Therapeutic plasmapheresis is nowadays the method of choice in severe cases of myasthenia gravis. The authors confirm the therapeutic effect of this method and emphasize its importance in the differential diagnosis of severe cases where myasthenia gravis is suspected only from clinical symptoms, and electromyographic, roentgenological and laboratory evidence (antibodies against acetylcholine receptors) is lacking. The mentioned procedure led to thymectomy with the finding of a tumour of the thymus within three and a half months after the development of the first symptoms of the disease.

• MeSH
• dospělí MeSH
• lidé MeSH
• myasthenia gravis terapie   MeSH
• plazmaferéza * MeSH
• Check Tag
• dospělí MeSH
• lidé MeSH
• ženské pohlaví MeSH
• Publikační typ
• anglický abstrakt MeSH
• časopisecké články MeSH
• kazuistiky MeSH

Surgical treatment of myasthenia gravis at the 3rd Department of Surgery, 1st Faculty of Medicine, Charles University in Prague, has long tradition. It originated in sixties of the 20th century. Over 1000 operations of a thymus has been carried out since that time, especially thymectomy for myasthenia gravis (MG) and for less frequent thymomas. The complete removal of thymus--thymectomy accompanied with exenteration of the fat in frontal mediastinum belongs to the principal MG surgery. According to our experience, the optimal approach to the thymus is a partial sternotomy. The jugular approach is not regarded as sufficiently radical. Videothoracoscopic approach and operation are possible but they are longer. They represent a method of choice at some workplaces. The combined MG treatment (conservative medicamentous and surgery treatment) brings in 80% a visible improvement and a deletion of symptoms of the disease.

• MeSH
• lidé MeSH
• myasthenia gravis chirurgie   MeSH
• nádory brzlíku chirurgie   MeSH
• pooperační komplikace MeSH
• thymektomie * škodlivé účinky   metody   MeSH
• thymom chirurgie   MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• anglický abstrakt MeSH
• časopisecké články MeSH
• přehledy MeSH

The authors describe a case of systemic lupus erythematosus in a 33-year-old female patient who developed the disease 12 years after thymectomy on account of myasthenia gravis. The activity of systemic lupus erythematosus was controlled by pulsed treatment with methyl prednisolone followed by long-term oral glucocorticoid and immunosuppressive therapy. The authors discuss the risk of systemic lupus erythematosus after thymectomy on account of myasthenia gravis with regard to the HLA haplotype.

• MeSH
• dospělí MeSH
• lidé MeSH
• mladiství MeSH
• myasthenia gravis chirurgie   MeSH
• systémový lupus erythematodes etiologie   MeSH
• thymektomie škodlivé účinky   MeSH
• Check Tag
• dospělí MeSH
• lidé MeSH
• mladiství MeSH
• mužské pohlaví MeSH
• ženské pohlaví MeSH
• Publikační typ
• anglický abstrakt MeSH
• časopisecké články MeSH
• kazuistiky MeSH

• MeSH
• lidé MeSH
• myasthenia gravis diagnóza   MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• anglický abstrakt MeSH
• časopisecké články MeSH

Myasthenia gravis is very rare autoimmune disease of neuromuscular junction, which presents as a weakness and increased fatiquability of striated muscles. Formerly, myasthenia was largely constraining disease and often ended by death. Recently, advanced diagnostic methods and variety of therapeutic options allow the full compensation of the disease and the quality of patient life is restored. In this review, the methods used during the myasthenia treatment along with the description of the disease itself were detailed.

• MeSH
• acetylcholinesterasa terapeutické užití   MeSH
• imunosupresiva terapeutické užití   MeSH
• lidé MeSH
• monoklonální protilátky terapeutické užití   MeSH
• myasthenia gravis diagnóza   farmakoterapie   MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• anglický abstrakt MeSH
• časopisecké články MeSH
• přehledy MeSH
• Názvy látek
• acetylcholinesterasa MeSH
• imunosupresiva MeSH
• monoklonální protilátky MeSH

• MeSH
• dospělí MeSH
• komplikace těhotenství * patofyziologie   MeSH
• lidé MeSH
• myasthenia gravis * patofyziologie   MeSH
• novorozenec MeSH
• porodní děj MeSH
• těhotenství MeSH
• vedení porodu MeSH
• Check Tag
• dospělí MeSH
• lidé MeSH
• novorozenec MeSH
• těhotenství MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH

Granulomatous myopathies are extremely rare. The finding of epithelioid granulomas in muscle biopsy indicates mostly an involvement of the skeletal muscle in systemic sarcoidosis. In this report we provide description of five patients with previously diagnosed sarcoidosis (pulmonary or cutaneous), in which a clinically significant muscle weakness developed. We aim at demonstrating the value of muscle biopsy for further treatment of the patients, since clinically indistinguishable myopathies can arise not due to the involvement of muscles in the granulomatous process, but due to the corticosteroid-induced changes (chronic steroid myopathy). The demonstration of a selective atrophy of type II muscle fibers can provide the clue for distinguishing the patients, in which the corticosteroid treatment should continue, from those, in which the treatment should be modified. Further, we discuss a rare finding of granulomas in muscle biopsies of two patients with myasthenia gravis (MG) associated with thymoma. Although it is difficult to explain the pathogenesis of this event, MG should be considered in the differential diagnosis of granulomatous myopathies. Moreover, the finding of granulomas along with lymphocytic infiltration in MG muscle should lead to a search for an underlying thymic neoplasm.

• MeSH
• dospělí MeSH
• granulom komplikace   diagnóza   patologie   MeSH
• jehlová biopsie MeSH
• kosterní svaly patologie   MeSH
• lidé středního věku MeSH
• lidé MeSH
• myasthenia gravis komplikace   diagnóza   patologie   MeSH
• nemoci svalů komplikace   diagnóza   patologie   MeSH
• sarkoidóza komplikace   patologie   MeSH
• senioři MeSH
• svalová vlákna typu II patologie   MeSH
• Check Tag
• dospělí MeSH
• lidé středního věku MeSH
• lidé MeSH
• mužské pohlaví MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• anglický abstrakt MeSH
• časopisecké články MeSH
• práce podpořená grantem MeSH

• Klíčová slova
• ELECTROMYOGRAPHY *, MYASTHENIA GRAVIS/diagnosis *,
• MeSH
• elektromyografie * MeSH
• lidé MeSH
• myasthenia gravis diagnóza   MeSH
• svalová slabost * MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• časopisecké články MeSH

Seronegative myasthenia gravis (MG) presents a serious gap in MG diagnosis and understanding. We applied a cell based assay (CBA) for the detection of muscle specific kinase (MuSK) antibodies undetectable by radioimmunoassay. We tested 633 triple-seronegative MG patients' sera from 13 countries, detecting 13% as positive. MuSK antibodies were found, at significantly lower frequencies, in 1.9% of healthy controls and 5.1% of other neuroimmune disease patients, including multiple sclerosis and neuromyelitis optica. The clinical data of the newly diagnosed MuSK-MG patients are presented. 27% of ocular seronegative patients were MuSK antibody positive. Moreover, 23% had thymic hyperplasia suggesting that thymic abnormalities are more common than believed.

• Klíčová slova
• Autoantibodies, Cell based assay, Diagnosis, MuSK, Myasthenia gravis,
• MeSH
• autoprotilátky krev   MeSH
• dospělí MeSH
• hyperplazie thymu diagnóza   MeSH
• lidé středního věku MeSH
• lidé MeSH
• mezinárodní spolupráce MeSH
• myasthenia gravis krev   diagnóza   patologie   MeSH
• neuromyelitis optica diagnóza   MeSH
• proteiny související s LDL-receptory imunologie   MeSH
• průtoková cytometrie MeSH
• radioimunoanalýza MeSH
• receptory cholinergní imunologie   MeSH
• senioři MeSH
• thymus patologie   MeSH
• tyrosinkinasové receptory imunologie   MeSH
• Check Tag
• dospělí MeSH
• lidé středního věku MeSH
• lidé MeSH
• mužské pohlaví MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• práce podpořená grantem MeSH
• Názvy látek
• autoprotilátky MeSH
• LRP4 protein, human MeSH Prohlížeč
• proteiny související s LDL-receptory MeSH
• receptory cholinergní MeSH
• tyrosinkinasové receptory MeSH

Non-neoplastic thymuses were examined in 63 patients suffering from myasthenia gravis. Germinal centres were found in 61 patients, minute capsular and septal foci consisting of histiocytes and eosinophilic leucocytes were in 27 cases. An opinion was discussed that lesion close to germinal centres could be related to immune reaction after myogenic induction in thymuses from cases of myasthenia gravis.

• MeSH
• lidé MeSH
• myasthenia gravis patologie   MeSH
• thymus patologie   MeSH
• Check Tag
• lidé MeSH
• mužské pohlaví MeSH
• ženské pohlaví MeSH
• Publikační typ
• anglický abstrakt MeSH
• časopisecké články MeSH