Cases reported "Myasthenia Gravis"

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1/15. Sevoflurane anesthesia in a myasthenic patient undergoing transsternal thymectomy.

    myasthenia gravis (MG) is an autoimmune disease resulting from the production of antibodies against the acetylcholine receptors of the neuromuscular synapse. The thymus gland is involved in the autosensitization process, and there is a consensus that all adults with generalized MG should have a thymectomy. Removal of a much thymic tissue as possible via the transsternal approach in the logic goal of thymectomy in the treatment of MG. Because of the unpredictable response to succinylcholine and the marked sensitivity to nondepolarizing muscle relaxants in the MG patients, some anesthesiologists avoid the use of muscle relaxants in the myasthenic patients, and depend on deep inhalation anesthesia, such as halothane, isoflurane or sevoflurane. In the present report, we used sevoflurane 4%, without supplementation by muscle relaxants, for the induction and maintenance of anesthesia in a MG patient undergoing transsternal thymectomy. The report reviews the anesthetic technique, and describes the electromyographic (EMG) changes following sevoflurane.
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2/15. thyroid gland tumour, pemphigus foliaceus and myasthenia gravis in the daughter of a woman with myasthenia gravis.

    We describe a rare case of pemphigus foliaceus associated with familial myasthenia gravis (MG). A 35-year-old woman developed MG during oral corticosteroid treatment for pemphigus foliaceus. She had been operated on for a thyroid gland tumour that was confirmed histopathologically to be papillary carcinoma without metastasis. At the time of treatment, her mother had had MG for 30 years and undergone thymectomy 22 years ago. A specific ELISA technique showed that antidesmoglein 1 antibody was present in the daughter. There are many reports of multiple diseases such as pemphigus, thymoma, malignancy, and other autoimmune diseases associated with MG. However, familial MG following pemphigus foliaceus has not been reported previously.
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3/15. Thymolipoma in association with myasthenia gravis.

    A 52-year-old male presented with an anterior mediastinal tumor associated with a 2-year history of myasthenia gravis. The patient underwent thymectomy and a 185-g, 10 X 8 X 3.5 cm, well-delineated tumor was resected. On histologic examination the tumor proved to be a thymolipoma composed of mature adipose elements containing cords and nests of thymic tissue. The latter consisted mainly of cortical areas, the thymocytes of which displayed an immunohistochemical profile of cortical cells, i.e., CD 1 , CD 4 , CD 8 , and frequently Ki 67 . Ultrastructural study confirmed the predominant cortical differentiation of the thymic component. No germinal centers, dendritic reticulum cells, or myoid cells were detected by histologic, immunohistochemical, and ultrastructural studies. The association of thymolipoma with myasthenia gravis is rare; this case is the 10th reported. Our findings lead us to believe that (a) the cortical differentiation of the thymic component and the active thymocyte proliferation could represent a factor leading to myasthenia gravis; and (b) thymolipoma could be a peculiar form of thymoma rather than a mixed tumor of mesenchymal and entodermal origin, a lipoma, or a hamartoma of the thymic gland. The reported association of thymolipomas with other immune disturbances or with neoplastic conditions usually associated with true thymomas support these findings.
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4/15. Signs of sjogren's syndrome in a patient with myasthenia gravis.

    A case of myasthenia gravis (MG) with recurrent submandibular salivary gland enlargement and focal lymphocytic infiltrates in lip salivary glands is described. Though the clinical and close histopathological resemblance with sjogren's syndrome (SS), a condition characterized by impaired exocrine function of affected glands, no exocrine disturbance evolved during 3-years follow up. The clinical and histopathological overlap between MG and SS, sharing several immunological characteristics, might be of relevance in further studies on the pathogenesis of the two conditions.
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5/15. Peripheral nerve involvement in myasthenia gravis.

    The dermal and muscular nerves in a 13-year-old female patient affected by myasthenia gravis (MG) were subjected to ultrastructural observation. The dermal nerves mainly examined were located around the sweat glands, which were thought to be autonomic cholinergic nerves. Axonal and Schwann cell pathologies, of various degrees, such as proliferation and/or disorganized axonal microorganelles, accumulation of Reich granules and lipopigments in the Schwann cell cytoplasm, and expansion of the intraperiod line of the outer myelin loop, were observed in both types of nerves. Although this patient showed no clinical signs of peripheral neuropathy, including dysautonomia, this study indicates that there is generalized cholinergic nerve involvement in MG.
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6/15. Massive benign thymoma presenting as ocular myasthenia.

    A case of ocular myasthenia was found to be associated with a massive benign thymoma and responded to its removal. This is an exception to the generalisation that larger tumours of the thymus gland are more likely to be malignant and that thymectomy is not the treatment of choice for ocular myasthenia.
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7/15. Thyroid uptake of gallium in Graves' disease.

    A patient with hyperthyroid Graves' disease presented with ptosis, leading to a workup for myasthenia gravis. An enlarged thymus gland was noted on computed tomography. A scan with gallium-67 citrate showed prominent and diffuse thyroid gland activity as well as prominent lacrimal activity. This finding of thyroid uptake of gallium led to the correct diagnosis of Graves' disease. Such a finding has not been reported previously. The associated thymic, thyroid, and orbital findings in Graves' disease are discussed.
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8/15. recurrence of thymic hyperplasia after trans-sternal thymectomy in myasthenia gravis.

    A 23-year-old woman with myasthenia gravis underwent thymectomy in September, 1975 by a trans-sternal approach for the removal of an hyperplastic gland. Surgical intervention was followed by marked improvement of her symptoms. Five years later, symptoms recurred. In March, 1982 diagnostic pneumomediastinum disclosed an anterosuperior mediastinal mass which, on excision, proved to be an hyperplastic thymus. This report confirms previous findings of thymus regrowth after surgery. It also demonstrates that surgical techniques using the trans-sternal approach do not guarantee complete removal of the thymus.
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9/15. Ventilatory failure in myasthenia gravis.

    This retrospective study over the decade 1969-1978 examines the precipitating factors and outcome in thirty-one patients with myasthenia gravis who developed ventilatory failure. An unusual example of chronic alveolar hypoventilation is discussed in detail. The most favourable outcome occurred in younger patients with a hyperplastic thymus, in contrast to a poorer outlook for older patients with an atrophic gland. Eleven patients died during the period of follow up: three deaths were unrelated to myasthenia but the remaining eight were attributed directly or indirectly to it. The mortality of 36% represents a marked improvement on a 70% mortality in a similar group of patients, reported from this hospital for the years 1960-1968.
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10/15. Age-related changes in the thymus gland: CT-pathologic correlation.

    Recent reports suggest that computed tomography (CT) is useful for thymoma detection in patients with myasthenia gravis. However, that usefulness may be conditioned by the state of the normal thymus. To examine this concept, the CT findings in 64 consecutive patients with histologic confirmation of thymic status after thymectomy or thymic biopsy during mediastinal exploration were reviewed. The normal thymus has a bilobed, arrowhead-shaped cross section at all ages, with gradual focal or diffuse fatty infiltration of the parenchyma usually occurring between 20 and 40 years of age. A thymoma is usually a spherical or oval mass, often producing a focal, distinct bulge in the adjacent pleural reflection. The differentiation of thymoma from normal thymus should be possible in most patients if age-related changes in the normal gland are appreciated.
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