Search results for "Myasthenia Gravis"

Bilateral thoracoscopic thymectomy using a novel positioning system.

2013

Several techniques of bilateral video-assisted thoracoscopic extended thymectomy have been proposed, and each has its own proponents. We summarize our experience in 20 patients who underwent bilateral video-assisted thoracoscopic extended thymectomy, using a new patient positioning that amplifies the thoracoscopic view of the cardiophrenic regions which are often difficult to visualize with standard techniques. In all cases, en-bloc thymectomy with complete dissection of the mediastinal fatty tissue was achieved without sternal retractors or additional incisions.

The Autoimmune Regulator AIRE in Thymoma Biology: Autoimmunity and Beyond

2010

Thymomas are tumors of thymic epithelial cells. They associate more often than any other human tumors with various autoimmune diseases; myasthenia gravis is the commonest, occurring in 10-50% of thymoma patients, depending on the World Health Organization-defined histologic subtype. Most thymomas generate many polyclonal maturing T lymphocytes but in disorganized microenvironments Failure to induce self-tolerance may be a key factor leading to the export of potentially autoreactive CD4 progeny, thus predisposing to autoimmune diseases. Normally, the master Autoimmune Regulator promotes expression of peripheral tissue-restricted antigens such as insulin by medullary thymic epithelial cells a…

Bilateral single-port thoracoscopic extended thymectomy for management of thymoma and myasthenia gravis: Case report

2016

Background Video-assisted thoracoscopy is become a widely accepted approach for the resection of anterior mediastinal masses, including thymoma. The current trend is to reduce the number of ports and minimize the length of incisions to further decrease postoperative pain, chest wall paresthesia, and length of hospitalization. Herein, we reported an extended resection of thymoma in a patient with myasthenia gravis through an uniportal bilateral thoracoscopic approach. Case presentation A 74 years old woman with myasthenia gravis was referred to our attention for management of a 3.5 cm, well capsulate, thymoma. All laboratory and cardio-pulmonary tests were within normal; thus, she was schedu…

Hsp60 and AChR cross-reactivity in myasthenia gravis: An update.

2010

Role of heat shock proteins in the pathogenesis of myasthenia gravis.

2010

Thymoma and paraneoplastic myasthenia gravis

2010

Paraneoplastic autoimmune diseases associate occasionally with small cell lung cancers and gynecologic tumors. However, myasthenia gravis (MG) occurs in at least 30% of all patients with thymomas (usually present at MG diagnosis). These epithelial neoplasms almost always have numerous admixed maturing polyclonal T cells (thymocytes). This thymopoiesis-and export of mature CD4(+)T cells-particularly associates with MG, though there are rare/puzzling exceptions in apparently pure epithelial WHO type A thymomas. Other features potentially leading to inefficient self-tolerance induction include defective epithelial expression of the autoimmune regulator (AIRE) gene and/or of major histocompatib…

Other Types of Chaperonopathies

2013

A mechanism causing a chaperonopathy that is introduced in this chapter consists of the absence of a chaperone from the place where it is needed (i.e., chaperonopathies by misplacement). Also in this chapter are discussed the unfolded-protein response (UPR), chaperone-mediated autophagy (CMA), and illustrative examples of chaperonopathies by mistake, or collaborationism. In these conditions, one or more chaperones, apparently normal in structure, perform functions that favor disease rather than the contrary, hence the name of chaperonopathy by mistake or collaborationism (a molecule that ought to protect the cell and the organism promotes pathogenesis instead). Many examples of chaperonopat…

Clinical characteristics and outcomes of thymoma-associated myasthenia gravis

2021

[Background and purpose] Prognosis of myasthenia gravis (MG) in patients with thymoma is not well established. Moreover, it is not clear whether thymoma recurrence or unresectable lesions entail a worse prognosis of MG.

Dropped head as an unusual presenting sign of myasthenia gravis.

2007

Prominent or isolated weakness of cervical extensor muscles is a relatively rare clinical sign. Commonly, this is known as "dropped-head syndrome". This abnormal flexion of the head may occur in a variety of neuromuscular diseases and in a few non-neurological disorders as well. The case we describe concerns a 61-year-old woman with dropped-head syndrome as the unique complaint of myasthenia gravis.

Immune-mediated rippling muscle disease with myasthenia gravis: a report of seven patients with long-term follow-up in two.

2009

We report seven patients with immune-mediated rippling muscle disease (iRMD) and AChR-antibody positive myasthenia gravis (MG) without germline caveolin-3 gene mutations. We describe the follow-up of two patients and the clinical features of five new patients (1 female, 4 male, aged 32 to 69 years). These presented with significant generalized, exercise-induced and electrically-silent muscle rippling with myalgia, combined with generalized MG. In two of the seven patients, MG appeared before iRMD. Mediastinal imaging excluded thymic alterations in all, although two had other coincident tumours. Myalgia and rippling were aggravated by acetylcholinesterase-inhibitor treatment. Generalized MG …