspected.

Child↗

PubMed1975

[Diagnosis and differential diagnosis of myasthenia gravis].

The author describes the possibilities of diagnosis and differential diagnosis of myasthenia gravis. The data is based on the literature and on case reports of personal observations of more than 270 patients with myasthenia. He inaugurates his own classification of myasthenia comprising of 8 types, among which there is one particular clinical type, described first by the author. The different problematic types of myasthenia are discussed together with the most important diagnostic tests and the principles of differential diagnosis. The clinical and pathological study is completed by illustrations.

Adolescent↗

PubMedDate not supplied

[Ecological aspects of myasthenia gravis in the Orenburg region].

The paper reflects the problem of adverse environmental factors on human health, including those on the prevalence of myasthenia and on the blood levels of trace elements in the patient. It outlines the environmental status in the Orenburg Region, which is most unfavorable in the Eastern and Central areas. Myasthenia morbidity trends for its increase are covered in 1967 to 2006. The paper also shows the prevalence of myasthenia, with predominance per 100,000 individuals in the Eastern and Central areas, which coincides with the highest anthropogenic load in these areas. The data of a preliminary analysis of trace elements, such as copper, zinc, manganese, nickel, lead, strontium, chromium, cadmium, cobalt, iron, in the blood of patients with myasthenia as compared with those in controls and by regions reflect a general trend for the blood levels of these trace elements to be decreased, as well as their imbalance by the areas of the Orenburg Region.

Catchment Area, Health↗