AGUIAR, Aline de Almeida Xavier. Miastenia gravis no Ceará, Brasil : aspectos clínicos e epidemiológicos. 2010. 116 f. Dissertação (Mestrado) - Universidade Federal do Ceará. Faculdade de Medicina. Programa de Pós-Graduação em Ciências Médicas, Fortaleza, 2010. / Submitted by denise santos (denise.santos@ufc.br) on 2011-10-07T16:01:23Z No. of bitstreams: 1 2010_dis_aaxaguiar.pdf: 755701 bytes, checksum: 53d91cb84d6291d111a641e9804b6b8b (MD5) / Approved for entry into archive by Eliene Nascimento(elienegvn@hotmail.com) on 2011-10-07T16:34:05Z (GMT) No. of bitstreams: 1 2010_dis_aaxaguiar.pdf: 755701 bytes, checksum: 53d91cb84d6291d111a641e9804b6b8b (MD5) / Made available in DSpace on 2011-10-07T16:34:05Z (GMT). No. of bitstreams: 1 2010_dis_aaxaguiar.pdf: 755701 bytes, checksum: 53d91cb84d6291d111a641e9804b6b8b (MD5) Previous issue date: 2010 / A retrospective chart review of patients diagnosed as having myasthenia gravis, followed in Hospital Universitário Walter Cantídio, Hospital Geral de Fortaleza and by neurologists in Ceará, Brazil, from October 1981 to June 2009 was performed. Clinical and epidemiologic aspects were evaluated. In this work, 122 patients were studied, of whom 85 (69.7%) were females and 37 (30.3%) were males. The disease duration ranged from five months to 50 years (8.9 ± 8.1 years). Age at the first symptoms varied from 0 to 74 years (31.9 ± 14.4 years). The main first symptoms and signs were ptosis, diplopia and member weakness. Generalized myasthenia was the most common clinical presentation, but 5.1% (n=6) persisted as ocular myasthenia. Thymectomy was performed in 42.6% (n=52) of myasthenic patients. A thymoma was present in 10 patients. Serum acetylcholine receptor (AChR) antibodies were present in 80% (n=20) of specimens tested. / Foram analisados, retrospectivamente, os prontuários de pacientes com diagnóstico de miastenia grave (MG), diagnosticados e seguidos no Hospital Universitário Walter Cantídio, Hospital Geral de Fortaleza e por neurologistas que tratavam doenças neuromusculares, no período de outubro de 1981 a junho de 2009 no Estado do Ceará, Brasil. Foram coletados dados clínicos e epidemiológicos. Foram estudados 122 pacientes, sendo 85 (69.7%) do sexo feminino e 37 (30.3%) do sexo masculino. O tempo de duração da doença variou de cinco meses a 50 anos (8.9 ± 8.1 anos). A idade de inicio da doença variou de zero a 74 anos (31.9 ± 14.4 anos). Na amostra estudada, os primeiros sintomas foram principalmente ptose palpebral superior, diplopia e fraqueza dos membros. A maioria dos pacientes apresentou a forma generalizada, enquanto 5.1% (n= 6) persistiram com miastenia ocular. Timectomia foi realizada em 42.6% (n=52) dos pacientes. A presença de timoma foi demonstrada em 10 pacientes. Anticorpos anti-receptor de acetilcolina estavam presentes em 80% (n=20) das amostras testadas.

重症肌無力是一種全身性的獲得性自身免疫性疾病，傳統的分型有眼肌型、延髓肌型和全身型。西醫目前主要以藥物治療和手術治療為主要方法，尚無特效藥物根治本病。中醫歷代文獻中並無“重症肌無力’，病名的記載，重症肌無力屬中醫學中“鞘、“棲症、“唯目、“胞垂、“臉廢等範疇。本病為現代難治性病症之一，而中醫對本病的治療有一定優勢。在治療上加用中醫中藥，可以減少免疫抑制劑帶來的副作用，中藥在重症肌無力的治療上起著保駕護航的作用，而且有重建自身免疫功能之功效。 通過對現代文獻中重症肌無力的中醫用藥進行統計，瞭解重症肌無力在醫學發展史上的歷史沿革與研究進展，汲取百家用藥經驗，對其病因病機，辨證分型進行探討，總結出中醫治療重症肌無力的用藥規律，為今後的學習、研究及應用提供一個思路和借鑒。 通過進人香港浸會大學圖書館“中文科技期刊資料庫（醫藥衛生及自然科學的生物學專輯）的“維普資訊網以“中藥治療重症肌無力’，為關鍵字進行搜索，搜索到近十年相關期刊文獻183 篇，根據文獻的納入標準與排除標準進行篩選後，得到5 0 篇符合標準的有關文獻，繼而進行資料整理和分析，對每一篇文獻中重症肌無力的治療方法、中醫用藥及研究進展進行記錄，針對中醫用藥情況建立頻數分佈表以分析其構成比， 從而探討中藥治療重症肌無力的用藥規律。最常見的重症肌無力中醫用藥有1 0 個，分別是黃、黨參、臼朮、當歸、升麻、甘草、拘把子、山藥、柴胡、陳皮。結論：黃是現代醫家普遍認同的重症肌無力中醫用藥，將為今後重症肌無力的中醫文獻及中醫基礎理論研究提供可行的借鑒。

Orientador: Lineu Cesar Werneck / Co-orientador: Rosana Herminia Scola / Dissertação (mestrado) - Universidade Federal do Paraná, Setor de Ciências da Saúde, Programa de Pós-Graduação em Medicina Interna / Resumo: A miastenia grave é uma doença da junção neuromuscular com sintomas marcantes de fraqueza e fatigabilidade fácil da musculatura ocular isolada ou associada com outros músculos esqueléticos de forma generalizada, piorando com esforço e melhorando com repouso e drogas anticolinesterásicas. Apresenta-se sob as formas neonatal, congênita e adquirida. O diagnóstico da miastenia grave fundamenta-se no quadro clínico, testes farmacológicos, eletrofisiológicos e dosagem de anticorpo anti-receptor de acetilcolina (AAChR), Este estudo objetivou uma avaliação de 153 miastênicos quanto aos aspectos clínicos, gerais e específicos da doença, verificando os melhores métodos de investigação complementar utilizados e comparando os diversos procedimentos terapêuticos conservador e cirúrgico diante da evolução dos doentes. O uso da piridostigmina, prednisona, outros imunossupressores e plasmaférese teve eficácia clínica, destacando os pacientes com idade abaixo de 50 anos. Não houve diferença estatística entre pacientes submetidos a tratamento cirúrgico e conservador quanto à remissão e melhora. Contudo, os dados deste estudo são sugestivos de que os diversos procedimentos terapêuticos influenciam favoravelmente a evolução clínica da doença que segue seu curso autolimitado independente do tratamento utilizado. / Abstract: Myasthenia gravis is a disease of the neuromuscular junction with remarkable symptoms of weakness and easy fatigability of ocular muscles isolated or associated with other skeletal muscles in a generalized form; this may increase with physical effort and it improves with rest and anticolinesterase drugs. Myasthenia gravis has three clinical forms: neonatal, congenital and acquired. The diagnosis of myasthenia gravis is based upon the clinical picture, pharmacological tests, electrophysiological studies and dosage of antibodies against receptors of acetylcholine (AAChR). Our study aimed at analyzing 153 myasthenic patients as to the specific and general clinical aspects of the disease in order to identify the best methods of complementary investigation usualy used and to compare with the different conservative and surgical therapeutical prodedures in correlation with the evolution of the patients. The use of prostigmine. prednisone, other immuosupressants and plasmapheresis has had clinical efficacy, mainly in patientes under 50 anos old. There was no significant statistical difference between patients submitted to surgical and conservative treatment as to remission and improvement. However, our findings suggest that the different therapies have a positive influence on the clinical evolution of the disease that follows its autolimited course independent on the kind of therapy.

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Orientador: Daniele Cristina Cataneo / Resumo: Objetivo: avaliar, por meio de uma revisão sistemática, a efetividade da plasmaférese no pré-operatório de timectomia em pacientes com miastenia gravis (MG). Métodos: Foram pesquisadas as bases Medline, Embase, Lilacs, Scopus e Central para busca de estudos experimentais e observacionais que avaliaram a plasmaférese no pré-operatório de timectomia em pacientes com MG. Grupo com plasmaférese (PPG) e grupo sem plasmaférese (NPPG). Os desfechos avaliados foram: crise miastênica, mortalidade, pneumonia, sangramento, uso de ventilação mecânica, tempo de permanência hospitalar e em unidade de terapia intensiva (UTI). Utilizou-se para metanálise o software RevMan 5.3 fornecido pela Colaboração Cochrane. Resultados: O número total de pacientes avaliados em seis estudos selecionados foi de 323 (143 PPG e 180 NPPG). A plasmaférese pré-operatória não diminuiu a crise miastênica (RR 0,36, IC 95% 0,08 a 1,66; I2 = 44%; 5 estudos, 243 pacientes). Também não houve alteração na mortalidade (RR 0,7, IC 95% 0,11 a 4,62; I² = 0%; 3 estudos, 172 pacientes) ou taxa de pneumonia (RR 0,28, IC 95% 0,07 a 1,09; I2 = 27%; 5 estudos, 272 pacientes). Os pacientes do grupo NPPG sangraram menos em comparação com o grupo PPG (diferença média 34,34 ml, IC 95% 24,93 a 43,75; I² = 0%). Foi avaliada a necessidade de ventilação mecânica em três estudos (213 pacientes) e a permanência hospitalar e em UTI em dois (121 pacientes), que não foram adequados para realizar a metanálise devido à alta heterogeneidade nes... (Resumo completo, clicar acesso eletrônico abaixo) / Abstract: Objective: to evaluate, through a systematic review, the efficacy of plasmapheresis in the preoperative thymectomy in patients with myasthenia gravis (MG). Methods: Medline, Embase, Lilacs, Scopus and Central databases were searched for experimental and observational studies that evaluated plasmapheresis in the preoperative period of thymectomy in MG patients. Plasmapheresis group (PPG) and without plasmapheresis group (NPPG). The outcomes evaluated were: myasthenic crisis, mortality, pneumonia, bleeding, use of mechanical ventilation, length of hospital stay and intensive care unit (ICU) stay. The RevMan 5.3 software provided by the Cochrane Collaboration was used for meta-analysis. Results: The total number of patients evaluated in six included studies was 323 (143 PPG and 180 NPPG). Preoperative plasmapheresis did not decrease the myasthenic crisis (RR 0.36, 95% CI 0.08 to 1.66, I2 = 44 %; 5 studies, 243 patients). There was also no change in mortality (RR 0.7, 95% CI 0.11 to 4.62, I² = 0%; 3 studies, 172 patients) or pneumonia (RR 0.28, 95% CI 07 to 1.09, I2 = 27%; 5 studies 272 patients). Patients in the NPPG group bleed less in comparison to the PPG group (mean difference 34.34 ml, 95% CI 24.93 to 43.75, I² = 0%). We evaluated the need for mechanical ventilation in three studies (213 patients) and hospital and ICU stay evaluated in two studies (121 patients), but they were not adequate to perform the meta-analysis due to the high heterogeneity among these outcomes. Subg... (Complete abstract click electronic access below) / Mestre

Diss. (sammanfattning) Stockholm : Karolinska institutet, 2005. / Härtill 4 uppsatser.

Diss. (sammanfattning) Stockholm : Karol. inst., 2003. / Härtill 5 uppsatser.

Myasthenia Gravis (MG) is an autoimmune disorder of neuromuscular transmission. Clinically, the disease is manifested by abnormal muscle fatigue with recovery on resting. Circulating nicotinic acetylcholine receptor antibodies (nAchR Ab) are highly characteristic of myasthenia gravis. These antibodies have been shown to be directly pathogenic at the muscle endplate and are responsible for impaired neuromuscular transmission through several mechanisms. While it is clear that the immune system does not function normally in MG, the mechanisms by which the response to nAchR is initiated and perpetuated remain unknown. Moreover, it is not clear whether immunoregulatory defects actually precede development of MG or are secondary features of the disease. The overall goal of the present investigation has been to more clearly define the nature of the immune regulatory defects existing in MG, both at the cellular level and in terms of possible relationship to disease progression. To begin these studies it was necessary to develop an assay that could be used to measure nAchR Ab secreted by lymphocytes in culture. Thus, we modified the original nAchR Ab immunoassay described by Lindstrom (1976) for this purpose. Additionally, in order to gain access to an appropriate patient base for our study, we established a further modification with improved sensitivity for detection of serum nAchR Ab. This important diagnostic test had not been available in this country. Therefore, our assay was made available in Canada for clinical purposes. Through the study of in vitro nAchR Ab and polyclonal IgG secretion by peripheral blood mononuclear cells (PBMNC), we were able to identify two previously unrecognized subgroups of seropositive, generalized MG patients. PBMNC from patients with long disease duration had low capacity for in vitro Ab production (Nonsecretors). Among patients of short disease duration, PBMNC produced nAchR Ab and also secreted higher than normal levels of polyclonal IgG (Secretors). The data suggested that there were nonspecific abnormalities affecting the immune response in myasthenia gravis. Moreover, regulation of B lymphocyte mediated immune function appeared to be related to disease progression. It was hypothesized that circulating auto-antibody may contribute to deregulation of the immune response at certain stages of disease through direct interactions with leukocyte determinants. Separation/reconstitution experiments with CD4+ enriched, T-helper/inducer lymphocytes and B enriched (E- cells) lymphocytes suggested that the control of antibody production in myasthenia gravis was operative at the T-helper/inducer level. Preliminary studies with serum pretreated, CD4+ enriched, T-helper/inducer lymphocytes suggested that serum of Secretor MG patients indeed contained a factor(s) which interfered with the function of a CD4+ lymphocyte subset. We further hypothesized that nAchR Ab would have the potential to behave as anti-lymphocyte Ab if nAchR were expressed on lymphocytes. Accordingly, direct binding studies, using the nicotinic antagonist, alpha-bungarotoxin, were carried out to look for such receptors on PBMNC. Specific, saturable binding of alpha-bungarotoxin to the rhabdomyosarcoma cell line, TE671, was confirmed and characterized. However, in parallel studies, alpha-bungarotoxin binding to PBMNC of healthy individuals or MG patients was not detected. These results suggested that nicotinic acetylcholine receptors, of the type expressed by muscle endplate, do not occur on human peripheral blood mononuclear cells. / Medicine, Faculty of / Pathology and Laboratory Medicine, Department of / Graduate

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