Demyelinating disease in patients with myasthenia gravis Doenças desmielinizantes em pacientes com miastenia gravis

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Denis Bernardi Bichuetti

2008-03-01

Full Text Available Myasthenia gravis (MG is an autoimmune disease characterized by fluctuating muscle weakness, caused by impaired neuromuscular transmission. Patients with MG can present other autoimmune diseases in association, commonly hypo or hyperthyroidism. The association of MG to demyelinating disease is rare and has been described before. We report on three Brazilian patients with MG that presented distinct demyelinating diseases, two monophasic and one recurrent neuromyelitis optica, several years after the diagnosis of MG, and discuss their clinical courses.Miastenia gravis (MG é doença autoimune caracterizada por episódios de fraqueza muscular alternados com melhora, causada por bloqueio da junção neuromuscular. Pacientes com MG podem apresentar outras doenças autoimunes, comumente hipo ou hipertiroidismo, e a associação de MG com doenças desmielinizantes é raramente descrita. Relatamos três pacientes brasileiros com MG que desenvolveram doenças desmielinizantes, dois monofásicos e um neuromielite óptica recorrente, vários anos após o diagnóstico de MG e discutimos seus cursos clínicos.

Miastenia grave: aspectos históricos Myasthenia gravis: historical aspects

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FRANCISCO MARCOS B. CUNHA

1999-06-01

Full Text Available Foram pesquisados aspectos históricos da miastenia grave desde as primeiras descrições da doença em 1672, pelo clínico inglês Thomas Willis. São descritas as dificuldades encontradas no manuseio dos primeiros pacientes diagnosticados. Pesquisaram-se fatos históricos ligados à investigação da doença, o tratamento, bem como curiosidades pouco citadas na literatura.We studied historical aspects of myasthenia gravis starting from its first description by the English physician, Sir Thomas Willis, in 1672. We also describe the difficulties in managing triating the first diagnosed patient. Historical facts related to the investigation and the initial treatment of this disorder as well as curiosities seldom mentioned in the literature are part of this paper.

Timectomia e imunossupressão em miastenia gravis estudo prospectivo

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Marcos C. Sandmann

1990-03-01

Full Text Available Entre 1982 e 1988 24 mulheres e 6 homens com miastenia gravis foram submetidos a protocolo diagnóstico e terapêutico prospectivo e estandardizado. A idade variou de 10 a 74 anos (34±16, média±desvio padrão. Três com forma ocular foram tratados com piridostigmina. Quatro com forma generalizada, de idade avançada, receberam corticosteróide e/ou azatioprina. Vinte e três com a forma generalizada foram submetidos a timectomia seguida de prednisona e/ou azatioprina. Um paciente faleceu após timectomia complexa de timoma invasivo. Qutro faleceu logo após a admissão, em crise miastênica/colinérgica. Dois outros tiveram complicações menores da timectomia. De 19 pacientes seguidos por 1-60 (média 24 meses, 11 (58% têm fraqueza residual mas levam vida normal e 8 (42% estão em remissão completa, um sem medicação.

Timectomía como tratamiento de miastenia gravis: Experiencia en el Hospital Nacional Dos de Mayo. Lima, Perú

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Julio VASQUEZ KOBASHIGAWA

2006-10-01

Full Text Available La timectomía es una opción terapéutica en el manejo de la miastenia gravis (MG. Aunque existe controversia sobre las indicaciones quirúrgicas y el mejor momento para operar, la timectomía se mantiene como un procedimiento seguro y efectivo para el tratamiento de MG. Aquí revisamos nuestra experiencia con esta operación en pacientes con esta enfermedad, y comparamos nuestros resultados con aquellos publicados en la literatura.

Estudio de apoptosis linfoide por esteroides en 1 caso con miastenia gravis: Seguimiento por ultrasonografía

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Leticia de la Caridad Christian López

2001-03-01

Full Text Available La miastenia gravis es una entidad clínica de origen autoinmune, cuya terapia habitual se realiza con drogas anticolinérgicas, la timectomía o la terapia con esteroides. Fue el objetivo del trabajo el conocer los cambios que se producen en el tamaño del área tímica, con el uso de esteroides, en una paciente de 2 años de edad, que presentaba miastenia gravis juvenil, con una hiperplasia tímica. Esta glándula alcanzó un área máxima de 1 928 mm. Con el uso de la prednisona a 60 mg por semanas se produjo una timectomía medicamentosa, con la reducción del área tímica a 439 mm y remisión total de la sintomatología. Se realizaron las mediciones periódicamente durante un año de los cambios del área de este órgano. No se produjeron recaídas de la enfermedad de base. La ultrasonografía demostró ser un método útil, por ser rápido, barato y no invasiva y permite un ajuste adecuado de la dosis de esteroides que se administrará.Myasthenia gravis is a clinical agent of autoimmune origin, whose habitual therapy includes anticholinergic drugs, thymectomy, or therapy with steroids. The objective of this paper was to know the changes that occur in the size of the thymic area in a 2-year-old patient with juvenile myasthenia gravis and with thymic hyperplasia. This gland reached a maximum area of 1 928 mm and with the use of 60 mg of prednisone per week a medicamentosus thymectomy was performed with the reduction of the thymic area to 439 mm and total remission of the symptomatology. The changes of the area of this organ were periodically measured during a year. There were no relapses of the base disease. The ultrasonography proved to be a useful method, since it is fast, cheap and noninvasive and allows an adequate control of the steroids dose to be administered.

Treatment of cervical dystonia with botulinum toxin in a patient with myasthenia gravis Tratamento de distonia cervical com toxina botulínica em uma paciente com miastenia gravis

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MARCIA RUBIA R. GONÇALVES

1999-09-01

Full Text Available We report the case of a 49-year-old woman who has the rare combination of myasthenia gravis and cervical dystonia. She was treated with botulinum toxin type A with good response and no evidence of deterioration of the myasthenic symptoms. We therefore conclude that it is possible to use botulinum toxin in the presence of defective neuromuscular transmission.Relatamos o caso de uma mulher de 49 anos com rara combinação de miastenia gravis e distonia cervical tratada com toxina botulínica tipo A, apresentando boa resposta e nenhuma evidência de piora do quadro miastênico. A partir dessas observações concluimos que é possível o uso de toxina botulínica na presença de doença da transmissão neuromuscular.

Estudo da correlação hla em uma população e miastenia gravis brasileira

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Elza Dias-Tosta

1990-03-01

Full Text Available Sabendo-se da miscigenação da população brasileira, o que a diferencia de caucasianos, amarelos ou negros americanos, os autores estudaram a correlação HLA e miastenia gravis em 37 pacientes e os compararam com o grupo controle estudado no mesmo laboratório, composto de 69 indivíduos normais. Foi encontrado um risco relativo (RR estatisticamente significante para HLA B8 em pacientes jovens do sexo feminino. Também alto foi o RR para B8 no grupo com hyperplasia tímica e de Al e B8, para o grupo da raça negra.

Resultado da timectomia ampliada no tratamento de pacientes com Miastenia gravis Extended thymectomy for treating patients with Myasthenia gravis (MG

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Raul Lopes Ruiz Jr

2004-04-01

Full Text Available INTRODUÇÃO: Diversas variações da timectomia podem ser realizadas, dentre elas a transesternal ampliada. A literatura sugere que, quanto mais extenso o procedimento para ressecção da glândula e tecidos do mediastino anterior, melhores os resultados e prognóstico. OBJETIVO: Avaliar retrospectivamente a resposta à timectomia ampliada em portadores de Miastenia gravis. MÉTODO: Foram avaliados 46 portadores de Miastenia gravis, submetidos à plasmaferese pré-operatória e à timectomia ampliada, entre agosto de 1992 e janeiro de 2003, divididos em três grupos, segundo o tempo decorrido desde o início dos sintomas: menor que 12 meses, 13 a 24 meses e maior que 25 meses. RESULTADOS: Trinta e um pacientes eram do sexo feminino e 15 do masculino. A média de idade foi de 30 anos. O tempo médio de evolução da doença foi de 26,3 meses. O acompanhamento ambulatorial pós-operatório foi em média de 26,6 meses. Quanto ao grau de resposta à timectomia, 89% dos pacientes tiveram boa resposta, sendo que 50% apresentaram remissão completa. Ocorreu um óbito nesta série. O exame anatomopatológico demonstrou que a hiperplasia tímica foi o achado mais freqüente. Apenas 3 pacientes (6,5% apresentaram timomas benignos. Em 5 pacientes (10,8% encontramos tecido tímico extraglandular: na gordura peritímica em 2 deles, na gordura pericárdica em 1, junto ao nervo frênico esquerdo em outro e na janela aorto-pulmonar em outro. CONCLUSÃO: A timectomia ampliada para tratamento da Miastenia gravis mostrou-se segura, eficiente, e apresentou alta porcentagem de remissão completa. Houve a detecção de tecido tímico extraglandular em alguns pacientes. Tão logo seja feito o diagnóstico, está indicada como terapêutica associada à plasmaferese pré-operatória e à medicamentosa, independentemente da idade, patologia tímica, e início dos sintomas.BACKGROUND: Extended thymectomy is one of several types of thymectomy. Literature suggests that the

Timectomía videotoracoscópica en la miastenia gravis Video-laparoscopic thymectomy in myasthenia gravis

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Miguel Ángel Martín González

2012-03-01

Full Text Available Introducción: el tratamiento videotoracoscópico permite la exéresis del timo y de la grasa peritímica, como en la cirugía abierta, pero con las ventajas del mínimo acceso. Objetivo: evaluar los resultados de esta vía con nuestros primeros pacientes. Métodos: se realizó un estudio descriptivo y prospectivo de 10 pacientes con miastenia gravis desde enero de 2007 hasta agosto de 2008, en el hospital "Hermanos Ameijeiras". Los resultados se presentaron en por cientos y para la relación de las variables se empleó chi cuadrado. Resultados: según la clasificación de Osserman de la miastenia, 8 se ubicaron en el grado II B, y 2 en el II A. El 50 % (5 de ellos tenía un timoma asociado. En el 50 % se emplearon 3 puertos de entrada. El tiempo quirúrgico varió de 60 a 180 minutos. Hubo un 37,5 % (3 de complicaciones posoperatorias sin mortalidad. En el informe anatomopatológico de la pieza (4 el 50 % tenía un timoma, todos en el estadio I de Masaoka. El 50 % (4 se encuentra en remisión y el otro 50 % en mejoría significativa. Conclusiones: la timectomía videotoracoscópica tiene grandes ventajas, ya que sin cambiar la técnica quirúrgica los pacientes se benefician de todas las ventajas del mínimo acceso.Introduction: the video-laparoscopy treatment allows the thymus exeresis and the peri-thymic fat like in the open surgery, but with the advantages of a minimal access. Objective: to assess the results of this route achieved in our first patients. Methods: a prospective and descriptive study was conducted in 10 patients presenting with myasthenia gravis from January, 2007 to August, 2008 admitted in the "Hermanos Ameijeiras" Clinical Surgical Hospital. The results were showed in percentages and for the relation of variables chi² was used. Results: according to the Osserman's classification of the myasthenia, 8 were located in the IIB degree and 2 in the IIA one. The 50 % (5 of them had an associated thymoma. In the 50 % three

Haptoglobin study in myasthenia gravis Estudo sobre a haptoglobina na miastenia grave

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Leonardo H. Mendonça Oliveira

2008-06-01

Full Text Available OBJECTIVE: A cross-sectional study of haptoglobin (Hp in myasthenia gravis (MG was designed, with the objective to identify its values and correlate them with different disease status. METHOD: 46 patients were enrolled in the study, all having disease severity established according to the quantitative myasthenia gravis strength scores (QMGSS. Based on the functional scale determined by Myasthenia Gravis Foundation of America (MGFA recommendations, patients were classified as having: complete stable remission (CSR; n=10; minimal manifestations-0 (MM0; n=6, minimal manifestations-1 (MM1; n=4; pharmacological remission (PR; n=6. Two other groups participated: thymomatous patients (T; n=10 and patients without imunosuppression or thymectomy, until the assessment for Hp (WIT; n=10. Hp dosage was done by immunonephelometry, blindly to clinical data. Student's t-test, Anova test and linear regression were employed for statistical analyses. RESULTS: Statistically significant differences occurred between CSR+MM0xWIT groups (86.62x157.57, pOBJECTIVO: Desenhou-se estudo transversal sobre a haptoglobina (Hp na miastenia grave (MG com o objetivo de identificar seus valores e correlacioná-los a diferentes condições na doença. MÉTODO: 46 pacientes foram incluídos, todos tendo a gravidade da doença estabelecida segundo escores internacionais (QMGSS. Os pacientes tiveram seu estado funcional determinado de acordo com a Myasthenia Gravis Foundation of América (MGFA e classificados em: remissão completa estável (CSR; n=10; mínima manifestação-0 (MM0; n=6, mínima manifestação-1 (MM1; n=4; remissão farmacológica (PR; n=6. Dois outros grupos participaram: pacientes timomatosos (T; n=10 e pacientes sem imunossupressão ou timectomia, até o momento da inclusão no estudo (WIT; n=10. A dosagem de Hp foi realizada por imunonefelometria, de modo cego quanto à clínica. As análises estatísticas incluíram o teste de Student, Anova e regressão linear

Corticosteroides no tratamento da miastenia grave. Estudo de 12 casos com revisão da literatura Corticosteroids in the management of myasthenia gravis

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José Lamartine de Assis

1975-03-01

Full Text Available Foram estudados os resultados do tratamento da miastenia grave em 12 pacientes, sendo 10 com a forma generalizada severa, um com a forma generalizada moderada e um com miastenia acentuada associada a polimiosite. Sete pacientes foram previamente timectomizados e somente um apresentava timoma. Todos estavam em uso de doses elevadas de drogas anticolinesterásicas com resposta terapêutica pobre ou nula. Um paciente foi tratado com dexametaxona, outro com dexametazona seguida de prednisona e os restantes com prednisona, sempre em doses altas inicialmente (100 mg por dia ou em dias alternados e com redução gradativa ulterior até doses de manutenção. Os corticosteróides foram administrados sempre pela via oral, por períodos que variaram de duas semanas até dois anos e meio. Em conclusão, 75% de 12 pacientes com miastenia grave generalizada foram influenciados favoravelmente com este método terapêutico. Deste grupo apenas um apresentava timoma e não respondeu a qualquer tratamento. Outro paciente apresentava polimiosite associada e teve excelente resposta terapêutica. Dois miastênicos sem timoma tiveram a sintomatologia exacerbada durante o tratamento. Nenhum efeito colateral importante foi observado, mesmo nos pacientes tratados a longo prazo.The results of treatment of myasthenia gravis in 12 patients are reported. There were 10 cases with severe generalized form and two cases with moderate generalized form, one of which associated with polymyositis. Seven patients had prior thymectomy and one of them had a thymoma. All the patients were receiving anticholinesterase drugs with poor response or without any response. One patient received a short, intensive course of dexametazone, and other patient used prednisone after a more prolonged dexametazone course. The remaining patients received prednisone, always beginning with high (100 mg alternate day oral single-doses. This therapy has been maintained for a period thought to be sufficient

Miastenia grave y miastenia grave ocular

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Rosa María Naranjo Fernández

Full Text Available La miastenia grave es una enfermedad caracterizada por debilidad y fatiga de los músculos voluntarios debido a una trasmisión anómala a nivel de la unión neuromuscular. La prevalencia es aproximadamente de 5 casos/100 000 personas. La miastenia grave puede ser bulbar, ocular o generalizada.Existen formas clínicas en la infancia como son la miastenia neonatal transitoria, la miastenia congénita y la miastenia juvenil. Los músculos oculares, faciales y bulbares son los más frecuentes afectados por la enfermedad. Cuando los síntomas se limitan a la musculatura cercana al ojo se denomina miastenia grave ocular. Una vez el oftalmólogo diagnostica o sospecha la miastenia grave, un neurólogo generalmente dirige la comprobación y tratamiento. El papel del oftalmólogo continúa siendo importante, además de chequear la motilidad y disfunción palpebral y proporcionar el alivio sintomático para estos desórdenes, debe estar alerta a la posibilidad de ambliopía.

Associação familiar de miastenia grave e síndrome miotônica Familial association of myasthenia gravis and myotonic syndrome

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Sylvio Saraiva

1969-12-01

Full Text Available É relatada uma associação familiar incomum, incidindo em mãe e filha, a primeira com síndrome miotônica e, a segunda, com miastenia grave, forma ocular. Um terceiro membro da família, irmão da paciente com miotonia, teria também manifestações dessa síndrome. São comentados aspectos clínico-laboratoriais dos casos estudados.An uncommon association of myasthenia gravis in a child and a myotonic syndrome in her mother is reported. A brother of the second patient probably had myotonic symptomatology. Clinical and laboratorial aspects are discussed.

Dandy-Walker syndrome in adult mimicking myasthenia gravis Síndrome de Dandy-Walker em adulto simulando miastenia gravis

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Juliana Cardoso

2007-03-01

Full Text Available The Dandy-Walker syndrome (DWS is a rare posterior fossa malformation. The DWS can occur associated with other brain or systemic malformations, but ocular abnormalities in this disease are rare and clinical findings mimicking myasthenia gravis have not been described to date. We report a 23-year-old woman who presented mild limitation of the ocular movements with progressive palpebral ptosis, which changed in intensity during the day. The investigation showed negative anti-acetylcholine receptor antibody, repetitive nerve stimulation and "Tensilon test", but the brain magnetic resonance image reveals DWS with hydrocephalus associated with calosal dysgenesis. The characteristic of disease, clinical manifestations and pathologic features, specially the clinical evaluation of ocular abnormalities in suspicion of DWS, including the MG in differential diagnosis are discussed.A síndrome de Dandy-Walker (DWS é uma rara malformação da fossa posterior que pode ocorrer associada com outras malformações cerebrais ou sistêmicas. As alterações oculares são raras e as manifestações clínicas, simulando miastenia gravis (MG, não foram descritas até o momento. Descrevemos uma mulher de 23 anos apresentando discreta limitação da movimentação ocular com progressiva ptose palpebral que mudava de intensidade durante o dia. A investigação mostrou negativos o anticorpo anti-receptor de acetilcolina, a estimulação nervosa repetitiva e o "teste do Tensilon", porém a ressonância magnética de crânio revelou DWS com hidrocefalia associada à disgenesia de corpo caloso. As características da doença, manifestações clínicas e patológicas, especialmente a avaliação clínica de anormalidade ocular na suspeita de DWS serão discutidas, incluindo a MG no diagnóstico diferencial.

Megaesophagus secondary to myasthenia gravis in a female German shepherd dog/ Megaesôfago secundário a miastenia grave em uma cadela da raça Pastor Alemão

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Osimar Sanches

2007-08-01

Full Text Available Megaesophagus is a common cause of regurgitation being one of the possible etiologies the myasthenia gravis characterized by a neuromuscular disorder that result in weakness of the skeletal muscles, of the esophagus, of the larynx and of the pharynx. The present report describes a case of a female German shepherd dog, with report of muscles weakness and frequent vomits, which was diagnosed as a megaesophagus secondary to myasthenia gravis. The radiograph of the animal presented an accentuated cervical and thoracic megaesophagus. After the institution of the recommended therapeutic protocol the animal presented visible improvement when walking, however the regurgitations worsened and it began to present cough. New radiograph was accomplished with presence, besides megaesophagus, of aspiration pneumonia. The owner opted for the euthanasia of the animal and the necropsy revealed severe pneumonia and an accentuated dilation of the posterior portion of the esophagus, collaborating with subsidies for an unfavorable prognostic of the case.Megaesôfago é uma causa comum de regurgitação sendo uma das possíveis etiologias a miastenia grave caracterizada por uma desordem neuromuscular que resulta em fraqueza dos músculos esqueléticos, do esôfago, da laringe e da faringe. O presente relato descreve um caso de uma cadela da raça Pastor Alemão, com histórico de fraqueza muscular e vômitos freqüentes, que foi diagnosticada como megaesôfago secundário à miastenia grave. A radiografia do animal apresentou um acentuado megaesôfago cervical e torácico. Após a instituição do protocolo terapêutico recomendado o animal apresentou melhora visível ao caminhar, porém as regurgitações pioraram e começou a apresentar tosse. Foi realizado novo exame radiográfico com presença, além de megaesôfago, de pneumonia aspirativa. A proprietária optou pela eutanásia do animal e a necropsia revelou severa pneumonia e dilatação acentuada da por

Timomas y miastenia gravis: Supervivencia y factores pronósticos, catorce años de experiencia

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Carlos A Martínez Blanco

2003-09-01

Full Text Available Se reporta un estudio de cohorte analítico para evaluar factores pronósticos en 34 pacientes con timoma y miastenia gravis, a los cuales se les realizó timectomía, entre enero de 1984 y diciembre de 1998, 22 femeninos y 12 masculinos, con un promedio de edad de 49,7 años. De acuerdo con la clasificación clínica de Masaoka, 14 pertenecieron al estadio I, 2 al II, 12 al III y 6 al IV. Histológicamente 17 fueron mixtos, 9 corticales, 5 medulares y 3 carcinomas tímicos bien diferenciados. Según la clasificación de Osserman 5 enfermos se encontraban en el grupo IIa, 23 en el IIb y 6 en el IV. La vía de acceso fue la esternotomía media. Los pacientes con tumores invasivos recibieron radioterapia y poliquimioterapia adyuvante. El índice de remisión de los síntomas miasténicos al año fue de 53,1%. Hubo 3 fallecidos en el período posoperatorio y 7 tardíamente. El timoma cortical y, en general, los tumores invasivos fueron los de peores resultados. Los factores que más se relacionaron con el pronóstico por su influencia sobre el intervalo libre de enfermedad y la supervivencia a largo plazo, fueron el estadio clínico del tumor, el tipo hístico y el tipo de miasteniaThis paper presents an analytical cohort study to evaluate prognostic factors in 34 patients with thymona and myasthemia gravis whom thymectomy from January 1984 to December 1998. Twenty two were females and 12 males aged 49,7 years of age as average. According to Masaoka´s clinical classification, 14 patients were at stage I; two at stage II, 12 at stage III and 6 at stage IV. From the histological viewpoint, 17 were mixed thymona, 9 cortical thymonas, 5 medullar thymonas and 3 well-differentiated thymic carcinomas. According to Osswerman´s classification, 5 patients were included in group IIa, 23 in group IIb and 6 in group IV. The access pathway was median sternotomy. Remission index of myasthemic symptoms after a year was 53.1%. there were 3 deaths in the

Sclerosing thymoma: case report Timoma esclerosante: relato de caso

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Gesine Gregorio Siqueira

2012-08-01

Full Text Available We present a rare case of thymoma in a 36-year old woman, who was initially diagnosed with severe myasthenia gravis and subsequently undergone surgical resection. During surgery tumor was found at the anterior mediastinum, tightly attached to the phrenic nerve, pleura and pericardium. Histological assessment showed large areas of sclerosis and fibrous collagenous tissue as well as islands of epithelial and lymphoid cells. Sclerosing thymoma, which is a rare subtype of thymoma (Relatamos um caso raro de timoma em uma mulher de 36 anos de idade, com clínica e diagnóstico de miastenia gravis de difícil controle clínico, submetida à ressecção cirúrgica. No intraoperatório, observou-se tumor no mediastino anterior, firmemente aderido ao nervo frênico, à pleura e ao pericárdio. Ao exame histológico, foram evidenciadas extensas áreas de tecido fibrocolagenoso e esclerose, assim como ilhas de células epiteliais e células linfoides. Diagnosticado timoma esclerosante, subtipo raro de timoma (< 1%, sendo este o primeiro caso relatado no Brasil. A paciente apresentou melhora parcial dos sintomas associados à miastenia gravis.

Myasthenia gravis and thymoma: evaluation of 41 patients Miastenia grave e timoma: avaliação de 41 pacientes

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JOSÉ LAMARTINE DE ASSIS

1999-03-01

Full Text Available We evaluated the epidemiological, clinical, laboratory and therapeutical aspects of 41 patients with thymomatous myasthenia gravis. Thirty five patients (85.36% were submitted to thymectomy. Follow-up ranged from two to 18 years. Diagnosis of thymoma was based upon clinical investigations and CT scan of the anterior mediastinum and in 11 patients supported by immunological tests of anti-striated muscle antibodies with a positive result in more than 80% of cases. Histopathologic examination of all thymomectomized patients confirmed the diagnosis of thymoma. There was a significant predominance of benign over malignant thymoma. Occurred higher prevalence of male patients and of patients over 40 years of age. The therapeutical strategy to control myasthenic clinical findings was the same as that for non-thymomatous myasthenia gravis. The corticosteroids associated to cytotoxic drugs were less often used. Radiotherapy of the anterior mediastinum was more often used in patients having invasive tumors submitted to surgery or not. With regard to survival and control of myasthenia gravis, especially in younger patients and in those submitted to early surgery, results of treatment were surprisingly favorable.Avaliamos 41 pacientes com miastenia grave timomatosa sob os aspectos epidemiológico, clínico e terapêutico. Trinta e cinco pacientes (85,36% foram timectomizados. O seguimento clínico variou de dois meses até 18 anos. O diagnóstico do timoma foi fundamentado no estudo de imagem do mediastino (tomografia axial computadorizada e, em 11 pacientes, complementado com a determinação sérica de anticorpos para músculo estriado com resultado positivo em mais de 80% dos casos e confirmado pelo exame anátomo-patológico do timo realizado em todos os pacientes operados. Ocorreu predomínio significante de timomas benignos sobre timomas malignos, forma clínica generalizada severa, frequente envolvimento do sexo masculino e, em pacientes com mais de

Plasmapheresis as preparatory method for thymectomy in myasthenia gravis Plasmaferese como método preparatório de timectomia em miastenia grave

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José Carlos Brant Seggia

1995-09-01

Full Text Available To study the effects of plasmapheresis in preparation for thymectomy, two groups of 40 patients were selected from a sample of 286 patients with myasthenia gravis examined by the first author Group 1 included patients (15 male and 25 female; age range 8-64 yrs who underwent thymectomy without previous plasmapheresis, whereas patients in group 2 (17 male and 23 female; age range 11-61 yrs were thymectomized after plasmapheresis. We required patients to have a minimum follow-up period of 12 months to be included in the study. A clinical evaluation protocol composed of 76 items was developed for the study. We found significant improvement in respiratory function and muscular strength in patients thymetomized after plasmapheresis. Furthermore, the combined treatment reduced cost and length of hospital stay. Therefore, we conclude that plasmapheresis should be considered as a coadjuvant to thymectomy in the treatment of myasthenia gravis.No intuito de estudar as repercussões de um curso de plasmaferese como método preparatório para a timectomia, tomamos duas amostras de 40 pacientes selecionados de um universo de 286 casos de miastenia gravis, em que a investigação clínica, eletrofisiológica, laboratorial, o seguimento e o protocolo foram completamente realizados. Consideramos como grupo 1 aquele em que a timectomia foi realizada sem preparação pre-operatória e grupo 2 aquele em que a plasmaferese foi executada como coadjuvante a timectomia. O grupo 1 era composto de 15 pacientes do sexo masculino e 25 do feminino; a idade variou de 8 a 64 anos com mediana de 30 anos. No segundo grupo, 17 eram masculinos e 23 femininos; a idade variou de 11 a 61 anos com mediana de 33 anos. Foi preenchida ficha de avaliação clínica sequencial de 76 itens e traçamos um curso de plasmaferese de 5 sessões seguido da timectomia, mantendo a mesma técnica operatória, realizada pela mesma equipe cirúrgica, tentando assim minimizar as distorções na an

Miastenia grave ocular Severe ocular myastenia

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Yaimir Estevéz Miranda

2010-01-01

Full Text Available La miastenia grave es una enfermedad autoinmune de la unión neuromuscular que se caracteriza por la variabilidad de la debilidad muscular, que empeora o está provocada por el ejercicio del músculo o de los grupos musculares implicados, mientras que el descanso la mejora. La primera descripción de la enfermedad data de 1672, su relación con una afección del timo no se estableció hasta 1901. La enfermedad es poco frecuente, afecta a todas las etnias por igual, y no tiene predilección geográfica. Se reporta un paciente masculino de 28 años de edad atendido en el Servicio de Oftalmología Pediátrica del Instituto Cubano de Oftalmología "Ramón Pando Ferrer" por presentar visión doble, ptosis palpebral ligera en ojo derecho que empeora con el transcurso del día. Por el interrogatorio y el cuadro clínico se sospecha de una miastenia grave, se remite al clínico y el neurólogo. En los estudios realizados de neuroimágenes, tomografía y electroencefalograma no se encontraron alteraciones. Se decide valorar el caso con el servicio de Neuroftalmología de nuestro centro y por el Instituto de Neurología y es confirmado el diagnóstico.Myasthenia gravis is an autoimmune illness of the neuromuscular joint that is characterized by the variability of the muscular weakness that worsens or caused by exercising of the muscle or the involved muscular groups; resting helps in improving the condition of the muscle. The first description of the illness dates back to 1672, but its linking to a thymus disorder was not discovered until 1901. This illness is not very frequent, affects equally to all the races, and it does not predominate in any particular geographic region. This paper presented a male patient aged 28 years who went to the Ophthalmologic Service at "Ramón Pando Ferrer" Cuban Institute of Ophthalmology because he suffered from double vision, slight palpebral ptosis in his right eye that became worse as the day went by. After the

[Premature birth in patient with cervix incompetence and history of myasthenia gravis].

Science.gov (United States)

Fuentealba, Maximiliano; Troncoso, Miguel; Vallejos, Joaquin; Ponce, Sebastian; Villablanca, Nelson; Melita, Pablo

2013-09-01

Cervical incompetence it's a dilatation of the cervix during the third trimester of pregnancy that ends with the interruption of it. The incidence in Chile is about 0.1-2% of the total pregnancies and it's one of the causes of preterm birth. A 34 years old pregnant patient. Timectomized at age 18 to treat her miastenia gravis, previously trated with medication, had 4 previous preterm labours all of them under 25 weeks and vaginal births. All fetuses died postpartum. A cerclage was made during the third, fourth and fifth pregnancies. She didn't present hypertension during the gestation and no cervical diameter under 15mm. Since the fourth gestation the following tests are taken: Antifosfolipidic antibodies, APTT,PT. All the results are either normal or negative. Microbial cultures were negative. No amniocentesis was made. A McDonald cervical cerclage was made during pregnancies number 3, 4 and 5 on the 16th week to delay the labor. Also oral micronized progesterone, on a 400mg/24 hours dosis, was administered to avoid preterm birth. On the 24th week the pharmacological treatment started including Intramuscular Betamethasone, 12 mg/24 hours (2 doses), to induce lung maturity on the fetus. It is thought that the administration of progesterone could have improved the situation of the patient, because it acts as a labour repressants. The use of cerclage could have helped, but the factors that may influence the effectiveness of this method are unknown. Perhaps there is some immunologic factor associated with the miastenia gravis that alters the normal course of pregnancy.

Confiabilidade do teste da caminhada de seis minutos em pacientes com miastenia gravis generalizada Reliability of the six-minute walk test in patients with generalized myasthenia gravis

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Vanessa Regiane Resqueti

2009-09-01

Full Text Available Este estudo objetivou determinar a confiabilidade do teste da caminhada de seis minutos (TC6M como um teste de capacidade funcional em pacientes com miastenia gravis generalizada (MG. Foram selecionados 11 pacientes com MG - 5 homens, 6 mulheres - com idade de 55±9 anos, avaliados inicialmente quanto à função fulmonar, que se submeteram a três TC6M em dias diferentes. Durante e/ou após cada teste foram medidas freqüência cardíaca e saturação de oxigênio (por oxímetro portátil, sensação de dispnéia (pela escala de Borg e distância percorrida. Nos três testes as distâncias percorridas foram 498 m, 517 m e 520 m (respectivamente 99%, 103% e 104% do valor predito. Em média, a freqüência cardíaca, dispnéia e saturação de oxigênio mostraram comportamento constante nos três testes. Foram encontradas alta confiabilidade relativa, com coeficiente de correlação interclasse maior que 0,90 entre os testes (TC6M1-TC6M2, 0,960; TC6M1-TC6M3, 0,945; e TC6M2-TC6M3, 0,970 e confiabilidade absoluta de 4%, 3,5% e 4,8%, com reprodutibilidade de 11%, 9,8% e 13,4%, respectivamente para o primeiro, segundo e terceiro testes. Os limites superiores e inferiores de concordância e o valor médio das médias das diferenças (bias calculados pelo teste de Bland-Altman mostraram-se clinicamente aceitáveis. Conclui-se que o TC6M se mostrou seguro, confiável e reprodutível, podendo ser aplicado para avaliação e seguimento da tolerância ao exercício em pacientes com MG generalizada.The purpose of this study was to assess the reliability of the six minutes walking test (6MWT as a functional capacity test for patients with generalized myasthenia gravis (MG. Eleven patients with generalized MG (5 men, six women, aged 55±9 years, were first assessed as to pulmonary function and then submitted to three 6MWT in different days. Heart rate and oxygen saturation were measured (by means of portable oxymeter during, and dyspnea (by the Borg scale and

Esclerodermia, tireoidite e miastenia grave: estudo de um caso

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Antonio L. dos Santos Werneck

1993-11-01

Full Text Available Uma paciente de 36 anos com miastenia grave desenvolveu após dois anos intolerância ao frio, o que conduziu ao diagnóstico de tireoidite de Hashimoto. Quatro anos mais tarde apresentou pele espessada nas mãos (esclerodermia limitada. O quadro clínico e os exames complementares encaminharam o diagnóstico para a forma CREST de esclerodermia sistêmica progressiva. Discute-se a dificuldade diagnóstica da esclerodermia, assim como suas síndromes de superposição. Doença de Hashimoto e miastenia grave constituem associação pouco frequente. A presença de esclerodermia e miastenia grave é rara. Não encontramos na literatura a coincidência destas três doenças.

[Thymus surgery in a general surgery department].

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Mega, Raquel; Coelho, Fátima; Pimentel, Teresa; Ribero, Rui; Matos, Novo de; Araújo, António

2005-01-01

Evaluation of thymectomy cases between 1990-2003, in a General Surgery Department. Evaluation of the therapeutic efficacy in Miastenia Gravis patients. Retrospective study based on evaluation of data from Serviço de Cirurgia, Neurologia and Consult de Neurology processes, between 1990-2003, of 15 patients submitted to total thymectomy. 15 patients, aged 17 to 72, 11 female and 4 male. Miastenia Gravis was the main indication for surgery, for uncontrollable symptoms or suspicion of thymoma. In patients with myasthenia, surgery was accomplish after compensation of symptoms. There weren't post-surgery complications. Pathology were divided in thymic hyperplasia and thymoma. Miastenia patients have there symptoms diminished or stable with reduction or cessation of medical therapy. Miastenia was the most frequent indication for thymectomy. Surgery was good results, with low morbimortality, as long as the protocols are respected.

Ophthalmological affectation: A way to mask Miastenia Gravis. A case purpose.

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Néstor R. Sánchez Dacal

2012-06-01

Full Text Available Myasthenia Gravis MG is an autoimmune and chronic neuromuscular disease characterized by variable of weakness in the skeletal muscles that control the eye movements and it is confused with an ophthalmological disorder. With this presentation we pretend to systematize the Theoretical references about MG which allow making a correct diagnosis of the disease from the experience of a clinical case. The theory about MG regarding the presentation of the disease is discussed, emphasising on the significance of its differential diagnosis with an ophthalmopathy, which will contribute to apply a proper treatment and a satisfactory evolution of the patient, arriving to the conclusion that affectation of the III cranial pair is a way of frequent presentation of MG, being valuable the differential diagnosis of the ophthalmopaties in these entities.

Esclerodermia, tireoidite e miastenia grave: estudo de um caso

OpenAIRE

Werneck,Antonio L. dos Santos; Checcacci-Balod,Tamara; Tuma,Graça

1993-01-01

Uma paciente de 36 anos com miastenia grave desenvolveu após dois anos intolerância ao frio, o que conduziu ao diagnóstico de tireoidite de Hashimoto. Quatro anos mais tarde apresentou pele espessada nas mãos (esclerodermia limitada). O quadro clínico e os exames complementares encaminharam o diagnóstico para a forma CREST de esclerodermia sistêmica progressiva. Discute-se a dificuldade diagnóstica da esclerodermia, assim como suas síndromes de superposição. Doença de Hashimoto e miastenia gr...

Timectomia estendida por cirurgia torácica videoassistida e cervicotomia no tratamento da miastenia Extended thymectomy through video assisted thoracic surgery and cervicotomy in the treatment of myasthenia

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Eduardo Haruo Saito

2003-10-01

Full Text Available INTRODUÇÃO: A relação entre a miastenia e o timo é evidente e o tratamento atual desta condição inclui a timectomia. No entanto, uma revisão de nossa experiência com a timectomia revelou a necessidade do uso de uma técnica mais radical. OBJETIVO: Analisar retrospectivamente pacientes portadores de miastenia gravis que foram submetidos a timectomia radical por videotoracoscopia, ressaltando vantagens do método, complicações, análise histopatológica e resultados em relação ao controle da doença. MATERIAL E MÉTODO: Vinte e um pacientes miastênicos (18 do sexo feminino e três do sexo masculino, com idade variando de 17 a 51 anos foram submetidos a timectomia por videotoracoscopia bilateral associada a cervicotomia transversa com ressecção da glândula tímica, dos tecidos peritímicos e das gorduras pericárdicas direita e esquerda. Todos os tecidos implicados foram analisados pela anatomia patológica separadamente. Houve acompanhamento por período médio de 39,2 meses. RESULTADOS: Não houve mortalidade operatória. Ocorreram duas (9,5% lesões vasculares e um (4,8% paciente passou a apresentar disfonia leve permanente. Dezenove (90,4% pacientes estão com boa evolução, sem medicação ou com dose reduzida da mesma. A histopatologia demonstrou 10 hiperplasias do timo, seis involuções tímicas e cinco timos normais. A presença de tecido tímico ectópico foi detectada em seis (28,6% pacientes. CONCLUSÃO: A timectomia radical por videotoracoscopia ofereceu bom controle da miastenia gravis. Fez-se ressecção de tecido tímico ectópico em alguns pacientes.BACKGROUND: The relationship between myasthenia and the thymus is evident and the current treatment of this condition includes thymectomy. However, a revision of our experience with thymectomy has revealed the necessity of a more radical technique. OBJECTIVE: To analyze, retrospectively, myasthenia gravis patients who underwent videothoracoscopic radical thymectomy

Evaluation of the respiratory function in myasthenia gravis: an important tool for clinical feature and diagnosis of the disease Avaliação da função respiratória na miastenia gravis: importância na caracterização clínica e no diagnóstico da doença

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Paulo A. P. Saraiva

1996-12-01

Full Text Available Myasthenic gravis may affect both inspiratory and expiratory muscles. Respiratory involvement occurred in almost all patients with myasthenia gravis in all clinical forms of the disease: 332 lung function tests done in 324 myasthenic patients without respiratory symptoms (age 34.6 ± 18.3 years were examined. Lung volumes analysis showed that all the patients of both sexes with generalized or ocular myasthenia gravis showed "myasthenic pattern". Male patients with "ocular" form only presented the "myasthenic pattern" with lung impairment and had, from the lung function point of view, a more benign behaviour. Female patients with the "ocular" form exhibited a behaviour of respiratory variables similar to that of the generalized form. It was not observed modification of the variables that suggested obstruction of the higher airways. The "myasthenic pattern" was rarely observed in other neuromuscular diseases, except in patients with laryngeal stenosis.O comprometimento respiratório é fator limitante na evolução clinica da miastenia gravis (MG e as formas clínicas mais graves apresentavam acometimento bulbar e respiratório. Para avaliar a reserva respiratória foram examinados em 324 pacientes com MG (forma ocular 62, generalizada 246 e timomatosa 16 as seguintes variáveis da prova de função pulmonar (PFP: capacidade vital forçada (FVC; volume onde o fluxo expiratório é igual a 1 litro por segundo (VF=1; volume expiratório forçado no primeiro segundo (FEV1; fluxo expiratório forçado medido entre 0,2 e 1,2 litros (FEF; fluxo médio expiratório forçado, medido entre 25 e 75% da FVC (FMF; intervalo de tempo entre 25 e 75% da FVC (FMFT; tempo médio de trânsito na expiração forçada (MTT; capacidade pulmonar total (TLC; volume residual (RV; curva fluxo-volume para pesquisa do "padrão miastênico". A análise estatística realizada foi: "t pareado" entre paciente e seu padrão e "t não pareado" entre grupos. Conclusões: Todos os

9 CFR 319.313 - Beef with gravy and gravy with beef.

Science.gov (United States)

2010-01-01

... 9 Animals and Animal Products 2 2010-01-01 2010-01-01 false Beef with gravy and gravy with beef. 319.313 Section 319.313 Animals and Animal Products FOOD SAFETY AND INSPECTION SERVICE, DEPARTMENT OF... Dehydrated Meat Food Products § 319.313 Beef with gravy and gravy with beef. “Beef with Gravy” and “Gravy...

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João Aris Kouyoumdjian

2013-04-01

Full Text Available Objective:To estimate jitter parameters in myasthenia gravis in stimulated frontalis and extensor digitorum muscles using the concentric needle electrode.Methods:Forty-two confirmed myasthenia gravis patients, being 22 males (aged 45.6±17.2 years-old were studied. Jitter was expressed as the mean consecutive difference (MCD. Results: MCD in extensor digitorum was 61.6 µs (abnormal in 85.7% and in frontalis 57.3 µs (abnormal in 88.1%. Outliers represented 90.5% for extensor digitorum and 88.1% for frontalis. At least one jitter parameter was abnormal in 90.5% of the combined studies. Acetylcholine receptor antibody was abnormal in 85.7% of the cases. Conclusions:Stimulated jitter recordings measured from muscles using concentric needle electrode can be used for myasthenia gravis diagnosis with high sensitivity. Extensive normative studies are still lacking and, therefore, borderline findings should be judged with great caution.Objetivo:Mensurar os valores do jitter em pacientes com miastenia gravis nos músculos frontalis e extensor digitorum pela técnica estimulada, utilizando-se eletrodo de agulha concêntrica.Métodos:Foram estudados 42 pacientes, sendo 22 homens (idade 45,6±17,2 anos, com miastenia gravis confirmada. O jitter foi expresso como a média das diferenças consecutivas (MDC.Resultados: A MDC para o extensor digitorum foi 61,6 µs (anormal em 85,7% e para o frontalis 57,3 µs (anormal em 88,1%. Outliers representaram 90,5% para o extensor digitorum e 88,1% para o frontalis. Pelo menos um parâmetro do jitter foi anormal em 90,5% dos estudos combinados. Anticorpo receptor de acetilcolina estava anormal em 85,7% dos casos. Conclusões:Jitter estimulado mensurado por meio de eletrodo de agulha concêntrica pode ser utilizado para diagnóstico de miastenia gravis com elevada sensibilidade. Estudos normativos mais amplos ainda são necessários e, portanto, valores limítrofes devem ser avaliados com cautela.

Myasthenia gravis - resources

Science.gov (United States)

Resources - myasthenia gravis ... The following organizations provide information on myasthenia gravis : Myasthenia Gravis Foundation of America -- www.myasthenia.org National Institute of Neurological Disorders and Stroke -- www.ninds.nih.gov/Disorders/Patient- ...

Timoma do mediastino médio: relato de caso Middle mediastinal thymoma: case report

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Elias Kallás

2005-06-01

Full Text Available Paciente do sexo feminino, 55 anos, branca, com sinais clínicos sugestivos de miastenia gravis há aproximadamente 3 meses. A prova terapêutica com neostigmina evidenciou melhora da disfagia e ptose palpebral. Na radiografia de tórax observou-se imagem ocupando o mediastino médio com projeção à direita. A tomografia computadorizada de tórax revelou a presença de massa no mediastino médio, sendo indicado o tratamento cirúrgico. Chamou nossa atenção a localização pouco usual, já que, preferencialmente, os timomas localizam-se no mediastino superior e anterior. A evolução pós-operatória foi boa, sem complicações.We report on a case of a 55-year-old female, patient, who presented with clinical signs suggestive of myasthenia gravis over a period of approximately 3 months. The therapeutic option using neostigmine gave an improvement of the dysphagia and palpebral ptosis. A chest radiograph image demonstrated a mass occupying the medium mediastinum with projection to the right. Computed tomography of the thorax revealed the presence of a mass in the medium mediastinum, and thus surgery was inicated. During the operation the was was observed in the medium mediastinum suggesting thymoma. What caught our attention was the unusual location of the tumor as normally thymomas are found in the upper and anterior mediastinum.

Myasthenia Gravis

Science.gov (United States)

Myasthenia gravis is a disease that causes weakness in your voluntary muscles. These are the muscles that ... gets worse with activity, and better with rest. Myasthenia gravis is an autoimmune disease. Your body's immune ...

Psychometric Properties of the Quantitative Myasthenia Gravis Score and the Myasthenia Gravis Composite Scale.

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Barnett, Carolina; Merkies, Ingemar S J; Katzberg, Hans; Bril, Vera

2015-09-02

The Quantitative Myasthenia Gravis Score and the Myasthenia Gravis Composite are two commonly used outcome measures in Myasthenia Gravis. So far, their measurement properties have not been compared, so we aimed to study their psychometric properties using the Rasch model. 251 patients with stable myasthenia gravis were assessed with both scales, and 211 patients returned for a second assessment. We studied fit to the Rasch model at the first visit, and compared item fit, thresholds, differential item functioning, local dependence, person separation index, and tests for unidimensionality. We also assessed test-retest reliability and estimated the Minimal Detectable Change. Neither scale fit the Rasch model (X2p Myasthenia Gravis Composite had lower discrimination properties than the Quantitative Myasthenia Gravis Scale (Person Separation Index: 0.14 and 0.7). There was local dependence in both scales, as well as differential item functioning for ocular and generalized disease. Disordered thresholds were found in 6(60%) items of the Myasthenia Gravis Composite and in 4(31%) of the Quantitative Myasthenia Gravis Score. Both tools had adequate test-retest reliability (ICCs >0.8). The minimally detectable change was 4.9 points for the Myasthenia Gravis Composite and 4.3 points for the Quantitative Myasthenia Gravis Score. Neither scale fulfilled Rasch model expectations. The Quantitative Myasthenia Gravis Score has higher discrimination than the Myasthenia Gravis Composite. Both tools have items with disordered thresholds, differential item functioning and local dependency. There was evidence of multidimensionality in the QMGS. The minimal detectable change values are higher than previous studies on the minimal significant change. These findings might inform future modifications of these tools.

Farmacologia da unidade motora: aspectos de interesse em neurologia Pharmacology of motor end-plate: aspects related to neurology

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Paulina Sannomiya

1975-09-01

Full Text Available Os AA fazem revisão sobre a unidade motora em seu aspecto morfológico, fisiológico e farmacológico como base para o estudo e tratamento da miastenia grave e da síndrome miastênica. Estudos eletromiográficos, farmacológicos e eletrofisiológicos, realizados por diversos autores, determinam características especiais que definem a miastenia grave e a síndrome miastênica. Certos sinais e sintomas, comumente observados em casos de envenenamento por elapídeos, são semelhantes aos causados por drogas bloqueadoras neuromusculares.In order to understand the treatment of miasthenia gravis and miasthenic sindrome, the morphology, physiology and pharmacology of the motor unit were reviewed. Electromiographic, pharmacologic and electrophysiologic studies accomplished by several authors have special caractheristics which define miasthenia gravis and miasthenic sindrome. Certain venoms such as those from Elapidae can produce signals and symptoms resembling those produced by neuromuscular blocking drugs.

Anàlisi del títol d'anticossos anti-achr i la seva correlació clínica en pacients amb miastenia gravis seropositiva

OpenAIRE

Ramos Fransi, Alba

2013-01-01

Els anticossos contra el receptor de l'acetilcolina representen el principal mecanisme patogènic en la Miastènia Gravis, però la seva utilitat com a biomarcador durant el seguiment de la malaltia és desconeguda.

Genetics Home Reference: myasthenia gravis

Science.gov (United States)

... increase the risk of myasthenia gravis , but the identity of these genes is unknown. Many factors likely ... Myasthenia Gravis Fact Sheet MalaCards: myasthenia gravis Merck Manual Home Health Handbook for Patients and Caregivers Myasthenia ...

Tratamento das formas severas de miastenia pelo ACTH por via intravenosa

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José Lamartine de Assis

1960-12-01

Full Text Available O autor inicia o trabalho referindo as bases bioquímicas, fisiopatológicas e anátomo-patológicas do tratamento da miastenia pelo ACTH. Na miastenia grave há diminuição da síntese da acetilcolina no organismo, atuando o ACTH no sentido de aumentar esta síntese seja diretamente, por ativação da colinacetilase, seja indiretamente, mediante a redução da massa dos tecidos linfóides, em particular do timo, responsáveis pela elaboração de substâncias que diminuem a síntese da acetilcolina. O autor empregou o ACTH "Armour" e a Cortrofina "Organon", nas doses de 2,5 a 25 mg, sempre pela via intravenosa, diluídos em 250 a 1.000 ml de soluto glicosado a 5%, administrado gota a gôta, na velocidade média de 20 gôtas por minuto, durante 8 horas. Como medicação associada foi administrada a Prostigmina a todos os pacientes, substituída, depois, em alguns casos, pelo Mestinon ou pela Mytelaze. Como adjuvantes foram empregados o cloreto de potássio (2 a 8 g por dia e o sulfato de efedrina (25 mg 3 vêzes ao dia. Os pacientes foram mantidos em regime hiperprotéico e acloretado, sendo tomados todos os cuidados inerentes ao uso do ACTH. Foram estudados 10 pacientes portadores de miastenia com sintomatologia acentuada (8 casos e média (2 casos. Todos os doentes vinham sendo tratados com drogas anticolinesterásicas em doses adequadas (Prostigmina, Mestinon, Mytelaze e a sua sintomatologia respondia cada vez menos a esta terapêutica. Em alguns casos haviam sido tentados outros tratamentos (timectomia, denervação do seio carotídeo, irradiação da região tímica sem resultado. É de notar que as remissões espontâneas neste grupo de enfermos foram excepcionais e de curta duração. A evolução foi acompanhada do ponto de vista clínico, com a sintomatologia classificada como muito acentuada, acentuada, média e leve. Em todos os casos houve remissão completa ou quase completa da sintomatologia após dosagens variáveis de ACTH; no

Gallium scans in myasthenia gravis

International Nuclear Information System (INIS)

Swick, H.M.; Preston, D.F.; McQuillen, M.P.

1976-01-01

A study was conducted to determine whether 67 Ga scans could be used for the detection of thymomas and to investigate the activity of the thymus gland in patients with myasthenia gravis. Scans of the anterior mediastinum proved to be a reliable way to detect thymomas. The scans were positive in eight patients including three with myasthenia gravis and histologically proved thymomas, three others with severe myasthenia gravis and thymic tumors, and two with histologically proved thymomas not associated with myasthenia. Activity on 67 Ga scans was not directly related to the increased activity of the thymus gland that is presumed to be associated with myasthenia gravis

Gallium scans in myasthenia gravis

Energy Technology Data Exchange (ETDEWEB)

Swick, H.M. (Univ. of Kentucky, Lexington); Preston, D.F.; McQuillen, M.P.

1976-01-01

A study was conducted to determine whether /sup 67/Ga scans could be used for the detection of thymomas and to investigate the activity of the thymus gland in patients with myasthenia gravis. Scans of the anterior mediastinum proved to be a reliable way to detect thymomas. The scans were positive in eight patients including three with myasthenia gravis and histologically proved thymomas, three others with severe myasthenia gravis and thymic tumors, and two with histologically proved thymomas not associated with myasthenia. Activity on /sup 67/Ga scans was not directly related to the increased activity of the thymus gland that is presumed to be associated with myasthenia gravis. (HLW)

Isolated laryngeal myasthenia gravis for 26 years.

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Renard, Dimitri; Hedayat, Amir; Gagnard, Corinne

2015-02-01

Laryngeal myasthenia gravis is a relatively rare variant of myasthenia gravis. A vast portion of patients with initial laryngeal myasthenia gravis develop involvement of ocular and/or extra-ocular muscles during the years after symptom onset although a minority of laryngeal myasthenia gravis patients continues to have isolated laryngeal muscle involvement for several years. We present a 58-year-old woman with recurrent episodic isolated dysphonia (associated with diffuse bilateral vocal cord paresis on laryngoscopy) since the age of 32. Dysphonia became permanent since 6 months. A diagnosis of laryngeal myasthenia gravis was made based on abnormal single-fiber electromyography and spectacular response to pyridostigmine treatment. Repetitive nerve stimulation was normal and anti-acetylcholine receptor and anti-muscle specific tyrosine kinase antibodies were absent. This case shows that laryngeal myasthenia gravis can be isolated during 26 years of follow-up. We propose that even when myasthenia gravis seems unlikely as underlying mechanism of isolated dysphonia (because of lack of antibodies, normal repetitive nerve stimulation, and absence of extra-laryngeal involvement after years of follow-up), single-fiber electromyography should be performed and myasthenia gravis treatment should be tried. Copyright © 2014 Elsevier B.V. All rights reserved.

Clinical predictors for the prognosis of myasthenia gravis.

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Wang, Lili; Zhang, Yun; He, Maolin

2017-04-19

Clinical predictors for myasthenia gravis relapse and ocular myasthenia gravis secondary generalization during the first two years after disease onset remain incompletely identified. This study attempts to investigate the clinical predictors for the prognosis of Myasthenia Gravis. Eighty three patients with myasthenia gravis were concluded in this study. Baseline characteristics were analyzed as predictors. Relapse of myasthenia gravis developed in 26 patients (34%). Generalization developed in 34 ocular myasthenia gravis patients (85%). Other autoimmune diseases were observed more commonly in relapsed myasthenia gravis (P = 0.012). Second generalization group contained more late onset patients (P = 0.021). Ocular myasthenia gravis patients with thymus hyperplasia progressed more rapidly than those with other thymus pathology (P = 0.027). Single onset symptom of ocular myasthenia gravis such as ptosis or diplopia predicted early progression than concurrence of ptosis and diplopia (P = 0.027). Treatment effect including glucocorticoid, pyridostigmine, thymectomy, IVIG, immunosuppressive drugs did not show significant difference between the relapsed and non-relapsed groups. The treatment outcome also showed no difference between the single OMG and second generalized groups. Occurrence of associated autoimmune disease can serve as a potential predictor for myasthenia gravis relapse. Either ptosis or diplopia, as well as thymic hyperplasia can predict generalization in the first six months.

Treatment for Myasthenia Gravis (MG)

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... are individualized There are many effective treatments for myasthenia gravis. Spontaneous improvement and even remission (although uncommon) ... Created by Kellen Interactive Web Design © copyright 2010 Myasthenia Gravis Foundation of America, Inc.

Masticatory performance in patients with myasthenia gravis

NARCIS (Netherlands)

Weijnen, FG; Kuks, JBM; van der Glas, HW; Oudenaarde, [No Value; Bosman, F

Masticatory muscle electromyograms (EMGs) were recorded while patients with bulbar myasthenia gravis chewed artificial food and compared with those of patients with ocular myasthenia gravis, patients in clinical remission who had previously suffered from bulbar myasthenia gravis and healthy

Diagnosis and management of myasthenia gravis.

Science.gov (United States)

Barber, Christopher

2017-06-21

Myasthenia gravis is a rare long-term neurological condition that is characterised by fluctuating skeletal muscle weakness and fatigue, as well as respiratory difficulties. It is both an acquired autoimmune disease and a chronic neuromuscular disorder. Because of its rarity, myasthenia gravis is relatively unknown and may be unfamiliar to many nurses. While there are various types of myasthenia, this article focuses on myasthenia gravis, exploring its symptoms, diagnosis and treatment, and examining the nurse's role in managing the condition. The symptoms of myasthenic crisis and cholinergic crisis are also explained, and the experience of patients with myasthenia gravis in hospital and community settings is illustrated using case studies.

Tongue force in patients with myasthenia gravis

NARCIS (Netherlands)

Weijnen, FG; Kuks, JBM; van der Glas, HW; Wassenberg, MWM; Bosman, F

2000-01-01

Objectives - The aim was to study tongue force in patients with bulbar myasthenia gravis and compare it with that of patients with ocular myasthenia gravis, patients in clinical remission who previously suffered from bulbar myasthenia gravis, and healthy subjects. Material and methods - Tongue force

A lesão muscular na miastenia grave: estudo de 17 casos com histoquimica muscular

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Lineu Cesar Werneck

1982-03-01

Full Text Available Estudo de 17 biópsias musculares de pacientes com miastenia grave, utilizando técnicas de coloração a fresco e histoquímica muscular. Foram encontradas 15 biópsias musculares anormais, sendo que as principais alterações foram fibras musculares angulares escuras atróficas, excesso de gotículas de gordura na membrana externa das fibras, variação no diâmetro das fibras e atrofia de fibras do tipo II. Os achados foram interpretados como denervação em 11 biópsias, atrofia de fibras do tipo II em 7, infiltrado linfocitário em 4, necrose de fibras musculares com fagocitose em 1 e em 2 biópsias não foi encontrada qualquer anormalidade. Quanto maior o tempo de doença, mais severa foi a anormalidade encontrada. Dois pacientes apresentavam timoma, um miastenia grave congênita, um artrite reumatoide, um neurite hipertrófica intersticial, um tireoidite de Hashimoto e um com síndrome miastênica concomitante. São discutidos os achados anatomopatológicos e sua possível explicação.

Myasthenia Gravis Medication Information Card (Drugs to be Avoided or Used with Caution in Myasthenia Gravis)

Science.gov (United States)

MYASTHENIA GRAVIS MEDICATION INFORMATION CARD Drugs to be Avoided or Used with Caution in MG My Name _______________________________________________ ... the MGFA web site; reference document “Medications and Myasthenia Gravis (A Reference for Health Care Professionals.” www. ...

Síntomas de miastenia grave en un paciente con antecedente de timectomía por timoma invasor

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Lilliana María Giraldo

2015-12-01

Conclusiones. Los pacientes con miastenia grave y aquellos con timoma asociado, deben someterse a la timectomía como parte del tratamiento. Sin embargo, la exacerbación de los síntomas o su reaparición después del procedimiento no necesariamente implica una nueva alteración en el timo.

Myasthenia Gravis and Its Aeromedical Implications.

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Jagathesan, Tania; O'Brien, Michael D

2017-01-01

Myasthenia gravis is an autoimmune condition where antibodies form against the acetylcholine receptors at the neuromuscular junction, eventually causing damage to the motor end plate. The clinical features include muscle fatigability as well as ocular, bulbar, and limb weakness, which can have implications on the role of a pilot or air traffic controller. This retrospective study reviewed the United Kingdom Civil Aviation Authority (UK CAA) experience of myasthenia gravis. A search of the United Kingdom Civil Aviation Authority medical records database from 1990 to 2016 identified 11 individuals with a diagnosis of myasthenia gravis. Data were extracted for the class of medical certificate, age at diagnosis, symptoms, acetylcholine receptor antibody status, treatment, the time from diagnosis to loss of medical certification, and the reasons for loss of certification. There were two Class 1 certificate holders (for professional flying) and six Class 2 certificate holders (for private pilot flying) and three air traffic controllers. The mean and median ages at diagnosis were 53 and 57 yr, respectively, with a range of 28-67 yr. The mean and median intervals from diagnosis to loss of certification were 22 and 11 mo, respectively, with a range of 0 to 108 mo. The aeromedical implications of myasthenia gravis, including complications, types of treatment, and functional impact, are considered. A policy for medical certification following a diagnosis of myasthenia gravis is proposed.Jagathesan T, O'Brien MD. Myasthenia gravis and its aeromedical implications. Aerosp Med Hum Perform. 2017; 88(1):30-33.

Ocular myasthenia gravis: Side effect of urografin

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Nitin Modi

2016-01-01

Full Text Available Ocular myasthenia gravis is an autoimmune disorder of the neuromuscular junction. Diplopia and ptosis are common symptoms at the onset of ocular myasthenia gravis. It may occur due to the antibodies developed against various drugs. We are reporting a case of ocular myasthenia gravis which was developed in a patient post angiography which may be due to antibody developed against the dye used in angiography.

SPECIFIC FEATURES OF ANESTHESIA IN PATIENTS WITH MYASTHENIA GRAVIS.

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Spasojevic, Ivana; Hajdukovic, Danica; Komarcevic, Milena; Petrovic, Stanislava; Jovanovic, Jelena; Ciric, Aleksandra

2016-09-01

Myasthenia gravis is an autoimmune disease caused by antibodies leading to the destruction of nicotinic acetylcholine receptors on the neuromuscular junction. It is characterized by muscle weakness that gets aggravated with physical activity and improves at rest. Myasthenia Gravis Foundation of America made the clinical classification of Myasthenia gravis which is still in use today. "Tensilon test" is still the gold standard for the diagnosis of Myasthenia gravis. In addition to this test repeated muscular stimulation can be used as well as the analysis of specific autoantibodies. Treatment of Myasthenia Gravis. In conservative treatment of Mysthenia gravis anticholinesterases, immunosuppressants and plasmapheresis can be used. If conservative treatment does not lead to the desired remission, surgical treatment is indicated. The most accepted indication for thymectomy is the presence of thymoma with generalized form of Myasthenia gravis in adults. How to Distinguish Myasthenic From Cholinergic Crisis.'The following is important to make a difference between these two crises: knowledge of the events that preceded the crisis, the size of pupils as well as the presence of muscarinic signs and tensilon test. Specific Features of Anesthesia in Patients with Myasthienia Gravis. Mechanism of the disease development is the reason'for the increased sensitivity or resistance of these patients to certain types of drugs used in anesthesia. Protocol of Perioperative Anesthesia in Patients with Myasthenia Gravis. Based on 35 years of experience in the surgical treatment of patients with Myasthenia gravis anesthesiologists at the Department of Thoracic Surgery, Institute for Pulmonary Diseases of Vojvodina. made the protocol of anesthesia and perioperative treatment for these patients. Anesthesiologists may have to deal with a patient with myasthenia gravis in different types of surgical interventions. The protocol for anesthesia and perioperative management of these patients

Estudo dos linfócitos circulantes por anticorpos monoclonais na miastenia grave

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Paulo E. Marchiori

1988-09-01

Full Text Available Os autores avaliam os linfócitos T (CD3, CD4, CD8, CD4/8 por anticorpos monoclonais e rosácea em 20 pacientes e linfócitos B por Fab' por imunofluorescência em 9 pacientes com miastenia grave. Observam elevação significante na população de linfócito B e redução nos linfócitos T totais CD3+ por rosáceas. Não foram observadas modificações nas subpopulações celulares com timectomia e corticosteróides.

Neuropsychological performance in patients with myasthenia gravis

OpenAIRE

María Bárbara Eizaguirre; Florencia Aguirre; Cecilia Yastremiz; Sandra Vanotti; Andrés Villa

2017-01-01

Myasthenia gravis is a chronic autoimmune disease that affects the neuromuscular transmission. Controversial findings had been reported about cognitive impairment in this disease. The aim of this study was to investigate the cognitive pattern of patients with myasthenia gravis. There were enrolled 24 patients with myasthenia gravis, anti-acetylcholine receptor antibodies (ACRA) positive, and 24 healthy controls. Patients: age 43.9 ± 14.8, years of education 10.9 ± 3.3. Controls: age 44.5 ± 15...

Myasthenia Gravis

Science.gov (United States)

... that medications can be discontinued. In a few cases, the severe weakness of myasthenia gravis may cause respiratory failure, which requires immediate emergency medical care. Clinical Trials Throughout the U.S. and Worldwide NINDS Clinical ...

Myasthenia gravis: subgroup classification and therapeutic strategies.

Science.gov (United States)

Gilhus, Nils Erik; Verschuuren, Jan J

2015-10-01

Myasthenia gravis is an autoimmune disease that is characterised by muscle weakness and fatigue, is B-cell mediated, and is associated with antibodies directed against the acetylcholine receptor, muscle-specific kinase (MUSK), lipoprotein-related protein 4 (LRP4), or agrin in the postsynaptic membrane at the neuromuscular junction. Patients with myasthenia gravis should be classified into subgroups to help with therapeutic decisions and prognosis. Subgroups based on serum antibodies and clinical features include early-onset, late-onset, thymoma, MUSK, LRP4, antibody-negative, and ocular forms of myasthenia gravis. Agrin-associated myasthenia gravis might emerge as a new entity. The prognosis is good with optimum symptomatic, immunosuppressive, and supportive treatment. Pyridostigmine is the preferred symptomatic treatment, and for patients who do not adequately respond to symptomatic therapy, corticosteroids, azathioprine, and thymectomy are first-line immunosuppressive treatments. Additional immunomodulatory drugs are emerging, but therapeutic decisions are hampered by the scarcity of controlled studies. Long-term drug treatment is essential for most patients and must be tailored to the particular form of myasthenia gravis. Copyright © 2015 Elsevier Ltd. All rights reserved.

Determinants of quality of life in Brazilian patients with myasthenia gravis.

Science.gov (United States)

Mourão, Aline Mansueto; Gomez, Rodrigo Santiago; Barbosa, Luiz Sergio Mageste; Freitas, Denise da Silva; Comini-Frota, Elizabeth Regina; Kummer, Arthur; Lemos, Stella Maris Aguiar; Teixeira, Antonio Lucio

2016-07-01

The aims of the current study were 1) to evaluate the reliability and validity of the Brazilian version of the 15-item Myasthenia Gravis Quality of Life Scale and 2) to investigate the quality of life of Brazilian patients with myasthenia gravis and its determinants. This cross-sectional study included 69 patients with myasthenia gravis who underwent neurological evaluation and completed questionnaires regarding quality of life (the 36-item Short Form of the Medical Outcomes Study and the 15-item Myasthenia Gravis Quality of Life Scale), anxiety and depressive symptoms. The Brazilian version of the 15-item Myasthenia Gravis Quality of Life Scale showed high internal consistency and good concurrent validity with the 36-item Short Form of the Medical Outcomes Study and its subscales. Determinants of quality of life in Brazilian patients with myasthenia gravis included the current status of myasthenia gravis as assessed by the Myasthenia Gravis Composite, the current prednisone dose and the levels of anxiety and depression. The Brazilian version of the 15-item Myasthenia Gravis Quality of Life Scale is a valid instrument. Symptom severity, prednisone dosage and anxiety and depression levels impact the quality of life of patients with myasthenia gravis.

Randomized Trial of Thymectomy in Myasthenia Gravis.

Science.gov (United States)

Wolfe, Gil I; Kaminski, Henry J; Aban, Inmaculada B; Minisman, Greg; Kuo, Hui-Chien; Marx, Alexander; Ströbel, Philipp; Mazia, Claudio; Oger, Joel; Cea, J Gabriel; Heckmann, Jeannine M; Evoli, Amelia; Nix, Wilfred; Ciafaloni, Emma; Antonini, Giovanni; Witoonpanich, Rawiphan; King, John O; Beydoun, Said R; Chalk, Colin H; Barboi, Alexandru C; Amato, Anthony A; Shaibani, Aziz I; Katirji, Bashar; Lecky, Bryan R F; Buckley, Camilla; Vincent, Angela; Dias-Tosta, Elza; Yoshikawa, Hiroaki; Waddington-Cruz, Márcia; Pulley, Michael T; Rivner, Michael H; Kostera-Pruszczyk, Anna; Pascuzzi, Robert M; Jackson, Carlayne E; Garcia Ramos, Guillermo S; Verschuuren, Jan J G M; Massey, Janice M; Kissel, John T; Werneck, Lineu C; Benatar, Michael; Barohn, Richard J; Tandan, Rup; Mozaffar, Tahseen; Conwit, Robin; Odenkirchen, Joanne; Sonett, Joshua R; Jaretzki, Alfred; Newsom-Davis, John; Cutter, Gary R

2016-08-11

Thymectomy has been a mainstay in the treatment of myasthenia gravis, but there is no conclusive evidence of its benefit. We conducted a multicenter, randomized trial comparing thymectomy plus prednisone with prednisone alone. We compared extended transsternal thymectomy plus alternate-day prednisone with alternate-day prednisone alone. Patients 18 to 65 years of age who had generalized nonthymomatous myasthenia gravis with a disease duration of less than 5 years were included if they had Myasthenia Gravis Foundation of America clinical class II to IV disease (on a scale from I to V, with higher classes indicating more severe disease) and elevated circulating concentrations of acetylcholine-receptor antibody. The primary outcomes were the time-weighted average Quantitative Myasthenia Gravis score (on a scale from 0 to 39, with higher scores indicating more severe disease) over a 3-year period, as assessed by means of blinded rating, and the time-weighted average required dose of prednisone over a 3-year period. A total of 126 patients underwent randomization between 2006 and 2012 at 36 sites. Patients who underwent thymectomy had a lower time-weighted average Quantitative Myasthenia Gravis score over a 3-year period than those who received prednisone alone (6.15 vs. 8.99, Pmyasthenia gravis. (Funded by the National Institute of Neurological Disorders and Stroke and others; MGTX ClinicalTrials.gov number, NCT00294658.).

Multiple Thymoma with Myasthenia Gravis

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Dong Hyun Seo

2017-02-01

Full Text Available The actual incidence of multiple thymoma is unknown and rarely reported because it remains controversial whether the cases represent a disease of multicentric origin or a disease resulting from intrathymic metastasis. In this case, a patient underwent total thymectomy for multiple thymoma with myasthenia gravis via bilateral video-assisted thoracic surgery. A well-encapsulated multinodular cystic mass, measuring 57 mm×50 mm×22 mm in the right lobe of the thymus, and a well-encapsulated mass, measuring 32 mm×15 mm×14 mm in the left lobe, were found. Both tumors were type B2 thymoma. Few cases of multiple thymoma with myasthenia gravis have ever been reported in the literature. We report a case of synchronous multiple thymoma associated with myasthenia gravis.

Myasthenia Gravis

Science.gov (United States)

... Institutes of Health (NIH), the leading supporter of biomedical research in the world. Although there is no cure ... understand the fundamental processes in neuromuscular development. This research could reveal new therapies for neuromuscular diseases like myasthenia gravis. Researchers are ...

Assays for myasthenia gravis

International Nuclear Information System (INIS)

Lindstrom, J.M.

1988-01-01

This patent describes an improvement in a process for diagnosing myasthenia gravis. The process comprises the steps of preparing a complex of acetycholine receptor protein, toxin and a radioactive isotope, incubating the complex with a serum sample from a patient so as to join antibodies engendered in connection with myasthenia gravis to the complex, precipitating the complex joined with antibody with anti-immunoglobulin and measuring radioactivity, from the radioactive isotope, of the precipitated complex. The improvement is that the acetylcholine receptor protein is derived from cells of the TE671 Line

Assays for myasthenia gravis

Energy Technology Data Exchange (ETDEWEB)

Lindstrom, J.M.

1988-12-06

This patent describes an improvement in a process for diagnosing myasthenia gravis. The process comprises the steps of preparing a complex of acetycholine receptor protein, toxin and a radioactive isotope, incubating the complex with a serum sample from a patient so as to join antibodies engendered in connection with myasthenia gravis to the complex, precipitating the complex joined with antibody with anti-immunoglobulin and measuring radioactivity, from the radioactive isotope, of the precipitated complex. The improvement is that the acetylcholine receptor protein is derived from cells of the TE671 Line.

Properties of gravi-inertial systems of reference

International Nuclear Information System (INIS)

Dozmorov, I.M.

1977-01-01

A number of papers of the author have been summarized devoted to gravi-inertial systems of reference in which the following problems are solved: a) analogs of inertial systems of reference (ISR), gravi-ISR, have been introduced into the general relativity the ory (GRT); b) using transformations between such ISR as symmetry transformation, obtained by variational methods are values with clear physical sense; c) using the gravi-ISR basis as the zero level of the deformation reading, the theory of elasticity in GRT has been constructed and someof its applications considered. The results are compared with those of other authors

Ocular myasthenia gravis in a setting of thyrotoxicosis

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Sarabjeet Chhabra

2013-01-01

Full Text Available Ocular myasthenia gravis in conjunction with thyroid disorders, although rare, has been reported in the past. However, the similarity in the presentation of both the entities and the tendency of myasthenia gravis to get overlooked easily, even by experienced clinicians, necessitates a thorough knowledge, a strong consideration, and a vigilant approach, to aid in its diagnosis. We discuss a case of a female in a thyrotoxic state, with symptoms of ocular myasthenia gravis, and a brief overview of this entity.

Reversible man-in-the-barrel syndrome in myasthenia gravis

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Poornima A Shah

2016-01-01

Full Text Available Man-in-the-barrel syndrome (MBS is an uncommon presentation due to bilateral, predominantly proximal muscle weakness that has not been described to be associated with myasthenia gravis. We describe a case of myasthenia gravis presenting as MBS. Additionally, he had significant wasting of the deltoids bilaterally with fibrillations on electromyography (EMG at rest and brief duration (3-6 ms bi/triphasic motor unit potentials (MUPs on submaximal effort apart from a decremental response on repetitive nerve stimulation (RNS at 2 Hz. While electrophysiology is an important tool in the diagnosis of myasthenia gravis, pathological EMG patterns do not exclude the diagnosis of myasthenia gravis.

Reversible man-in-the-barrel syndrome in myasthenia gravis

Science.gov (United States)

Shah, Poornima A; Wadia, Pettarusp Murzban

2016-01-01

Man-in-the-barrel syndrome (MBS) is an uncommon presentation due to bilateral, predominantly proximal muscle weakness that has not been described to be associated with myasthenia gravis. We describe a case of myasthenia gravis presenting as MBS. Additionally, he had significant wasting of the deltoids bilaterally with fibrillations on electromyography (EMG) at rest and brief duration (3-6 ms) bi/triphasic motor unit potentials (MUPs) on submaximal effort apart from a decremental response on repetitive nerve stimulation (RNS) at 2 Hz. While electrophysiology is an important tool in the diagnosis of myasthenia gravis, pathological EMG patterns do not exclude the diagnosis of myasthenia gravis. PMID:27011638

RARITY OF MYASTHENIA GRAVIS IN NORTHERN NIGERIANS ...

African Journals Online (AJOL)

This is a 10 year retrospective review of patients managed at the Ahmadu Bello Teaching hospital, Zaria, Nigeria for myasthenia gravis. Only 4 patients were identified from the hospitals records. Myasthenia gravis appears uncommon in this environment. Reasons for the apparent rarity of this condition and other ...

Transient neonatal myasthenia gravis due to a mother with ocular onset of anti-muscle specific kinase myasthenia gravis.

Science.gov (United States)

Lee, Ju-Yeun; Min, Ju-Hong; Han, Sueng-Han; Han, Jinu

2017-07-01

We describe a 27-year-old pregnant female with new onset of conjugate gaze deficit during the third trimester of pregnancy. Repetitive nerve stimulation tests, neostigmine tests, and acetylcholine receptor antibody assays were all negative. The patient delivered a normal healthy baby at a local clinic via cesarean section. The baby became hypotonic and had respiratory failure several minutes after birth. The result of acetylcholine receptor antibody was negative in the neonate. The neonate became healthy spontaneously and was extubated after 21 days of ventilation care. Two months after delivery, the mother developed ptosis and generalized symptoms and subsequent workup revealed she was muscle specific kinase (MuSK) antibody positive. The neonate was presumed to have an anti-MuSK-mediated transient neonatal myasthenia gravis. Although MuSK antibody testing is rarely indicated in ocular myasthenia gravis, MuSK antibody testing is necessary in pregnant women who are presumed ocular myasthenia gravis to warn occurrence of transient neonatal myasthenia gravis. Copyright © 2017 Elsevier B.V. All rights reserved.

Myopathy in Childhood Muscle-Specific Kinase Myasthenia Gravis.

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Kirzinger, Lukas; Khomenko, Andrei; Schulte-Mattler, Wilhelm; Backhaus, Roland; Platen, Sabine; Schalke, Berthold

2016-12-01

Adult and pediatric patients suffering from MuSK (muscle-specific kinase) -antibody positive myasthenia gravis exhibit similar features to individuals with acetylcholine receptor (AChR) antibodies, but they differ in several characteristics such as a predominant bulbar, respiratory and neck weakness, a generally worse disease severity and a tendency to develop muscle atrophy. Muscle atrophy is a rare phenomenon that is usually restricted to the facial muscles. We describe a girl with MuSK-antibody positive myasthenia gravis who developed a myopathy with severe generalized muscular weakness, muscle atrophy, and myopathic changes on electromyography. This is the first published example of a generalized myopathic syndrome in myasthenia gravis. We review the relevant literature and discuss the hypothesis of a mitochondrial myopathy as a pathogenic mechanism in MuSK-antibody positive myasthenia gravis. Copyright © 2016 Elsevier Inc. All rights reserved.

Muscle-Specific Tyrosine Kinase and Myasthenia Gravis Owing to Other Antibodies.

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Rivner, Michael H; Pasnoor, Mamatha; Dimachkie, Mazen M; Barohn, Richard J; Mei, Lin

2018-05-01

Around 20% of patients with myasthenia gravis are acetylcholine receptor antibody negative; muscle-specific tyrosine kinase antibodies (MuSK) were identified as the cause of myasthenia gravis in 30% to 40% of these cases. Anti MuSK myasthenia gravis is associated with specific clinical phenotypes. One is a bulbar form with fewer ocular symptoms. Others show an isolated head drop or symptoms indistinguishable from acetylcholine receptor-positive myasthenia gravis. These patients usually respond well to immunosuppressive therapy, but not as well to cholinesterase inhibitors. Other antibodies associated with myasthenia gravis, including low-density lipoprotein receptor-related protein 4, are discussed. Copyright © 2018 Elsevier Inc. All rights reserved.

Maximal bite force and surface EMG in patients with myasthenia gravis

NARCIS (Netherlands)

Weijnen, FG; Wokke, JHJ; Kuks, JBM; van der Glas, HW; Bosman, F

2000-01-01

Masticatory muscle strength was quantified in patients with bulbar myasthenia gravis and compared with that of patients with ocular myasthenia gravis, patients in clinical remission (whether or not pharmacological) who previously suffered from bulbar myasthenia gravis, and healthy subjects. Maximal

Seronegative myasthenia gravis associated with malignant thymoma.

Science.gov (United States)

Richards, Jason; Howard, James F

2017-05-01

Myasthenia gravis (MG) is generally caused by antibodies directed against the neuromuscular junction, including antibodies against the postsynaptic nicotinic acetylcholine receptor (AChR). Pathologic abnormalities of the thymus gland, including thymoma, are associated with MG. We report a 56-year-old woman who presented with double vision. Single fiber EMG confirmed myasthenia gravis. AChR, striational muscle and MuSK antibodies were absent in the serum. Chest CT demonstrated a malignant thymoma. We report the first case of seronegative myasthenia gravis associated with malignant thymoma. The case challenges the conventional wisdom that all patients with thymoma associated MG test positive for antibodies against AChR. Copyright © 2017 Elsevier B.V. All rights reserved.

Immune-mediated rippling muscle disease and myasthenia gravis.

Science.gov (United States)

Bettini, Mariela; Gonorazky, Hernan; Chaves, Marcelo; Fulgenzi, Ernesto; Figueredo, Alejandra; Christiansen, Silvia; Cristiano, Edgardo; Bertini, Enrico S; Rugiero, Marcelo

2016-10-15

Cases of acquired rippling muscle disease in association with myasthenia gravis have been reported. We present three patients with iRMD (immune-mediated rippling muscle disease) and AChR-antibody positive myasthenia gravis. None of them had thymus pathology. They presented exercise-induced muscle rippling combined with generalized myasthenia gravis. One of them had muscle biopsy showing a myopathic pattern and a patchy immunostaining with caveolin antibodies. They were successfully treated steroids and azathioprine. The immune nature of this association is supported by the response to immunotherapies and the positivity of AChR-antibodies. Copyright © 2016 Elsevier B.V. All rights reserved.

Case Report: Ocular Myasthenia Gravis Associated with In Vitro Fertilization Procedures.

Science.gov (United States)

Yoo, Yung Ju; Han, Sang Beom; Yang, Hee Kyung; Hwang, Jeong-Min

2018-05-01

Ocular myasthenia gravis is a localized form of myasthenia gravis, which is a postsynaptic disorder of the neuromuscular junction that causes fluctuating weakness of extraocular muscles resulting from autoimmune mechanisms. In women with myasthenia, changes in sex hormone levels and administration of corticosteroids can trigger or worsen symptoms of myasthenia gravis. To describe a case of seronegative ocular myasthenia gravis whose first symptom appeared a day after in vitro fertilization procedure. A 37-year-old woman suddenly developed mild ptosis and fluctuating diplopia that worsened in the evening. Before the development of symptoms, she had undergone in vitro fertilization procedure and had taken oral steroids. Ocular motility examination revealed an intermittent exotropia in primary gaze at both distance and near. The neostigmine test confirmed her diagnosis as ocular myasthenia gravis. When taking a history for young women with sudden onset of binocular diplopia, steroids and sex hormones should be taken into account, which may trigger or exacerbate symptoms of ocular myasthenia gravis.

Pembrolizumab-induced myasthenia gravis: A fatal case report.

Science.gov (United States)

March, Katherine L; Samarin, Michael J; Sodhi, Amik; Owens, Ryan E

2018-03-01

Purpose Pembrolizumab, a monoclonal antibody which inhibits the programmed cell death 1 receptor, has been shown to efficaciously enhance pre-existing immune responses to malignancies. However, safety concerns must also be considered as pembrolizumab use has been associated with several life-threatening immune-related adverse events (irAEs). We report a fatal case of pembrolizumab-induced myasthenia gravis in a patient with no prior myasthenia gravis history. Case report A 63-year-old male presented with right eyelid drooping, puffiness, blurred vision, and shortness of breath two weeks after an initial infusion of pembrolizumab. He was subsequently diagnosed with new onset acetylcholine-receptor positive myasthenia gravis. Despite aggressive treatment with corticosteroids, pyridostigmine, intravenous immunoglobulin, and plasmapheresis, the patient clinically deteriorated and ultimately expired from acute respiratory failure after a 12-day hospitalization. Discussion Current package labeling for pembrolizumab warns against various irAEs associated with its use including pneumonitis, colitis, and endocrinopathies. To date, only one case of new onset myasthenia gravis and two case reports of myasthenia gravis exacerbation have been identified. This case further highlights the mortality risk associated with development of irAEs. Conclusion While rare, evidence for the development of MG associated with pembrolizumab is growing. Prompt recognition of symptoms and discontinuation of pembrolizumab is necessary to help improve prognosis.

An Unusual Presentation of Myasthenia Gravis | Chijioke | West ...

African Journals Online (AJOL)

BACK GROUND: Myasthenia gravis (MG) is generally a rare disorder and may thus be easily misdiagnosed. Misdiagnosis is even more likely when the presentation is atypical. OBJECTIVE: To present and discuss an unusual presentation of myasthenia gravis METHOD: A 67-year-old man presented with progressive ...

Neuromuscular disease mimicking myasthenia gravis in a Nigerian ...

African Journals Online (AJOL)

Background : Nemaline rod disease is a congenital myopathy, presentation of which may mimic myasthenia gravis. Methods : We report a suspected case of nemaline rod disease in a female adolescent who presented with features similar to myasthenia gravis but failed to respond effectively to its conventional management ...

Thymus irradiation for myasthenia gravis

International Nuclear Information System (INIS)

Currier, R.D.; Routh, A.; Hickman, B.T.; Douglas, M.A.

1983-01-01

Twenty-eight patients with progressive myasthenia gravis without thymoma received treatment of 3000 rads (30 Gy) to the anterior mediastinum, and a followup was conducted for five to 18 years. Twenty-four patients had generalized myasthenia, and four had ocular myasthenia gravis. Twenty patients with generalized myasthenia survived the several month post-treatment period and improved, but four died during that period. The improvement lasted a median of 1.5 years, and older patients had longer remissions than younger patients. The four patients who had ocular myasthenia did not change after treatment. Mediastinal irradiation produces a temporary remission in generalized myasthenia

Thymus irradiation for myasthenia gravis

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Currier, R.D.; Routh, A.; Hickman, B.T.; Douglas, M.A.

1983-01-01

Twenty-eight patients with progressive myasthenia gravis without thymoma received treatment of 3000 rads (30 Gy) to the anterior mediastinum, and a followup was conducted for five to 18 years. Twenty-four patients had generalized myasthenia, and four had ocular myasthenia gravis. Twenty patients with generalized myasthenia survived the several month post-treatment period and improved, but four died during that period. The improvement lasted a median of 1.5 years, and older patients had longer remissions than younger patients. The four patients who had ocular myasthenia did not change after treatment. Mediastinal irradiation produces a temporary remission in generalized myasthenia.

Eight-year follow-up of patients with myasthenia gravis after thymectomy.

Science.gov (United States)

Yu, S; Li, F; Chen, B; Lin, J; Yang, M; Fu, X; Li, J; Bu, B

2015-02-01

To depict the long-term outcome of patients with myasthenia gravis after thymectomy in combination with immunotherapy, and the factors that may potentially affect the outcome. The 306 patients with myasthenia gravis who underwent extended thymectomy from January 1984 to December 2011 at Tongji Hospital were retrospectively evaluated. The patients consisted of 174 cases with thymoma and 132 cases without thymoma. Pharmaceutical treatment was tailored for each case during follow-up. Nine patients with thymomatous myasthenia gravis died during the perioperative period, and 297 patients were followed for 8.6 years. By their latest visits, 241 patients (81.1%) gained satisfactory efficacy, 24 cases died (8.1%), and 32 cases (10.8%) remained unchanged or deteriorated. Favorable factors for satisfactory efficacy included the presence of ocular myasthenia gravis before operation, no presence of thymoma, and lack of concomitant diseases. It is interesting to mention that, patients with non-thymomatous myasthenia gravis obtained significantly higher rates of complete stable remission and clinical remission than the patients with thymomatous myasthenia gravis. Extended thymectomy combined with immunotherapy is a preferred treatment with a satisfactory long-term remission rate. Patients with non-thymomatous myasthenia gravis have a much more promising prognosis than the patients with thymomatous myasthenia gravis. However, appropriate caution must be taken to discontinue pharmaceutical therapy as relapse remains a major concern after a patient who has already undergone thymectomy becomes symptom-free. © 2014 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

Myasthenia Gravis Associated With Autoimmune Thyroid Disease: A ...

African Journals Online (AJOL)

Myasthenia gravis (MG) is an acquired autoimmune disorder causing skeletal muscle fatigue and weakness. This is a report of one woman and her daughter presenting with myasthenia and gravis and Grave\\'s disease. It highlights possible hereditary component of this condition which has not been commonly reported in ...

Informes clínicos breves

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Facultad de Medicina Revista

1997-01-01

Full Text Available Reversión con nalbufina de la depresión hemodinámica inducida por Fentanyl en pacientes sépticos / Factores de riesgo para el desarrollo de epilepsia refractaria, en el Hospital San Juan de Dios / Vasculitis Séptica, factores de riesgo y hallazgos histopatológicas en autopsias / Miastenia Gravis, efectos de la timectomía radical / Luxación anterior habitual de cadera / Disección anatómica del espacio parafaríngeo

Adult celiac disease with acetylcholine receptor antibody positive myasthenia gravis

Institute of Scientific and Technical Information of China (English)

Hugh J Freeman; Helen R Gillett; Peter M Gillett; Joel Oger

2009-01-01

Celiac disease has been associated with some autoimmune disorders. A 40-year-old competitive strongman with celiac disease responded to a glutenfree diet, but developed profound and generalized motor weakness with acetylcholine receptor antibody positive myasthenia gravis, a disorder reported to occur in about 1 in 5000. This possible relationship between myasthenia gravis and celiac disease was further explored in serological studies. Frozen stored serum samples from 23 acetylcholine receptor antibody positive myasthenia gravis patients with no intestinal symptoms were used to screen for celiac disease. Both endomysial and tissue transglutaminase antibodies were examined. One of 23 (or, about 4.3%) was positive for both IgA-endomysial and IgA tissue transglutaminase antibodies. Endoscopic studies subsequently showed duodenal mucosal scalloping and biopsies confirmed the histopathological changes of celiac disease. Celiac disease and myasthenia gravis may occur together more often than is currently appreciated. The presence of motor weakness in celiac disease may be a clue to occult myasthenia gravis, even in the absence of intestinal symptoms.

Myasthenia gravis and pregnancy: clinical implications and neonatal outcome

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Estanol Bruno

2004-11-01

Full Text Available Abstract Background The myasthenia gravis is twice as common in women as in men and frequently affects young women in the second and third decades of life, overlapping with the childbearing years. Generally, during pregnancy in one third of patients the disease exacerbates, whereas in two thirds it remains clinically unchanged. Complete remission can occur in some patients. Methods To describe the clinical course, delivery and neonatal outcome of 18 pregnant women with the diagnosis of myasthenia gravis. Retrospective chart review of pregnant patients with myasthenia gravis, followed at the National Institute of Perinatology in Mexico City over an 8-year period. Data was abstracted from the medical records on the clinical course during pregnancy, delivery and neonatal outcome. Results From January 1, 1996 to December 31, 2003 18 patients with myasthenia gravis were identified and included in the study. The mean ± SD maternal age was 27.4 ± 4.0 years. During pregnancy 2 women (11% had an improvement in the clinical symptoms of myasthenia gravis, 7 women (39% had clinical worsening of the condition of 9 other patients (50% remained clinically unchanged. Nine patients delivered vaginally, 8 delivered by cesarean section and 1 pregnancy ended in fetal loss. Seventeen infants were born at mean ± SD gestational age of 37.5 ± 3.0 weeks and a mean birth weight of 2710 ± 73 g. Only one infant presented with transient neonatal myasthenia gravis. No congenital anomalies were identified in any of the newborns. Conclusions The clinical course of myasthenia gravis during pregnancy is variable, with a significant proportion of patients experiencing worsening of the clinical symptoms. However, neonatal transient myasthenia was uncommon in our patient population.

Tacrolimus Improves Symptoms of Children With Myasthenia Gravis Refractory to Prednisone.

Science.gov (United States)

Liu, Chanchan; Gui, Mengcui; Cao, Yayun; Lin, Jing; Li, Yue; Ji, Suqiong; Bu, Bitao

2017-12-01

Myasthenia gravis tends to affect children in China. Oral pyridostigmine and prednisone could effectively improve the symptoms, but multiple side effects become a major concern after long-term oral prednisone. To avoid the long-term complications of prednisone therapy and to obtain more satisfactory improvement, we tested the efficacy and safety of tacrolimus in children with myasthenia gravis. Children with myasthenia gravis who had not achieved satisfactory improvement or who experienced severe side effects after prednisone therapy were recruited between January 2015 and December 2016 at Tongji Hospital. All the children were treated with tacrolimus 1 mg to 2 mg daily and the dose was adjusted on the basis of the clinical response and the serum concentration. The dosage of prednisone, the severity of symptoms, blood samples, the serum concentration of tacrolimus, and titers of antiacetylcholine receptor antibodies were evaluated every four weeks. Fourteen children were enrolled. One child withdrew two weeks after the enrollment. Thirteen children have completed the therapy for one year. At the end point, the dosage of prednisone was significantly decreased (P myasthenia gravis score, and myasthenia gravis-specific manual muscle testing and myasthenia gravis-activities of daily living scores were significantly improved (P myasthenia gravis. Randomized clinical trials are needed to confirm the observation. Copyright © 2017 Elsevier Inc. All rights reserved.

Miastenia gravis: resultados de timectomia em 52 pacientes

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José Teotonio de Oliveira

1995-06-01

Full Text Available Durante o período de 1971 a 1993, 52 miastênicos sem timoma (7 homens e 45 mulheres foram timectomizados por via esternal. Os resultados foram classificados em remissão e não-remissão . A taxa de remissão foi 48%. No grupo de remissões havia 5 homens e 20 mulheres. O tempo de seguimento foi de 5,5 anos em ambos os grupos. Os pacientes foram classificados clinicamente segundo a classificação de Osserman. De 16 pacientes na categoria II A, 11 entraram em remissão; de 36 pacientes nas categorias II B e III, 14 entraram em remissão. O tempo de duração de doença foi de 1,8 e 4,3 anos nos grupos de remissões e não-remissões, respectivamente. Todos os pacientes que entraram em remissão tinham menos de quatro anos de doença. Dos 43 pacientes com menos de quatro anos de doença, 42% não apresentaram remissão significando que um tempo de doença de menos de quatro anos não é indicador de remissão. Nossos dados indicam um melhor prognóstico quando a timectomia é feita nos primeiros quatro anos de doença.

Relato literario, relato periodístico: el caso Elián González

OpenAIRE

Lic. María Inés Loyola; Lic. Miriam Eugenia Villa

2000-01-01

Nos proponemos estudiar las relaciones entre relato literario y relato periodístico a partir del caso del niño balsero cubano, Elián González, que estuvo retenido en Estados Unidos durante siete meses. En este análisis, el principal objetivo es describir las diferencias y similitudes respecto a la construcción discursiva de la historia narrada, sus personajes y escenarios. La finalidad de la comparación es demostrar cómo los relatos "no ficcionales" que los medios imponen como verdaderos y ac...

Higgs decays and brane gravi-vectors

International Nuclear Information System (INIS)

Clark, T. E.; Liu Boyang; Love, S. T.; Xiong, C.; Veldhuis, T. ter

2008-01-01

Higgs boson decays in flexible brane world models with stable, massive gravi-vectors are considered. Such vectors couple bilinearly to the standard model fields through either the standard model energy-momentum tensor, the weak hypercharge field strength, or the Higgs scalar. The role of the coupling involving the extrinsic curvature is highlighted. It is found that within the presently allowed parameter space, the decay rate of the Higgs into two gravi-vectors (which would appear as an invisible Higgs decay) can be comparable to the rate for any of the standard model decay modes.

Nutrition and Myasthenia Gravis

Science.gov (United States)

Good nutrition is important for everyone. This is especially true when you have a chronic disorder like myasthenia gravis ( ... difficulty with chewing and swallowing may interfere with nutrition and create safety issues. Diet modifications may be ...

Successful use of sugammadex for caesarean section in a patient with myasthenia gravis

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Lokman Soyoral

Full Text Available Abstract Myasthenia gravis is an autoimmune disorder that is characterized by muscle weakness that fluctuates, worsening with exertion, and improving with rest. Diagnosis of myasthenia gravis is made following clinical and physical examination and is confirmed by serum immunoassays to measure autoantibody levels. Myasthenia gravis especially when associated with pregnancy is a high-risk disease, and its course is unpredictable. We described the second report about use of sugammadex after rocuronium for a caesarean delivery with myasthenia gravis, but, unlike our case that formerly was diagnosed with myasthenia gravis, the patient was extubated on postoperative successfully and we did not encounter any respiratory problems.

Hickam's dictum: Myasthenia Gravis presenting concurrently with Graves' disease.

Science.gov (United States)

Sehgal, Shekhar; Rebello, Roshan; Wolmarans, Louise; Elston, Marianne

2017-09-07

We present two patients with Graves' disease and concurrent myasthenia gravis. The impact of the dual diagnosis on the clinical course and the potential for a delayed diagnosis of myasthenia gravis is discussed. Patient 1, a 28-year-old man was diagnosed with Graves' disease following his second respiratory arrest. His history was strongly suggestive of a second pathology. Patient 2, a 66-year-old Cantonese woman with established Graves' disease presented with thionamide-related neutropaenia. Examination revealed bilateral ptosis and right lateral rectus palsy. Both patients had thyrotoxicosis secondary to Graves' disease with concurrent myasthenia gravis. Although neuromuscular weakness is common in Graves' disease, coexisting myasthenia gravis (MG) is rare and can cause profound morbidity. Ocular signs in both diseases may cause diagnostic confusion although ptosis suggests coexisting MG. In both cases, the thyrotoxicosis delayed the diagnosis of MG. © BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

Computer tomography in myasthenia gravis

International Nuclear Information System (INIS)

Suzuki, Masayuki; Takashima, Tsutomu; Itoh, Hiroshi

1985-01-01

In this report, preoperative CT diagnosis of the thymus was reevaluated in 15 operated patients with myasthenia gravis. Using GE CT/T 8800 wholebody scanner, pre- and/or post-contrast study was performed from the apex to the diaphragm level. Five cases of thymoma, 5 out of 6 cases of thymic hyperplasia and 3 cases of normal thymus were correctly diagnosed. One hyperplasia was diagnosed as normal and one normal thymus as hyperplasia. At the CT evaluation of the thymus in patients with myasthenia gravis, it is easy to detect thymoma, but it is often difficult to differentiate thymic hyperplasia and normal thymus due to the aging changes in the gland and the presence of localized hyperplasia. (author)

Successful use of sugammadex for caesarean section in a patient with myasthenia gravis.

Science.gov (United States)

Soyoral, Lokman; Goktas, Ugur; Cegin, Muhammed Bilal; Baydi, Volkan

Myasthenia gravis is an autoimmune disorder that is characterized by muscle weakness that fluctuates, worsening with exertion, and improving with rest. Diagnosis of myasthenia gravis is made following clinical and physical examination and is confirmed by serum immunoassays to measure autoantibody levels. Myasthenia gravis especially when associated with pregnancy is a high-risk disease, and its course is unpredictable. We described the second report about use of sugammadex after rocuronium for a caesarean delivery with myasthenia gravis, but, unlike our case that formerly was diagnosed with myasthenia gravis, the patient was extubated on postoperative successfully and we did not encounter any respiratory problems. Copyright © 2014 Sociedade Brasileira de Anestesiologia. Published by Elsevier Editora Ltda. All rights reserved.

[Successful use of sugammadex for caesarean section in a patient with myasthenia gravis].

Science.gov (United States)

Soyoral, Lokman; Goktas, Ugur; Cegin, Muhammed Bilal; Baydi, Volkan

Myasthenia gravis is an autoimmune disorder that is characterized by muscle weakness that fluctuates, worsening with exertion, and improving with rest. Diagnosis of myasthenia gravis is made following clinical and physical examination and is confirmed by serum immunoassays to measure autoantibody levels. Myasthenia gravis especially when associated with pregnancy is a high-risk disease, and its course is unpredictable. We described the second report about use of sugammadex after rocuronium for a caesarean delivery with myasthenia gravis, but, unlike our case that formerly was diagnosed with myasthenia gravis, the patient was extubated on postoperative successfully and we did not encounter any respiratory problems. Copyright © 2014 Sociedade Brasileira de Anestesiologia. Publicado por Elsevier Editora Ltda. All rights reserved.

Multiple thymoma in a patient with myasthenia gravis: case report

International Nuclear Information System (INIS)

Ko, Eun Sook; Jeon, Kyung Nyeo; Bae, Kyung Soo; Yoo, Jin Jong; Kang, Duk Sik

2004-01-01

A thymoma often occurs in patients with myasthenia gravis, but the development of multiple thymoma is very rare. The authors report the radiologic and pathologic findings of multiple invasive thymoma in a 59-year-old male with myasthenia gravis

Multiple thymoma in a patient with myasthenia gravis: case report

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Ko, Eun Sook; Jeon, Kyung Nyeo; Bae, Kyung Soo; Yoo, Jin Jong [College of Medicine, Gyeongsang National Univ., Jinju (Korea, Republic of); Kang, Duk Sik [College of Medicine, Kyungpook National Univ., Daegu (Korea, Republic of)

2004-02-01

A thymoma often occurs in patients with myasthenia gravis, but the development of multiple thymoma is very rare. The authors report the radiologic and pathologic findings of multiple invasive thymoma in a 59-year-old male with myasthenia gravis.

Muscle autoantibodies in myasthenia gravis: beyond diagnosis?

Science.gov (United States)

Meriggioli, Matthew N; Sanders, Donald B

2012-01-01

Myasthenia gravis is an autoimmune disorder of the neuromuscular junction. A number of molecules, including ion channels and other proteins at the neuromuscular junction, may be targeted by autoantibodies leading to abnormal neuromuscular transmission. In approximately 85% of patients, autoantibodies, directed against the postsynaptic nicotinic acetylcholine receptor can be detected in the serum and confirm the diagnosis, but in general, do not precisely predict the degree of weakness or response to therapy. Antibodies to the muscle-specific tyrosine kinase are detected in approximately 50% of generalized myasthenia gravis patients who are seronegative for anti-acetylcholine receptor antibodies, and levels of anti-muscle-specific tyrosine kinase antibodies do appear to correlate with disease severity and treatment response. Antibodies to other muscle antigens may be found in the subsets of myasthenia gravis patients, potentially providing clinically useful diagnostic information, but their utility as relevant biomarkers (measures of disease state or response to treatment) is currently unclear. PMID:22882218

Early onset bilateral juvenile myasthenia gravis masquerading as simple congenital ptosis

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Alam, Md. Shahid

2017-03-01

Full Text Available Myasthenia gravis is an autoimmune disorder affecting the neuromuscular junction. Ocular myasthenia gravis presents as ptosis with extraocular motility restriction and is prone to be misdiagnosed as third nerve palsy or congenital or aponeurotic ptosis. Juvenile ocular myasthenia gravis in very young children is difficult to diagnose and can be easily labeled as a case of congenital ptosis, the more so when the condition is bilateral. We present a case of a two-year-old child who presented with bilateral ptosis and was diagnosed as a case of simple congenital ptosis elsewhere with the advice to undergo tarsofrontalis sling surgery. The child was diagnosed with juvenile myasthenia gravis on thorough history, examination, and systemic evaluation and was started on anti-myasthenic treatment.

Results of surgical treatment for juvenile myasthenia gravis.

Science.gov (United States)

Vázquez-Roque, F J; Hernández-Oliver, M O; Medrano Plana, Y; Castillo Vitlloch, A; Fuentes Herrera, L; Rivero-Valerón, D

2017-04-01

Radical or extended thymectomy is an effective treatment for myasthenia gravis in the adult population. There are few reports to demonstrate the effectiveness of this treatment in patients with juvenile myasthenia gravis. The main objective of this study was to show that extended transsternal thymectomy is a valid option for treating this disease in paediatric patients. Twenty-three patients with juvenile myasthenia gravis underwent this surgical treatment in the period between April 2003 and April 2014; mean age was 12.13 years and the sample was predominantly female. The main indication for surgery, in 22 patients, was the generalised form of the disease (Osserman stage II) together with no response to 6 months of medical treatment. The histological diagnosis was thymic hyperplasia in 22 patients and thymoma in one patient. There were no deaths and no major complications in the postoperative period. After a mean follow-up period of 58.87 months, 22 patients are taking no medication or need less medication to manage myasthenic symptoms. Extended (radical) transsternal thymectomy is a safe and effective surgical treatment for juvenile myasthenia gravis. Copyright © 2015 Sociedad Española de Neurología. Publicado por Elsevier España, S.L.U. All rights reserved.

Myasthenia gravis and pregnancy: anaesthetic management--a series of cases.

Science.gov (United States)

Almeida, Carlos; Coutinho, Ester; Moreira, Daniela; Santos, Ernestina; Aguiar, José

2010-11-01

Myasthenia gravis is an autoimmune neuromuscular disease, usually affecting women in the second and third decades. The course is unpredictable during pregnancy and puerperium. Myasthenia gravis can cause major interference in labour and partum and exacerbations of the disease frequently occur. The aim of this series of cases is to analyse retrospectively the anaesthetic management of myasthenia gravis patients and complications during the peripartum period. Retrospective, single centre study from clinical files of female myasthenia gravis patients who delivered between 1985 and 2007 at Hospital de Santo António, Porto, Portugal. Seventeen myasthenia gravis patients delivered between 1985 and 2007 in Hospital Santo António. Two women were not included in the study as they had a spontaneous abortion in the first trimester. Four patients presented exacerbations of the disease during pregnancy, no exacerbation occurred in eight patients and three patients presented their first symptoms of myasthenia gravis during pregnancy (without diagnosis at time of delivery). Concerning the eight patients without exacerbations of the disease during pregnancy, pregnancy was brought to term in 87.5% of the cases; five women were submitted to nonurgent caesarean section (62.5%); and epidural block was performed in six patients (75%). No complications related to anaesthesia occurred in the peripartum period. Concerning the four patients with exacerbations of the disease, pregnancy was brought to term in three cases (75%); three women were submitted to nonurgent caesarean section (75%); and epidural block was performed in three patients (75%). One patient underwent an uncomplicated thymectomy under general anaesthesia during pregnancy and, in the postpartum period, there was a myasthenic crisis in another patient. Concerning the three patients without a myasthenia gravis diagnosis at partum, one woman already being followed for presenting muscular weakness had a vaginal delivery under

[Neuropsychological performance in patients with myasthenia gravis].

Science.gov (United States)

Eizaguirre, María Bárbara; Aguirre, Florencia; Yastremiz, Cecilia; Vanotti, Sandra; Villa, Andrés

2017-01-01

Myasthenia gravis is a chronic autoimmune disease that affects the neuromuscular transmission. Controversial findings had been reported about cognitive impairment in this disease. The aim of this study was to investigate the cognitive pattern of patients with myasthenia gravis. There were enrolled 24 patients with myasthenia gravis, anti-acetylcholine receptor antibodies (ACRA) positive, and 24 healthy controls. age 43.9 ± 14.8, years of education 10.9 ± 3.3. age 44.5 ± 15.4, years of education 11.5 ± 3.3. The following areas were evaluated: verbal memory: (long-term storage, retrieval, delayed recall) of the Selective Remained Test; attention: Paced Auditory Serial Addition Task (PASAT 2 and 3 seconds); executive functions: analogies and numbers-letters sequence. Also, it was administered the Beck Depression Inventory II (BDI II). About 33.3% of patients obtained abnormal performance in two or more cognitive tests. 37.5% showed deterioration in attention; 33.3% in verbal memory; 29.2% in executive functions. Significant differences between patients and healthy controls were found in long-term storage (p = 0.001); retrieval (p = 0.007); delayed recall (p = 0.000); PASAT 3 (p = 0.009); PASAT 2 (p = 0.009) and analogies (p = 0.003). Evidence of depression was found: mild in 4.2% of patients; moderate in 25% and severe in 29.2%. Neuropsychological performance declines in patients with myasthenia gravis: attention was more affected than other cognitive areas.

CT evaluation of thymus in myasthenia gravis

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Kim, Guk Hee [Insung Hospital, Chuncheon (Korea, Republic of); Kang, Eun Young; Lee, Nam Joon; Suh, Won Hyuck [Korea University College of Medicine, Seoul (Korea, Republic of)

1989-12-15

The relationship between myasthenia gravis and the thymus was well establish and myasthenia gravis occurs in the presence of thymic hyperplasia or thymoma or occasionally in histologically normal thymus. Since not every patients with myasthenia gravis is a candidate for thymectomy, unless a thymoma is present, the differentiation of normal and hyperplastic thymus from thymoma becomes important. Authors reviewed retrospectively clinical records and chest CT scans of 18 patients with myasthenia gravis who underwent thymectomy during recent 5 years, to evaluate the role of CT scan. The results were as follows. 1 Of total 18 cases, 5 cases had normal thymus, 6 cases had thymic hyperplasia, 4 cases had benign thymoma and 3 cases had malignant thymoma. 2. Of 5 cases of normal thymus, no false positive cases were noted in CT scan. 3. Of 6 cases of thymic hyperplasia, CT findings were normal except 1 cases of thickened left thymic lobe. 4. Of 7 cases of thymoma, no false negative cases were noted in CT scan. 5. CT findings of benign thymoma were round or oval shaped, discrete, slightly enhancing soft tissue mass in anterior mediastinum. 6. CT findings of malignant thymoma were lobulated contoured, infiltrative, soft tissue mass lesion in anterior mediastinum with calcifications, pleural tumor implants, and SVC compression. CT yielded valuable information on differential diagnosis of thymoma, thymic hyperplasia and normal thymus. Also CT was a highly sensitive method in the detection of thymoma and determining the extent and invasiveness.

CT evaluation of thymus in myasthenia gravis

International Nuclear Information System (INIS)

Kim, Guk Hee; Kang, Eun Young; Lee, Nam Joon; Suh, Won Hyuck

1989-01-01

The relationship between myasthenia gravis and the thymus was well establish and myasthenia gravis occurs in the presence of thymic hyperplasia or thymoma or occasionally in histologically normal thymus. Since not every patients with myasthenia gravis is a candidate for thymectomy, unless a thymoma is present, the differentiation of normal and hyperplastic thymus from thymoma becomes important. Authors reviewed retrospectively clinical records and chest CT scans of 18 patients with myasthenia gravis who underwent thymectomy during recent 5 years, to evaluate the role of CT scan. The results were as follows. 1 Of total 18 cases, 5 cases had normal thymus, 6 cases had thymic hyperplasia, 4 cases had benign thymoma and 3 cases had malignant thymoma. 2. Of 5 cases of normal thymus, no false positive cases were noted in CT scan. 3. Of 6 cases of thymic hyperplasia, CT findings were normal except 1 cases of thickened left thymic lobe. 4. Of 7 cases of thymoma, no false negative cases were noted in CT scan. 5. CT findings of benign thymoma were round or oval shaped, discrete, slightly enhancing soft tissue mass in anterior mediastinum. 6. CT findings of malignant thymoma were lobulated contoured, infiltrative, soft tissue mass lesion in anterior mediastinum with calcifications, pleural tumor implants, and SVC compression. CT yielded valuable information on differential diagnosis of thymoma, thymic hyperplasia and normal thymus. Also CT was a highly sensitive method in the detection of thymoma and determining the extent and invasiveness

Neuropsychological performance in patients with myasthenia gravis

Directory of Open Access Journals (Sweden)

María Bárbara Eizaguirre

2017-04-01

Full Text Available Myasthenia gravis is a chronic autoimmune disease that affects the neuromuscular transmission. Controversial findings had been reported about cognitive impairment in this disease. The aim of this study was to investigate the cognitive pattern of patients with myasthenia gravis. There were enrolled 24 patients with myasthenia gravis, anti-acetylcholine receptor antibodies (ACRA positive, and 24 healthy controls. Patients: age 43.9 ± 14.8, years of education 10.9 ± 3.3. Controls: age 44.5 ± 15.4, years of education 11.5 ± 3.3. The following areas were evaluated: verbal memory: (long-term storage, retrieval, delayed recall of the Selective Remained Test; attention: Paced Auditory Serial Addition Task (PASAT 2 and 3 seconds; executive functions: analogies and numbers-letters sequence. Also, it was administered the Beck Depression Inventory II (BDI II. About 33.3% of patients obtained abnormal performance in two or more cognitive tests. 37.5% showed deterioration in attention; 33.3% in verbal memory; 29.2% in executive functions. Significant differences between patients and healthy controls were found in long-term storage (p = 0.001; retrieval (p = 0.007; delayed recall (p = 0.000; PASAT 3 (p = 0.009; PASAT 2 (p = 0.009 and analogies (p = 0.003. Evidence of depression was found: mild in 4.2% of patients; moderate in 25% and severe in 29.2%. Neuropsychological performance declines in patients with myasthenia gravis: attention was more affected than other cognitive areas

[An update on myasthenia gravis].

Science.gov (United States)

Martínez Torre, S; Gómez Molinero, I; Martínez Girón, R

2018-03-16

Myasthenia gravis is one of the most common disorders that affect neuromuscular transmission. It is currently one of the most understood and characterised autoimmune disorders Its typical symptoms are fluctuating weakness and fatigue that affects a combination of ocular muscles, bulbar functions, as well as limb and respiratory muscles, which are due to an immune attack against the postsynaptic membrane of the neuromuscular junction. The diagnosis of myasthenia gravis is based on clinical and serological test. It is a disease that can be effectively controlled with the current therapeutic lines, even achieving a complete remission. An update of this interesting disorder is now presented. Copyright © 2018 Sociedad Española de Médicos de Atención Primaria (SEMERGEN). Publicado por Elsevier España, S.L.U. All rights reserved.

Ephedrine for myasthenia gravis, neonatal myasthenia and the congenital myasthenic syndromes

NARCIS (Netherlands)

Vrinten, C.; van der Zwaag, A.M.; Weinreich, S.S.; Scholten, R.J.; Verschuuren, J.J.

2014-01-01

BACKGROUND: Myasthenia is a condition in which neuromuscular transmission is affected by antibodies against neuromuscular junction components (autoimmune myasthenia gravis, MG; and neonatal myasthenia gravis, NMG) or by defects in genes for neuromuscular junction proteins (congenital myasthenic

The diagnosis of thymoma and thymic atrophy in patients with myasthenia gravis; Dignostikk av tymom og thymusatrofi hos pasienter med myasthenia gravis

Energy Technology Data Exchange (ETDEWEB)

Sund, K.K.; Skeie, G.O.; Gilhus, N.E.; Aarli, J.A.; Varhaug, J.E. [Haukeland Sykehus, Bergen (Norway)

1997-11-01

The authors have compared clinical, immunological and radiological data in 20 patients with myasthenia gravis and thymoma and in 21 patients with myasthenia gravis and thymic atrophy. The median age at onset was 54 years in the thymoma group and 63 years in the thymic atrophy group. The severity of the disease was similar in the two groups, and there was no significant difference in the concentration of acetylcholine receptor antibodies. CA antibodies were demonstrated in 17/20 thymoma patients and in 6/21 with thymic atrophy, while 19/20 thymoma patients had antibodies to titin, compared with 9/21 among those with thymic atrophy. The diagnosis and treatment of patients with myasthenia gravis is based upon an evaluation of clinical, immunological and radiological data. 28 refs., 2 tabs.

Prevalence of myasthenia gravis in the Catalan county of Osona.

Science.gov (United States)

Aragonès, J M; Altimiras, J; Roura, P; Alonso, F; Bufill, E; Munmany, A; Alfonso, S; Illa, I

The reported prevalence of myasthenia gravis ranges between 5 and 24 cases per 100,000, and people over 65years account for less than 50% of all cases. The prevalence and clinical characteristics of myasthenia gravis in the county of Osona were studied in patients younger and older than 65. The study draws from the county-based prospective myasthenia gravis register implemented by the Neurology Department at Hospital General de Vic in 1991. The prevalence of myasthenia gravis was 32.89×10 5 inhabitants (95%CI, 23.86-41.91). The standardized prevalence (European population) was 35.47×10 5 inhabitants (95%CI, 26.10-44.84). The ratio of women to men was 1.3. Overall, the group of patients older than 65 accounted for 62.75% of all cases. The prevalence of myasthenia gravis increased considerably in older age groups. No cases were registered among patients under 25years old, prevalence was 21.87×10 5 in the 25 to 64 age group, and prevalence in patients over 65 years increased to 122.35×10 5 . The clinical characteristics prior to treatment and at the cut-off date are similar (P>.05) in patients younger than 65 and those aged 65 and older. These figures show the highest prevalence rate reported to date. This high prevalence is due to the rate observed among patients older than 65. These results provide a new warning that myasthenia gravis may be underdiagnosed in the elderly population. Copyright © 2014 Sociedad Española de Neurología. Publicado por Elsevier España, S.L.U. All rights reserved.

An increase in circulating B cell-activating factor in childhood-onset ocular myasthenia gravis.

Science.gov (United States)

Motobayashi, Mitsuo; Inaba, Yuji; Nishimura, Takafumi; Kobayashi, Norimoto; Nakazawa, Yozo; Koike, Kenichi

2015-04-01

Myasthenia gravis is a B cell-mediated autoimmune disorder. The pathophysiology of childhood-onset ocular myasthenia gravis remains unclear. We investigated serum B cell-activating factor levels and other immunological parameters in child patients with ocular myasthenia gravis. Blood samples were obtained from 9 children with ocular myasthenia gravis and 20 age-matched controls. We assayed serum concentrations of B cell-activating factor, anti-acetylcholine receptor antibody titers, 7 types of cytokines (interleukins-2, -4, -6, -10, and -17A; interferon-γ; tumor necrosis factor-α) as well as the percentages of peripheral blood CD4+, CD8+, and CD19+ cells. Serum B cell-activating factor levels were significantly higher before immunosuppressive therapy in patients with childhood-onset ocular myasthenia gravis than in controls and decreased after immunosuppressive therapy. A significant positive correlation was observed between serum B cell-activating factor levels and anti-acetylcholine receptor antibody titers in patients with myasthenia gravis. Serum B cell-activating factor concentrations did not correlate with the percentages of CD4+, CD8+, and CD19+ cells or the CD4+/CD8+ ratio. No significant differences were observed in the levels of the 7 different types of cytokines examined, including interleukin-17A, between preimmunosuppressive therapy myasthenia gravis patients and controls. Circulating B cell-activating factor may play a key role in the pathophysiology of childhood-onset ocular myasthenia gravis. Copyright © 2015 Elsevier Inc. All rights reserved.

Myasthenia Gravis – a beginning with no end

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Croitoru Cristina Georgiana

2016-03-01

Full Text Available Myasthenia gravis is one of the neurological diseases with a relatively recent history, full of mistakes, in which the British and German neurology schools have attempted to find answers when confronted with the unknown. The paper aims at making a historical account of the disease from its discovery in the 16th century, when the first case of myasthenia gravis was medically diagnosed, to the beginning of the 20th century, when the dawn of modern therapy started to show.

Myasthenia gravis: recent advances in immunopathology and therapy.

Science.gov (United States)

Lee, John-Ih; Jander, Sebastian

2017-03-01

Myasthenia gravis is the most frequent acquired disorder of neuromuscular transmission. In the majority of cases, pathogenic antibodies against components of the postsynaptic muscle endplate membrane can be detected. In recent years there have been significant advances in the pathophysiological understanding and therapy of the disease. Areas covered: PubMed searches were conducted for the term 'myasthenia gravis' cross-referenced with the terms 'immunology', 'subgroups', 'antibody', 'ocular', 'thymoma', 'treatment' and 'thymectomy'. Additionally, we summarized the current state of immunopathology and therapy. Expert commentary: Immunological research defined new target antigens at the postsynaptic neuromuscular junction which along with clinical features allow a refined definition of disease subgroups. Overall the prognosis of myasthenia gravis with best possible symptomatic, immunosuppressive and supportive treatment is good but new immunomodulatory treatment options are developed for patients who do not respond well to the first line therapy. For most patients individually adapted long-term drug therapy is needed.

Anestesia para timectomía toracoscópica, vídeo asistida: Reporte de 4 casos

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Mercedes Argudín Cordero

2000-06-01

Full Text Available La timectomía toracoscópica con vídeo asistida en el niño es un hecho verdaderamente novedoso en Cuba, en atención a la incidencia de la miastenia gravis en la población joven, aunque dicho procedimiento no está exento de producir complicaciones; ahora bien, si se tiene en cuenta la técnica clásica de abordaje del timo, éstas son mínimas. En el presente trabajo se relaciona la información correspondiente de 4 pacientes, de uno y otro sexos, adolescentes, ASA II y programados para cirugía mayor electiva (timectomía, en los cuales se midieron: frecuencia cardíaca, electrocardiograma, presión arterial sistólica y diastólica respectivamente, frecuencia respiratoria, temperatura esofágica, oximetría de pulso y capnometría, antes, durante y después del procedimiento. Se obtuvieron resultados que concuerdan con la literatura médica revisada, y se concluye como una superior alternativa en los pacientes con miastenia gravis, por las bondades que se observan en los aspectos de baja invasividad, mínimas complicaciones y menor estadía hospitalaria.Video-assisted thoracoscopic thymectomy in children is a real novelty in Cuba to take care of the incidence of myasthenia gravis in the young population. Although such method is not wholly complication-free, if we compare it with the classical technique to thymus approach, then the risks are minimal. The present paper provides information on 4 patients of both sexes, adolescents, ASA II and due to undergo major elective surgery (thymectomy. Parameters such as heart rate, electrocardiogram, systolic and diastolic arterial pressures, respiratory rate, esophagic temperature, pulse oxymetry and capnometry were measured before, during and after surgery. The results achieved were in line with the reviewed literature so the conclusion was reached that this technique is a better choice for patients with myasthenia gravis because of the advantages it offers such as low invasiveness, minimum

Ocular surface alterations and in vivo confocal microscopic characteristics of corneas in patients with myasthenia gravis.

Science.gov (United States)

Erkan Turan, Kadriye; Kocabeyoglu, Sibel; Bekircan-Kurt, Can Ebru; Bezci, Figen; Erdem-Ozdamar, Sevim; Irkec, Murat

2018-03-01

To evaluate ocular surface alterations and characteristics of corneal basal epithelium and subbasal nerves in patients with myasthenia gravis. Myasthenia gravis patients (n = 21) and healthy controls (n = 20) were enrolled. All participants underwent ocular surface testing in the following order: tear break-up time, lissamine green staining, Schirmer I test with anesthesia, and Ocular Surface Disease Index questionnaire. The Cochet-Bonnet esthesiometer was used to measure corneal sensitivity. Basal epithelial cells and subbasal nerves were evaluated using in vivo confocal microscopy. Myasthenia gravis patients had higher Ocular Surface Disease Index score (13.9 ± 15.0 vs 1.4 ± 2.2, p myasthenia gravis had lower basal epithelial cell density (3775.7 ± 938.1 vs 4983.1 ± 608.5, p myasthenia gravis and the number of corneal nerves (rho = -0.497, p = 0.022). Significant alterations of basal epithelial cells and subbasal nerves were demonstrated in myasthenia gravis patients although there was no difference of corneal sensitivity between myasthenia gravis patients and healthy controls. Thus, it should be borne in mind that myasthenia gravis patients deserve further evaluation with regard to ocular surface disease.

The pattern and presentation of myathenia gravis in Al-Shaab and ...

African Journals Online (AJOL)

Introduction: Myasthenia gravis (MG) is the most common acquired autoimmune disorder of neuromuscular transmission. It has different patterns of presentation. Objective: to study the pattern and mode of presentation of Myasthenia Gravis in Al-Shaab and Omdurman Teaching Hospitals, Khartoum Sudan. Methods: The ...

Matrix Metalloproteinases in Myasthenia Gravis

NARCIS (Netherlands)

Helgeland, G.; Petzold, A.F.S.; Luckman, S.P.; Gilhus, N.E.; Plant, G.T.; Romi, F.R.

2011-01-01

Introduction: Myasthenia gravis (MG) is an autoimmune disease with weakness in striated musculature due to anti-acetylcholine receptor (AChR) antibodies or muscle specific kinase at the neuromuscular junction. A subgroup of patients has periocular symptoms only; ocular MG (OMG). Matrix

Muscle strength in myasthenia gravis

DEFF Research Database (Denmark)

Cejvanovic, S; Vissing, J

2014-01-01

OBJECTIVE: Myasthenia gravis (MG) is characterized by fatigue and fluctuating muscle weakness as a result of impaired neuromuscular transmission (NMT). Although MG is a prototypic fatiguing disorder, little is known about how the condition affects fixed weakness, and if present, whether weakness...

Myasthenia Gravis in Pregnancy: A Case Report

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Sebastian Berlit

2012-01-01

Full Text Available Objective. To present a case of maternal myasthenia gravis in pregnancy and give a systematic review of the literature. Case. We report the case of a 38-year-old parturient with a life-threatening complication of immune-mediated myasthenia gravis shortly after an elective cesarean section on patient's request under spinal anesthesia at 35 + 3 weeks of gestation. The newborn was transferred to the pediatric unit for surveillance and did not show any signs of muscular weakness in the course of time. The mother developed a respiratory insufficiency on the second day postpartum. The myasthenic crisis led to a progressive dyspnoea within minutes, which exacerbated in a secondary generalized seizure with cardiac-circulatory arrest. After successful cardiopulmonary resuscitation, the patient was transferred to intensive care. The interdisciplinary therapeutic approach included ventilatory assistance via endotracheal intubation, parenteral pyridostigmine, azathioprine, and steroids. By interdisciplinary measures, a stable state was regained. Conclusion. Myasthenia gravis especially when associated with pregnancy is a high-risk disease. As this disease predominantly occurs in women of reproductive age, it is important to be aware of this condition in obstetrics and its interdisciplinary diagnostic and therapeutic management.

Thymectomy for Nonthymomatous Myasthenia Gravis: Comparison of Video-Assisted Thoracoscopic and Transsternal Thymectomy.

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Bagheri, Reza; Boonstani, Reza; Sadrizadeh, Ali; Salehi, Maryam; Afghani, Reza; Rahnama, Ali; Azmounfar, Vahab; Hakimian, Saeed; Baradaran Firoozabadi, Mohammad

Thymectomy considered as a standard procedure in treatment of all the steps of myasthenia gravis. Video-assisted thoracoscopic surgery (VATS) thymectomy is one of the minimally invasive procedures that because of the short duration of hospitalization, less postoperative pain, and scar after surgery, nowadays it is replaced the traditional methods of surgery for patients with myasthenia gravis, but there are still differences and concerns. The aim of this study was to compare outcomes of two different techniques of surgery, VATS versus transsternal (TS) in the treatment of myasthenia gravis. In this pilot study, 42 patients with myasthenia gravis and without a thymus tumor were evaluated based on Myasthenia Gravis Foundation of America classification and drug consumption. Then, they randomly underwent two different techniques of surgery: VATS versus TS. Patients were evaluated based on preoperative and postoperative variables. Duration of intensive care unit stay and hospitalization has been reduced in patients who underwent VATS thymectomy operation technique. In addition, duration of surgical procedure has been reduced significantly in these patients. These patients have less blood loss during surgery compared with TS group. The Myasthenia Gravis Foundation of America postoperative status in VATS thymectomy revealed that the number of patients with complete stable remission was higher and number of persons remained unchanged was lower in this group. VATS thymectomy is a safe and appropriate approach comparing with traditional methods such as TS thymectomy for patients with myasthenia gravis. This method has better results after surgery and can be used as a minimally invasive alternative method instead of TS thymectomy.

Familial myasthenia gravis: report of four cases

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José Lamartine de Assis

1976-09-01

Full Text Available Two pairs of siblings with myasthenia gravis, belonging to two different families, are reported. This is the only record of familial myasthenia during the past twenty years, in a total of 145 patients seen at the Neurological Clinic of the São Paulo Medical School. In spite of the fact that myasthenia gravis does not show hereditary characteristics, the peculiar features of the four cases justify the present report. The two pairs of siblings were born from non myasthenic nor consanguineous parents. The disease started at birth showing bilateral partial eyelid ptosis in all patients. The course of the illness has been favorable. There was no thymoma.

Exercise in myasthenia gravis: A feasibility study of aerobic and resistance training

DEFF Research Database (Denmark)

Rahbek, Martin Amadeus; Mikkelsen, Erik Elgaard; Overgaard, Kristian

2017-01-01

Introduction: It has not been established whether progressive resistance training (PRT) and aerobic training (AT) are feasible and efficient in myasthenia gravis (MG). Methods: Fifteen subjects with generalized MG (Myasthenia Gravis Foundation of America (MGFA) clinical classification II-IV) were...... randomly assigned to 20 training sessions during 8 weeks of either PRT or AT. Feasibility was evaluated based on adherence, drop-out rate, adverse events, and Quantitative Myasthenia Gravis (QMG) score. Results: Twelve subjects (MGFA II, n = 11; MGFA III, n=1) completed the intervention with a mean...

Clinical Utility of Acetylcholine Receptor Antibody Testing in Ocular Myasthenia Gravis.

Science.gov (United States)

Peeler, Crandall E; De Lott, Lindsey B; Nagia, Lina; Lemos, Joao; Eggenberger, Eric R; Cornblath, Wayne T

2015-10-01

The sensitivity of acetylcholine receptor (AChR) antibody testing is thought to be lower in ocular myasthenia gravis (OMG) compared with generalized disease, although estimates in small-scale studies vary. There is little information in the literature about the implications of AChR antibody levels and progression from OMG to generalized myasthenia gravis. To test the hypothesis that serum AChR antibody testing is more sensitive in OMG than previously reported and to examine the association between AChR antibody levels and progression from OMG to generalized myasthenia gravis. A retrospective, observational cohort study was conducted of 223 patients (mean [SD] age, 59.2 [16.4] years; 139 [62.3%] male) diagnosed with OMG between July 1, 1986, and May 31, 2013, at 2 large, academic medical centers. Baseline characteristics, OMG symptoms, results of AChR antibody testing, and progression time to generalized myasthenia gravis (if this occurred) were recorded for each patient. Multiple logistic regression was used to measure the association between all clinical variables and antibody result. Kaplan-Meier survival analysis was performed to examine time to generalization. Among the 223 participants, AChR antibody testing results were positive in 158 participants (70.9%). In an adjusted model, increased age at diagnosis (odds ratio [OR], 1.03; 95% CI, 1.01-1.04; P = .007) and progression to generalized myasthenia gravis (OR, 2.92; 95% CI, 1.18-7.26; P = .02) were significantly associated with positive antibody test results. Women were less likely to have a positive antibody test result (OR, 0.36; 95% CI, 0.19-0.68; P = .002). Patients who developed symptoms of generalized myasthenia gravis had a significantly higher mean (SD) antibody level than those who did not develop symptoms of generalized myasthenia gravis (12.7 [16.5] nmol/L vs 4.2 [7.9] nmol/L; P = .002). We demonstrate a higher sensitivity of AChR antibody testing than previously reported in the

[Pharmacological treatment compliance and a description of its associated factors in patients with myasthenia gravis].

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Idiaquez, J F; Gonzalez, S; Lasso-Penafiel, J; Barnett, C

2018-01-01

Medication adherence is a public health problem and this has not been previously studied in myasthenia gravis patients. To determine if patients with myasthenia gravis are adherent to treatment and to describe the clinical factors of patients who are non-adherent to treatment. Cross-sectional study of patients with myasthenia gravis followed at Padre Hurtado Hospital, Santiago de Chile, who received their medication through the hospital and therefore were on the pharmacy's list. Patients' participation was voluntary and anonymous. Medication adherence was assessed with the Morisky-Green-Levine survey (4 items). Patients were assessed for myasthenia gravis severity with the Manual Muscle Test, and myasthenia gravis-related quality of life with the MG-QOL15. Finally, patients were screened for depression with the 12-Item General Health Questionnaire. 26 patients were enrolled and 15 (57.7%) were women. Only 10 (38.5%) of patients were adherent to treatment. Patients who were not adherent to medication had more weakness (p = 0.06), worse quality of life (p = 0.008), were taking a greater number of myasthenia gravis drugs (p = 0.003) and had a higher risk of depression (p = 0.03). In this cohort of myasthenia gravis patients, three out of five patients were not adherent to treatment. These patients tended to have more weakness, worse quality of life and higher risk of depression. Medication adherence should be assessed routinely in patients with myasthenia gravis.

Prognosis of thymectomy in myasthenia gravis patients with thymus hyperplasia.

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Yang, Jing; Liu, Chanchan; Li, Tao; Li, Chengyan

2017-09-01

To compare the post-thymectomy prognosis in different conditions of myasthenia gravis (MG) patients with thymus hyperplasia. Collecting medical record and carrying out the follow-up study of 123 myasthenia gravis patients with thymus hyperplasia who have underwent thymectomy during the period between 2003 and 2013. Dividing into different groups based on gender, age of onset, duration of disease and Myasthenia Gravis Association of America (MGFA) clinical classification to analyze different prognosis in different groups. Complete stable remission (CSR) was achieved in 71 of 123 patients (59.5%). There is no gender-related difference in achieving CSR. Patients with early onset of MG (≤40 years old) or disease duration less than 12 months had significantly higher CSR rates than those with late onset of MG (>40 years old) or disease duration more than 12 months respectively, while no difference was found in remission rate between MGFA clinical classification I and MGFA II. Myasthenia gravis patients with thymus hyperplasia who had thymectomy are proved to possess greater chance of achieving CSR. The onset age of disease and duration are the prognostic factors.

ACTOS DE HABLA EN RELATOS DE TRADICION ORAL*: Notas sobre un relato oral cusqueño

OpenAIRE

Espino Relucé, Gonzalo

2014-01-01

En este artículo se explora el comportamiento de los hablantes dela construcción de relatos orales a partir del texto Saqsaywaman, recogido por el autor en Cusco. Se propone una categoría operativa sobrelos actos del habla haciéndose lo propio con relatos orales. Se transcribe Saqsaywaman y se analiza el texto.

Reversible man-in-the-barrel syndrome in myasthenia gravis

OpenAIRE

Shah, Poornima A; Wadia, Pettarusp Murzban

2016-01-01

Man-in-the-barrel syndrome (MBS) is an uncommon presentation due to bilateral, predominantly proximal muscle weakness that has not been described to be associated with myasthenia gravis. We describe a case of myasthenia gravis presenting as MBS. Additionally, he had significant wasting of the deltoids bilaterally with fibrillations on electromyography (EMG) at rest and brief duration (3-6 ms) bi/triphasic motor unit potentials (MUPs) on submaximal effort apart from a decremental response on r...

Myasthenia Gravis: Drugs to be Avoided

Science.gov (United States)

... Many different drugs have been associated with worsening myasthenia gravis (MG). However, these drug associations do not ... review article for health professionals, found at the myasthenia.org website. For this app, we are only ...

Proofs concerning the existence, in the blood of hypertensive patients, of some serum factors influencing the vascular smooth muscle and the myocardium physiology.

Science.gov (United States)

Mocanu, M; Botea, S; Dragomir, C T

1991-01-01

Starting from the existence of some autoimmune diseases (i.e. bronchial asthma or miastenia gravis) we asked ourselves if some plasmatic factors do exist, influencing the receptor--mediator relations in cardiovascular system during some illnesses having unknown etiology, as arterial hypertension. For this reason, in this work was tested the hypothesis that, in some chronic cardiovascular diseases would exist factors circulating and affecting the functions of the cellular membranes of the arterial wall, particularly of the smooth muscle cells and myocardial cells. Our results show a significant modification of the calcium fluxes and of some neuromediators uptake at the hypertensive patients.

Myasthenia gravis: new developments in research and treatment.

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Evoli, Amelia

2017-10-01

Myasthenia gravis, a rare disorder of the neuromuscular transmission, is increasingly acknowledged as a syndrome more than as a single disease. This review summarizes recent advances in pathophysiology which confirm the disease heterogeneity, and may help find disease-targeted and patient-targeted therapies. Antibodies to the acetylcholine receptor, the muscle-specific tyrosine kinase and the lipoprotein receptor protein 4, characterize disease subtypes with distinct clinical traits and immune-pathogenic mechanisms. Genome-wide approaches have identified susceptibility loci within genes that participate in the immune response. Regulatory T and B cells appear to be defective in myasthenia gravis. In patients with acetylcholine receptor antibodies, thymectomy associated with prednisone proved more effective than prednisone alone in a multicenter randomized trial. New therapeutic options target B cells, B-cell growth factors and complement inhibition, and are currently reserved for patients with refractory disease. In the recent past, there has been an active search for new antigens in myasthenia gravis, whereas clinical and experimental studies have provided new insights of crucial pathways in immune regulation, which might become the targets of future therapeutic interventions.

Association of myasthenia gravis and Behçet's disease: A case report.

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Kisabay, Aysin; Sari, Ummu Serpil; Boyaci, Recep; Batum, Melike; Yilmaz, Hikmet; Selcuki, Deniz

2016-01-01

Myasthenia gravis is a disease of neuromuscular junction due to auto-immune destruction of the acetylcholine receptors. Behçet's disease, on the other hand, is a multisystemic vascular-inflammatory disease. Both conditions are not common in the general population although their association has not been reported in the literature. We wanted to present our patient who developed clinical course of myasthenia gravis following discontinuation of medications due to complications of corticosteroid for Behçet's disease. It was observed that clinical findings of myasthenia gravis recovered following restarting steroid treatment and he did not experience attacks of both conditions. Although Myasthenia gravis and Behçet's disease are distinct entities clinically as well as in terms of pathogenesis, they share common physiopathological features and their treatment is based on their common features. Copyright © 2016 Polish Neurological Society. Published by Elsevier Urban & Partner Sp. z o.o. All rights reserved.

Whole-exome sequencing reveals a rare interferon gamma receptor 1 mutation associated with myasthenia gravis.

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Qi, Guoyan; Liu, Peng; Gu, Shanshan; Yang, Hongxia; Dong, Huimin; Xue, Yinping

2018-04-01

Our study is aimed to explore the underlying genetic basis of myasthenia gravis. We collected a Chinese pedigree with myasthenia gravis, and whole-exome sequencing was performed on the two affected siblings and their parents. The candidate pathogenic gene was identified by bioinformatics filtering, which was further verified by Sanger sequencing. The homozygous mutation c.G40A (p.V14M) in interferon gamma receptor 1was identified. Moreover, the mutation was also detected in 3 cases of 44 sporadic myasthenia gravis patients. The p.V14M substitution in interferon gamma receptor 1 may affect the signal peptide function and the translocation on cell membrane, which could disrupt the binding of the ligand of interferon gamma and antibody production, contributing to myasthenia gravis susceptibility. We discovered that a rare variant c.G40A in interferon gamma receptor 1 potentially contributes to the myasthenia gravis pathogenesis. Further functional studies are needed to confirm the effect of the interferon gamma receptor 1 on the myasthenia gravis phenotype.

Cigarette Smoking and Activities of Daily Living in Ocular Myasthenia Gravis.

Science.gov (United States)

Gratton, Sean M; Herro, Angela M; Feuer, William J; Lam, Byron L

2016-03-01

Myasthenia gravis is an autoimmune disease of the neuromuscular junction, commonly affecting the ocular muscles. Cigarette smoking has been shown to influence many autoimmune diseases, including multiple sclerosis and rheumatoid arthritis, but its effect on myasthenia gravis has not been well studied. We sought to determine whether cigarette smoking influenced disease-related symptoms in ocular myasthenia gravis (OMG). We performed a prospective, clinic-based cross-sectional study in a single academic neuro-ophthalmology practice. All patients diagnosed with OMG between November 2006 and April 2014 were included. A prospective telephone survey was administered to determine smoking status and myasthenia gravis-related symptom severity. The main outcome measure was the myasthenia gravis-specific activities of daily living (MG-ADL) score, a well-validated marker of symptoms and quality of life in myasthenia gravis. Forty-four patients were included in the analysis. Comparison of MG-ADL ocular subscores between current smokers (3.4 ± 2.6), former smokers (1.8 ± 2.1), and never smokers (1.1 ± 1.5) revealed a statistically significant relationship (P = 0.031) where current smokers had the highest MG-ADL ocular subscores and never smokers the lowest. Comparison of MG-ADL total scores revealed the same relationship (current 5.6 ± 4.5, former 2.9 ± 3.1, never 1.4 ± 2.5, P = 0.003). There were borderline significant correlations of pack years with MG-ADL ocular subscore (r = 0.27, P = 0.074) and MG-ADL total score (r = 0.30, P = 0.051). Our findings indicate an association between cigarette smoking and symptom severity in OMG. This association suggests that smoking cessation in OMG patients may lead to improved symptom-related quality of life.

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

Science.gov (United States)

Horigome, H; Nomura, T; Saso, K; Joh, T; Ohara, H; Akita, S; Sobue, S; Mizuno, Y; Kato, Y; Itoh, M

2000-01-01

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.

[Thymectomy in myasthenia gravis: video-assisted procedures].

Science.gov (United States)

Tessitore, Adele; Vita, Maria Letizia; Cusumano, Giacomo; Congedo, Maria Teresa; Filotico, Mariella; Meacci, Elisa; Porziella, Venanzio; Margaritora, Stefano; Granone, Pierluigi

2007-01-01

We describe the technique, the benefits and the drawbacks of an original video-assisted thymectomy (VAT), performed through an inframammary cosmetic incision and median sternotomy in myasthenia gravis (MG) patients. This procedure is clinically valuable and cosmetically satisfactory so as to be very well accepted by patients, especially by young women. Minimal-access thymectomy has become increasingly popular as surgical treatment for patients with nonthymomatous myasthenia gravis because of its comparable efficacy, safety, and lesser degree of tissue trauma with conventional open surgery. We report a review/interview of 180 MG patients treated between 1993 and 2005. According to Myasthenia Gravis Foundation of America (MGFA), complete stable remission (CSR) and pharmacologic remission (PR) were calculated at the end of a minimal period of 12 months. A clinical remission was obtained in 41.1% (CR 27.8%, PR 13.3%), who had been followed for at least 12 months from surgery. 95% of these patients judged their cosmetic results to be excellent or good. Thymectomy in MG video-assisted infra-mammary cosmetic incision has shown to be a useful surgical approach as demonstrated by the good functional and very good aesthetic results, associated with a very low morbidity and no mortality.

Myasthenia Gravis Impairment Index: Responsiveness, meaningful change, and relative efficiency.

Science.gov (United States)

Barnett, Carolina; Bril, Vera; Kapral, Moira; Kulkarni, Abhaya V; Davis, Aileen M

2017-12-05

To study responsiveness and meaningful change of the Myasthenia Gravis Impairment Index (MGII) and its relative efficiency compared to other measures. We enrolled 95 patients receiving prednisone, IV immunoglobulin (IVIg), or plasma exchange (PLEX) and 54 controls. Patients were assessed with the MGII and other measures-including the Quantitative Myasthenia Gravis Score, Myasthenia Gravis Composite, and Myasthenia Gravis Activities of Daily Living-at baseline and 3-4 weeks after treatment. Statistical markers of responsiveness included between-groups and within-group differences, and we estimated the relative efficiency of the MGII compared to other measures. Patient-meaningful change was assessed with an anchor-based method, using the patient's impression of change. We determined the minimal detectable change (MDC) and the minimal important difference (MID) at the group and individual level. Treated patients had a higher change in MGII scores than controls (analysis of covariance p 1 favoring the MGII. The MGII demonstrated responsiveness to prednisone, IVIg, and PLEX in patients with myasthenia. There is a differential response in ocular and generalized symptoms to type of therapy. The MGII has higher relative efficiency than comparison measures and is viable for use in clinical trials. Copyright © 2017 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Academy of Neurology.

Avaliação clínica, endoscópica e manométrica da deglutição em pacientes com miastenia grave autoimune adquirida Clinical, endoscopical and manometric evaluation of swallowing in patients with myasthenia gravis

Directory of Open Access Journals (Sweden)

Adriana Leico Oda

2002-12-01

Full Text Available Apresentamos 22 pacientes com diagnóstico de miastenia grave autoimune adquirida (MGAA, com queixa de disfagia orofaríngea, avaliados pela análise fonoaudiológica, nasofibrolaringoscópica e manométrica de esfíncter esofágico superior, com o objetivo de avaliar as fases do processo de deglutição e os aspectos anatômicos e funcionais do sistema estomatognático. A idade dos pacientes variou de 19 a 74 anos; 19 (86,4% eram do sexo feminino e 3 (13,6% do masculino. Os principais achados foram: (1 correlação entre avaliação fonoaudiológica e nasofibrolaringoscópica; (2 alterações do sistema estomatognático, em 100% dos casos; (3 desordens da mastigação / deglutição presentes em 100% dos pacientes, à avaliação fonoaudiológica e em 81,8%, à nasofibrolaringoscópica; (4 associação entre penetração/aspiração e episódios de pneumonia; (5 comprometimento muscular do sistema estomatognático, acúmulo de resíduos, escape precoce do bolo para a faringe e episódios de penetração/aspiração apresentaram correlação nítida com diminuição da contração faríngea. Estes achados justificam a necessidade de avaliar as fases do processo de deglutição em pacientes com MGAA, com o objetivo de propiciar uma orientação adequada e prevenir episódios de pneumonia aspirativa, desnutrição e desidratação, devido à administração inadequada de alimentação via oral.Dysphagia, or difficulty swallowing, is a common problem following myasthenia gravis (MG and may lead to aspiration of saliva, food or liquids. We herein present 22 MG patients, with complaint of dysphagia, evaluated by phonoaudiological evaluation, nasofibrolaryngoscopical analysis and manometry of upper esophageal sphincter. The main objective was to evaluate the phases of the swallowing process and anatomical and functional aspects of oropharyngeal musculature. The age of patients varied from 19 to 74 years; being 19 female and 3 male. The main data were

Open source modular ptosis crutch for the treatment of myasthenia gravis.

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Saidi, Trust; Sivarasu, Sudesh; Douglas, Tania S

2018-02-01

Pharmacologic treatment of Myasthenia Gravis presents challenges due to poor tolerability in some patients. Conventional ptosis crutches have limitations such as interference with blinking which causes ocular surface drying, and frequent irritation of the eyes. To address this problem, a modular and adjustable ptosis crutch for elevating the upper eyelid in Myasthenia Gravis patients has been proposed as a non-surgical and low-cost solution. Areas covered: This paper reviews the literature on the challenges in the treatment of Myasthenia Gravis globally and focuses on a modular and adjustable ptosis crutch that has been developed by the Medical Device Laboratory at the University of Cape Town. Expert commentary: The new medical device has potential as a simple, effective and unobtrusive solution to elevate the drooping upper eyelid(s) above the visual axis without the need for medication and surgery. Access to the technology is provided through an open source platform which makes it available globally. Open access provides opportunities for further open innovation to address the current limitations of the device, ultimately for the benefit not only of people suffering from Myasthenia Gravis but also of those with ptosis from other aetiologies.

Hope, Coping, and Quality of Life in Adults with Myasthenia Gravis.

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Koopman, Wilma J; LeBlanc, Nicole; Fowler, Sue; Nicolle, Michael W; Hulley, Denise

2016-01-01

Myasthenia gravis significantly impacts quality of life. However, the relationship between hope, coping, and quality of life (QOL)in myasthenia patients has not been studied (Kulkantrakorn & Jarungkiatkul, 2009; Raggi et al., 2010). The aim of this study was to explore the relationship between hope, coping, and quality of life in adults with myasthenia gravis. Subjects with MG (n = 100) completed six questionnaires, including a demographic profile, the Myasthenia Gravis Activities of Daily Living Scale (MG-ADL), Herth Hope Index (HHI), Jalowiec Coping Scale (JCS), Myasthenia Gravis Quality-of-Life Scale (MG-QOL15), and Short Form Health Survey (SF-36v2). Mean hope scores indicated a high level of hope. An optimistic coping style was the most common and effective coping strategy identified by subjects. Positive thinking and humour were also frequently used strategies. Participants identified quality of life as good tolerability, above general population mental well-being, and below general populationphysical well-being. Participants who identified good quality of life had low scores on the MG-QOL15 scale and high scores on the SF36v2. Hope and independence for activities of daily living were found to correlate with improved quality of life and mental well-being(p life in the relationship between hope and coping. Hope and coping were not important factors for well-being or quality of life. Nurses caring for adults with myasthenia gravis should use interventions that continue to support hope, quality of life, and coping throughout the unpredictable and chronic course of MG.

Response to rocuronium and its determinants in patients with myasthenia gravis: A case-control study.

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Fujimoto, Masafumi; Terasaki, Shuhei; Nishi, Masaaki; Yamamoto, Tatsuo

2015-10-01

Several previous studies using univariate analysis have suggested that the pre-anaesthetic train-of-four (TOF) ratio, concentration of anti-acetylcholine receptor (AChR) antibodies and the presence of preoperative generalised muscular involvement are determinants of an increased response to neuromuscular blocking agents (NMBAs) in patients with myasthenia gravis. However, the determinants of the response of patients with myasthenia gravis to rocuronium, which is expected to be used more frequently since the advent of sugammadex, have not been studied. To clarify whether previously suggested determinants of the response to other intermediate-acting NMBAs would also affect the response to rocuronium and to reveal the determinants of the increased response to rocuronium in individual patients with myasthenia gravis using multivariate analysis. Case control study. Kumamoto University Hospital, November 2010 to September 2013. Thirty-eight patients with myasthenia gravis having surgery using a total intravenous anaesthetic technique were investigated. After induction of general anaesthesia, the 95% effective dose (ED95) of rocuronium was calculated using cumulative dose-finding methods. Neuromuscular function was monitored by acceleromyographic assessment of TOF responses of the adductor pollicis muscle to ulnar nerve stimulation. Patients were then divided into the increased response (ED95 rocuronium injection (baseline TOF ratio), concentration of anti-AChR antibodies, Osserman classification and treatment for myasthenia gravis in the two groups were compared. Stepwise logistic regression identified baseline TOF ratio and age of onset of myasthenia gravis as determinants of the increased response to rocuronium in patients with myasthenia gravis [odds ratios (95% confidence interval) of 0.87 (0.77 to 0.98; P = 0.02) and 0.92 (0.86 to 0.99; P = 0.03), respectively]. Multivariate analysis identified baseline TOF ratio and age of disease onset as determinants of

Refractory myasthenia gravis - clinical profile, comorbidities and response to rituximab.

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Sudulagunta, Sreenivasa Rao; Sepehrar, Mona; Sodalagunta, Mahesh Babu; Settikere Nataraju, Aravinda; Bangalore Raja, Shiva Kumar; Sathyanarayana, Deepak; Gummadi, Siddharth; Burra, Hemanth Kumar

2016-01-01

Introduction: Myasthenia gravis (MG) is an antibody mediated autoimmune neuromuscular disorder characterized by fatigable muscle weakness. A proportion of myasthenia gravis patients are classified as refractory due to non responsiveness to conventional treatment. This retrospective study was done to evaluate clinical profile, epidemiological, laboratory, and features of patients with MG and mode of management using rituximab and complications. Methods: Data of myasthenia gravis patients admitted or presented to outpatient department (previous medical records) with MG between January 2008 and January 2016 were included. A total of 512 patients fulfilled the clinical and diagnostic criteria of myasthenia gravis of which 76 patients met the diagnostic certainty for refractory myasthenia gravis and were evaluated. Results: Out of 76 refractory MG patients, 53 (69.73%) patients fulfilled all the three defined criteria. The median age of onset of the refractory MG group was 36 years with a range of 27-53 years. In our study 25 patients (32.89%) belonged to the age group of 21-30 years. Anti-MuSK antibodies were positive in 8 non-refractory MG patients (2.06%) and 36 refractory MG patients (47.36%). Mean HbA 1C was found to be 8.6±2.33. The dose of administered prednisone decreased by a mean of 59.7% ( p =3.3x10 -8 ) to 94.6% ( p =2.2x10 -14 ) after the third cycle of rituximab treatment. Conclusion: The refractory MG patients are most commonly female with an early age of onset, anti-MuSK antibodies, and thymomas. Refractory MG patients have higher prevalence and poor control (HbA 1C >8%) of diabetes mellitus and dyslipidemia probably due to increased steroid usage. Rituximab is very efficient in treatment of refractory MG with adverse effects being low.

Epidemiology of myasthenia gravis with anti-muscle specific kinase antibodies in the Netherlands

NARCIS (Netherlands)

Niks, Erik H.; Kuks, Jan B. M.; Verschuuren, Jan J. G. M.

The epidemiology of myasthenia gravis subtypes and the frequency of antibodies to muscle-specific kinase (MuSK) was studied in patients with generalised myasthenia gravis without anti-acetylcholine receptor antibodies who had an onset of symptoms between 1990 and 2004 in a well-defined region in the

Autoimmune polyglandular syndrome, type 2 associated with myasthenia gravis

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Pejin Radoslav

2012-01-01

Full Text Available Introduction. Autoimmune polyglandular syndrome type 2 is defined as adrenal insufficiency associated with autoimmune primary hypothyroidism and/or with autoimmune type 1 diabetes mellitus, but very rare with myasthenia gravis. Case report. We presented a case of an autoimmune polyglandular syndrome, type 2 associated with myasthenia gravis. A 49-year-old female with symptoms of muscle weakness and low serum levels of cortisol and aldosterone was already diagnosed with primary adrenal insufficiency. Primary hypothyroidism was identified with low values of free thyroxine 4 (FT4 and raised values of thyroidstumulating hormone (TSH. The immune system as a cause of hypothyroidism was confirmed by the presence of thyroid antibodies to peroxidase and TSH receptors. Myasthenia gravis was diagnosed on the basis of a typical clinical feature, positive diagnostic tests and an increased titre of antibodies against the acetylcholine receptors. It was not possible to confirm the immune nature of adrenal insufficiency by the presence of antibodies to 21- hydroxylase. The normal morphological finding of the adrenal glands was an indirect confirmation of the condition as well as the absence of other diseases that might have led to adrenal insufficiency and low levels of both serum cortisol and aldosterone. Hormone replacement therapy, anticholinergic therapy and corticosteroid therapy for myasthenia gravis improved the patient’s general state of health and muscle weakness. Conclusion. This case report indicates a need to examine each patient with an autoimmune disease carefully as this condition may be associated with another autoimmune diseases.

Controlled study of EMG activity of the jaw closers and openers during mastication in patients with myasthenia gravis

NARCIS (Netherlands)

Weijnen, FG; Bosman, F; van der Glas, HW; Kuks, JBM

Mastication was evaluated in patients with bulbar myasthenia gravis and compared with that of patients with ocular myasthenia gravis, patients in remission who previously suffered from bulbar symptoms, and healthy controls. Bulbar myasthenia gravis may impair mastication due to weakness of the

Netrin-1 receptor antibodies in thymoma-associated neuromyotonia with myasthenia gravis.

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Torres-Vega, Estefanía; Mancheño, Nuria; Cebrián-Silla, Arantxa; Herranz-Pérez, Vicente; Chumillas, María J; Moris, Germán; Joubert, Bastien; Honnorat, Jérôme; Sevilla, Teresa; Vílchez, Juan J; Dalmau, Josep; Graus, Francesc; García-Verdugo, José Manuel; Bataller, Luis

2017-03-28

To identify cell-surface antibodies in patients with neuromyotonia and to describe the main clinical implications. Sera of 3 patients with thymoma-associated neuromyotonia and myasthenia gravis were used to immunoprecipitate and characterize neuronal cell-surface antigens using reported techniques. The clinical significance of antibodies against precipitated proteins was assessed with sera of 98 patients (neuromyotonia 46, myasthenia gravis 52, thymoma 42; 33 of them with overlapping syndromes) and 219 controls (other neurologic diseases, cancer, and healthy volunteers). Immunoprecipitation studies identified 3 targets, including the Netrin-1 receptors DCC (deleted in colorectal carcinoma) and UNC5A (uncoordinated-5A) as well as Caspr2 (contactin-associated protein-like 2). Cell-based assays with these antigens showed that among the indicated patients, 9 had antibodies against Netrin-1 receptors (7 with additional Caspr2 antibodies) and 5 had isolated Caspr2 antibodies. Only one of the 219 controls had isolated Caspr2 antibodies with relapsing myelitis episodes. Among patients with neuromyotonia and/or myasthenia gravis, the presence of Netrin-1 receptor or Caspr2 antibodies predicted thymoma ( p myasthenia gravis, and neuromyotonia, often with Morvan syndrome ( p = 0.009). Expression of DCC, UNC5A, and Caspr2 proteins was demonstrated in paraffin-embedded thymoma samples (3) and normal thymus. Antibodies against Netrin-1 receptors (DCC and UNC5a) and Caspr2 often coexist and associate with thymoma in patients with neuromyotonia and myasthenia gravis. This study provides Class III evidence that antibodies against Netrin-1 receptors can identify patients with thymoma (sensitivity 21.4%, specificity 100%). © 2017 American Academy of Neurology.

Juvenile Myasthenia Gravis: A Paediatric Perspective

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Maria F. Finnis

2011-01-01

Full Text Available Myasthenia gravis (MG is an autoimmune disease in which antibodies are directed against the postsynaptic membrane of the neuromuscular junction, resulting in muscle weakness and fatigability. Juvenile myasthenia gravis (JMG is a rare condition of childhood and has many clinical features that are distinct from adult MG. Prepubertal children in particular have a higher prevalence of isolated ocular symptoms, lower frequency of acetylcholine receptor antibodies, and a higher probability of achieving remission. Diagnosis in young children can be complicated by the need to differentiate from congenital myasthenic syndromes, which do not have an autoimmune basis. Treatment commonly includes anticholinesterases, corticosteroids with or without steroid-sparing agents, and newer immune modulating agents. Plasma exchange and intravenous immunoglobulin (IVIG are effective in preparation for surgery and in treatment of myasthenic crisis. Thymectomy increases remission rates. Diagnosis and management of children with JMG should take account of their developmental needs, natural history of the condition, and side-effect profiles of treatment options.

Vegetables, Soups, Sauces, Gravies and Beverages.

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Marine Corps Inst., Washington, DC.

Developed as part of the Marine Corps Institute (MCI) correspondence training program, this course on vegetables, soups, sauces, gravies, and beverages is designed to increase Marine Corps cooks' effectiveness as food handlers, using the proper techniques in the preparation of these items. Introductory materials include specific information for…

Plasmapheresis in myasthenia gravis. A survey

NARCIS (Netherlands)

Kuks, JBM; Skallebaek, D

Plasmapheresis plays an important role in the acute management of patients with severe myasthenia gravis. Although plasmapheresis is now in use for more than 20 years, some controversies remain about the indication and the place in the therapy. It is generally found that the effect starts one week

Pyrosequencing analysis of the microbiota of kusaya gravy obtained from Izu Islands.

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Fujii, Tateo; Kyoui, Daisuke; Takahashi, Hajime; Kuda, Takashi; Kimura, Bon; Washizu, Yukio; Emoto, Eiji; Hiramoto, Tadahiro

2016-12-05

Kusaya is a salted, dried fish product traditionally produced on the Izu Islands in Japan. Fish are added to kusaya gravy repeatedly and intermittently, and used over several hundred years, which makes unique microbiota and unique flavors. In this study, we performed a metagenomic analysis to compare the composition of the microbiota of kusaya gravy between different islands. Twenty samples obtained from a total of 13 manufacturers on three islands (Hachijojima, Niijima, and Oshima Islands) were analyzed. The statistical analysis revealed that the microbiota in kusaya gravy maintain a stable composition regardless of the production steps, and that the microbiota are characteristic to the particular islands. The bacterial taxa common to all of the samples were not necessarily the dominant ones. On the other hand, the genera Halanaerobium and Tissierella were found to be characteristic to the microbiota of one or two islands. Because these genera are known to be present in the natural environment, it is likely that the bacterial strains peculiar to an island had colonized kusaya gravy for many years. The results of this study revealed an influence of geographical conditions on the microbiota in fermented food. Copyright © 2016 Elsevier B.V. All rights reserved.

A Genome-wide Association Study of Myasthenia Gravis

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Renton, Alan E.; Pliner, Hannah A.; Provenzano, Carlo; Evoli, Amelia; Ricciardi, Roberta; Nalls, Michael A.; Marangi, Giuseppe; Abramzon, Yevgeniya; Arepalli, Sampath; Chong, Sean; Hernandez, Dena G.; Johnson, Janel O.; Bartoccioni, Emanuela; Scuderi, Flavia; Maestri, Michelangelo; Raphael Gibbs, J.; Errichiello, Edoardo; Chiò, Adriano; Restagno, Gabriella; Sabatelli, Mario; Macek, Mark; Scholz, Sonja W.; Corse, Andrea; Chaudhry, Vinay; Benatar, Michael; Barohn, Richard J.; McVey, April; Pasnoor, Mamatha; Dimachkie, Mazen M.; Rowin, Julie; Kissel, John; Freimer, Miriam; Kaminski, Henry J.; Sanders, Donald B.; Lipscomb, Bernadette; Massey, Janice M.; Chopra, Manisha; Howard, James F.; Koopman, Wilma J.; Nicolle, Michael W.; Pascuzzi, Robert M.; Pestronk, Alan; Wulf, Charlie; Florence, Julaine; Blackmore, Derrick; Soloway, Aimee; Siddiqi, Zaeem; Muppidi, Srikanth; Wolfe, Gil; Richman, David; Mezei, Michelle M.; Jiwa, Theresa; Oger, Joel; Drachman, Daniel B.; Traynor, Bryan J.

2016-01-01

IMPORTANCE Myasthenia gravis is a chronic, autoimmune, neuromuscular disease characterized by fluctuating weakness of voluntary muscle groups. Although genetic factors are known to play a role in this neuroimmunological condition, the genetic etiology underlying myasthenia gravis is not well understood. OBJECTIVE To identify genetic variants that alter susceptibility to myasthenia gravis, we performed a genome-wide association study. DESIGN, SETTING, AND PARTICIPANTS DNA was obtained from 1032 white individuals from North America diagnosed as having acetylcholine receptor antibody–positive myasthenia gravis and 1998 race/ethnicity-matched control individuals from January 2010 to January 2011. These samples were genotyped on Illumina OmniExpress single-nucleotide polymorphism arrays. An independent cohort of 423 Italian cases and 467 Italian control individuals were used for replication. MAIN OUTCOMES AND MEASURES We calculated P values for association between 8114394 genotyped and imputed variants across the genome and risk for developing myasthenia gravis using logistic regression modeling. A threshold P value of 5.0 × 10−8 was set for genome-wide significance after Bonferroni correction for multiple testing. RESULTS In the over all case-control cohort, we identified association signals at CTLA4 (rs231770; P = 3.98 × 10−8; odds ratio, 1.37; 95% CI, 1.25–1.49), HLA-DQA1 (rs9271871; P = 1.08 × 10−8; odds ratio, 2.31; 95% CI, 2.02 – 2.60), and TNFRSF11A (rs4263037; P = 1.60 × 10−9; odds ratio, 1.41; 95% CI, 1.29–1.53). These findings replicated for CTLA4 and HLA-DQA1 in an independent cohort of Italian cases and control individuals. Further analysis revealed distinct, but overlapping, disease-associated loci for early- and late-onset forms of myasthenia gravis. In the late-onset cases, we identified 2 association peaks: one was located in TNFRSF11A (rs4263037; P = 1.32 × 10−12; odds ratio, 1.56; 95% CI, 1.44–1.68) and the other was detected

Relatos de pesquisas científicas

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Congresso Internacional de Dislexia

2011-05-01

Full Text Available Relatos de pesquisas científicas apresentadas na terceira edição do Congresso Internacional de Dislexia (CID, realizado nas dependências do Centro Universitário Metodista Izabela Hendrix, em maio de 2011. Tratam-se de relatos de resultados de pesquisas científicas, descritivas ou experimentais das áreas da Psicologia, Fonoaudiologia, Piscopedagogia, Pedagogia e Neurociências realizadas no contexto nacional e internacional em torno da temática dislexia. 

Malignant thymona with symptoms of myasthenia gravis

International Nuclear Information System (INIS)

Miarzynska, M.; Szlezak, L.; Fibak, J.; Wolski, M.; Lis-Podrzycka, E.; Miarzynski, K.

1994-01-01

The rare case of malignant tumor of thymus - Thymoma malignum was described. The initial diagnosis was difficult, because of the irregular symptoms of myasthenia gravis. The diagnostic difficulties, treatment and clinical features of this neoplasm were also discussed. (author)

Anti-MuSK-Positive Myasthenia Gravis in a Patient with Parkinsonism and Cognitive Impairment

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S. Lanfranconi

2011-01-01

Full Text Available Muscle-specific tyrosine kinase- (MuSK- antibodies-positive Myasthenia Gravis accounts for about one third of Seronegative Myasthenia Gravis and is clinically characterized by early onset of prominent bulbar, neck, shoulder girdle, and respiratory weakness. The response to medical therapy is generally poor. Here we report a case of late-onset MuSK-antibodies-positive Myasthenia Gravis presenting with signs of cognitive impairment and parkinsonism in addition to bulbar involvement and external ophthalmoplegia. The pattern of involvement of both peripheral and central nervous system dysfunction might suggest a common pathogenic mechanism, involving impaired cholinergic transmission.

Metaplastic thymoma with myasthenia gravis presumably caused by an accumulation of intratumoral immature T cells: a case report.

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Tajima, Shogo; Yanagiya, Masahiro; Sato, Masaaki; Nakajima, Jun; Fukayama, Masashi

2015-01-01

Among human neoplasms, thymomas are well known for their association with paraneoplastic autoimmune diseases such as myasthenia gravis. However, regarding rare metaplastic thymoma, only one case of an association with myasthenia gravis has been reported. Here, we present the second case of a 44-year-old woman with metaplastic thymoma associated with myasthenia gravis. In metaplastic thymoma, intratumoral terminal deoxynucleotidyl transferase-positive T-cells (immature T-cells) are generally scarce, while they were abundant in the present case. We believe that these immature T-cells could be related to the occurrence of myasthenia gravis.

Experience of thymectomy by median sternotomy in patients with myasthenia gravis

International Nuclear Information System (INIS)

Hussain, N.; Ahmed, S.W.; Ahmed, T.; Hafeez, A.B.; Baloch, R.; Ali, S.

2010-01-01

Objective: To determine the outcome of thymectomy in patients with myasthenia gravis and safety of median sternotomy approach. Methodology: An observational descriptive study was conducted in the department of thoracic surgery JPMC from February 2005 to January 2009. Twenty two patients having persistent generalized or ocular myasthenia gravis referred to our department by neurologists and general physicians, partially or not responding to medical treatment with or without thymoma, were included in the study. Those who were not fit for anaesthesia were excluded. Preoperatively 2 to 3 sessions of plasmaphaeresis were done and each patient was given anti myasthenia gravis treatment. Clinical staging was done by Modified Osserman classification. Median sternotomy approach was used. Outcome was assessed on the basis of remission of disease in different Osserman groups. All patients were followed for a minimum of 6 months. Results: Out of 22 patients, 16 (72.7%) were females and 6 (27.2%) males. Mean age at presentation was 35.2 +- 14.5 years. Mean duration of symptoms was 1.5 +- 1.2 years. A total of 4 (18.1%) patients with myasthenia gravis had thymoma and histopathology of 18 (81.9%) patients showed thymic hyperplasia. Remission was seen in most grades of Osserman. The best response was seen in Grade I where all patients achieved remission. Most patients in Grade II A and II B were benefited. The only patient in Grade III had no improvement of symptoms. No patient in Grade IV underwent thymectomy. Overall 86.3 % had a positive outcome on basis of remission and improvement. Conclusion: Thymectomy by median sternotomy is safe and effective with more favourable outcomes for patients of myasthenia gravis not responding to medical treatment. (author)

Refractory myasthenia gravis – clinical profile, comorbidities and response to rituximab

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Sudulagunta, Sreenivasa Rao; Sepehrar, Mona; Sodalagunta, Mahesh Babu; Settikere Nataraju, Aravinda; Bangalore Raja, Shiva Kumar; Sathyanarayana, Deepak; Gummadi, Siddharth; Burra, Hemanth Kumar

2016-01-01

Introduction: Myasthenia gravis (MG) is an antibody mediated autoimmune neuromuscular disorder characterized by fatigable muscle weakness. A proportion of myasthenia gravis patients are classified as refractory due to non responsiveness to conventional treatment. This retrospective study was done to evaluate clinical profile, epidemiological, laboratory, and features of patients with MG and mode of management using rituximab and complications. Methods: Data of myasthenia gravis patients admitted or presented to outpatient department (previous medical records) with MG between January 2008 and January 2016 were included. A total of 512 patients fulfilled the clinical and diagnostic criteria of myasthenia gravis of which 76 patients met the diagnostic certainty for refractory myasthenia gravis and were evaluated. Results: Out of 76 refractory MG patients, 53 (69.73%) patients fulfilled all the three defined criteria. The median age of onset of the refractory MG group was 36 years with a range of 27–53 years. In our study 25 patients (32.89%) belonged to the age group of 21–30 years. Anti-MuSK antibodies were positive in 8 non-refractory MG patients (2.06%) and 36 refractory MG patients (47.36%). Mean HbA1C was found to be 8.6±2.33. The dose of administered prednisone decreased by a mean of 59.7% (p=3.3x10–8) to 94.6% (p=2.2x10–14) after the third cycle of rituximab treatment. Conclusion: The refractory MG patients are most commonly female with an early age of onset, anti-MuSK antibodies, and thymomas. Refractory MG patients have higher prevalence and poor control (HbA1C >8%) of diabetes mellitus and dyslipidemia probably due to increased steroid usage. Rituximab is very efficient in treatment of refractory MG with adverse effects being low. PMID:27790079

Adhesive Ileus Complicating Recurrent Intestinal Pseudo-Obstruction in a Patient with Myasthenia Gravis

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Charalampos Seretis

2012-06-01

Full Text Available Intestinal pseudo-obstruction is considered to be one of the most frequent gastrointestinal manifestations of myasthenia gravis, accompanied by the presence of neoplasia of the thymus gland in the vast majority of the cases presented in the international literature. Despite the fact that myasthenia gravis has been implicated to be the cause of recurrent episodes of intestinal pseudo-obstruction, adhesive ileus has never been reported to complicate this – in any sense rare – condition. We present a unique case of a patient with myasthenia gravis, free of thymus neoplasia, who was submitted to emergency surgery due to the presence of extended adhesive ileus as a complication of chronic intestinal functional obstruction.

The diagnosis of thymoma and thymic atrophy in patients with myasthenia gravis

International Nuclear Information System (INIS)

Sund, K.K.; Skeie, G.O.; Gilhus, N.E.; Aarli, J.A.; Varhaug, J.E.

1997-01-01

The authors have compared clinical, immunological and radiological data in 20 patients with myasthenia gravis and thymoma and in 21 patients with myasthenia gravis and thymic atrophy. The median age at onset was 54 years in the thymoma group and 63 years in the thymic atrophy group. The severity of the disease was similar in the two groups, and there was no significant difference in the concentration of acetylcholine receptor antibodies. CA antibodies were demonstrated in 17/20 thymoma patients and in 6/21 with thymic atrophy, while 19/20 thymoma patients had antibodies to titin, compared with 9/21 among those with thymic atrophy. The diagnosis and treatment of patients with myasthenia gravis is based upon an evaluation of clinical, immunological and radiological data. 28 refs., 2 tabs

Síndrome de Usher asociado a miastenia gravis y esquizofrenia

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Santisteban Aguilera, Francisca; Gómez Álvarez, Francisco; Freyre Luque, Rásife; Gorguet Pi, Iliana; Moreno López, Lissett

2009-01-01

Se presenta el caso de un paciente atendido en el Hospital Provincial “Saturnino Lora” y el Centro de Retinosis Pigmentaria de Santiago de Cuba con características audiológicas, propias de una hipoacusia neurosensorial moderada, afecciones oftalmológicas, cansancio generalizado, disfagia, ptosis palpebral bilateral asimétrica con predominio en el lado izquierdo, afectación del oblicuo, del recto interno y ausencia del reflejo nauseoso, además de ideas delirantes y alucinaciones. Las alteracio...

Effect of marination in gravy on the radio frequency and microwave processing properties of beef

OpenAIRE

Basaran-Akgul, Nese; Rasco, Barbara A.

2013-01-01

Dielectric properties (the dielectric constant (ε′) and the dielectric loss factor (ε″)) and the penetration depth of raw eye of round beef Semitendinosus muscle, raw beef marinated in gravy, raw beef cooked in gravy, and gravy alone were determined as a function of the temperature (20–130 °C) and frequency (27–1,800 MHz). Both ε′ and ε″ values increased as the temperature increased at low frequencies (27 and 40 MHz). At high frequencies (915 and 1,800 MHz), ε′ showed a 50 % decrease while ε″...

Home sleep study for patients with myasthenia gravis.

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Yeh, J-H; Lin, C-M; Chiu, H-C; Bai, C-H

2015-09-01

The objective of the study was to examine predictors for sleep-disordered breathing (SDB) in patients with myasthenia gravis (MG) using Watch-PAT. We prospectively studied 58 consecutive patients with MG without respiratory symptoms for a full-night Watch-PAT with concomitant recording of the MG score and acetylcholine receptor antibody concentration and analyzed potential risk factors of SDB. Twenty-four patients (41%) had definitive SDB, which was mild in 12 patients, moderate in six, and severe in six. Assessing risk factors with multivariate models, we found four significant predictors (BMI, age, male gender, and use of azathioprine); BMI was the most powerful predictor. The severity and prevalence of sleep-disordered breathing had no significant association with MG score, myasthenia stage, or seropositivity of acetylcholine receptor antibody. The prevalence of SDB in myasthenic patients with mild and moderate weakness was high when using the Watch-PAT. Both myasthenia-specific factors (use of azathioprine) and general predictors in terms of BMI, age, and male gender predisposed the development of SDB in patients with myasthenia gravis. Careful screening of patients with myasthenia gravis at risk of SDB using Watch-PAT might improve the quality of sleep and cardiovascular health through proper treatment of underlying SDB. © 2015 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

A new approach to anesthesia management in myasthenia gravis: reversal of neuromuscular blockade by sugammadex.

NARCIS (Netherlands)

Boer, H.D. de; Egmond, J. van; Driessen, J.J.; Booij, L.H.D.J.

2010-01-01

A neuromuscular blocking drug (NMBD) induced neuromuscular blockade (NMB) in patients with myasthenia gravis usually dissipates either spontaneously or by administration of neostigmine. We administered sugammadex to a patient with myasthenia gravis to reverse a rocuronium-induced profound NMB. NMBDs

Association of sarcoidosis and myasthenia gravis: Case Report ...

African Journals Online (AJOL)

Whereas the coexistence of different autoimmune or rheumatologic diseases with myasthenia gravis (MG) is well documented, its combination with sarcoidosis is extremely rare. Presented here is an interesting case with coexisting MG and sarcoidosis.

Infantile Onset Myasthenia Gravis with MuSK Antibodies

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J Gordon Millichap

2006-08-01

Full Text Available A late infantile case of myasthenia gravis (MG with anti-muscle-specific receptor tyrosine kinase (MuSK high antibody (Ab titer is reported from Kyushu University, Fukuoka; and Nagasaki University, Japan.

Steroids induce acetylcholine receptors on cultured human muscle: Implications for myasthenia gravis

International Nuclear Information System (INIS)

Kaplan, I.; Blakely, B.T.; Pavlath, G.K.; Travis, M.; Blau, H.M.

1990-01-01

Antibodies to the acetylcholine receptor (AChR), which are diagnostic of the human autoimmune disease myasthenia gravis, block AChR function and increase the rate of AChR degradation leading to impaired neuromuscular transmission. Steroids are frequently used to alleviate symptoms of muscle fatigue and weakness in patients with myasthenia gravis because of their well-documented immunosuppressive effects. The authors show here that the steroid dexamethasone significantly increases total surface AChRs on cultured human muscle exposed to myasthenia gravis sera. The results suggest that the clinical improvement observed in myasthenic patients treated with steroids is due not only to an effect on the immune system but also a direct effect on muscle. They propose that the identification and development of pharmacologic agents that augment receptors and other proteins that are reduced by human genetic or autoimmune disease will have broad therapeutic applications

Steroids induce acetylcholine receptors on cultured human muscle: Implications for myasthenia gravis

Energy Technology Data Exchange (ETDEWEB)

Kaplan, I.; Blakely, B.T.; Pavlath, G.K.; Travis, M.; Blau, H.M. (Stanford Univ. School of Medicine, CA (USA))

1990-10-01

Antibodies to the acetylcholine receptor (AChR), which are diagnostic of the human autoimmune disease myasthenia gravis, block AChR function and increase the rate of AChR degradation leading to impaired neuromuscular transmission. Steroids are frequently used to alleviate symptoms of muscle fatigue and weakness in patients with myasthenia gravis because of their well-documented immunosuppressive effects. The authors show here that the steroid dexamethasone significantly increases total surface AChRs on cultured human muscle exposed to myasthenia gravis sera. The results suggest that the clinical improvement observed in myasthenic patients treated with steroids is due not only to an effect on the immune system but also a direct effect on muscle. They propose that the identification and development of pharmacologic agents that augment receptors and other proteins that are reduced by human genetic or autoimmune disease will have broad therapeutic applications.

Difference in distribution of muscle weakness between myasthenia gravis and the Lambert-Eaton myasthenic syndrome

NARCIS (Netherlands)

Wirtz, P. W.; Sotodeh, M.; Nijnuis, M.; van Doorn, P. A.; van Engelen, B. G. M.; Hintzen, R. Q.; de Kort, P. L. M.; Kuks, J. B.; Twijnstra, A.; de Visser, M.; Visser, L. H.; Wokke, J. H.; Wintzen, A. R.; Verschuuren, J. J.

2002-01-01

Background: Myasthenia gravis and the Lambert-Eaton myasthenic syndrome (LEMS) may have a similar distribution of muscle weakness. Deciding on a diagnosis of myasthenia gravis or LEMS on clinical grounds may therefore be difficult. Objective: To compare the localisation of initial muscle weakness

Safety and efficacy of eculizumab in anti-acetylcholine receptor antibody-positive refractory generalised myasthenia gravis (REGAIN)

DEFF Research Database (Denmark)

Howard, James F; Utsugisawa, Kimiaki; Benatar, Michael

2017-01-01

BACKGROUND: Complement is likely to have a role in refractory generalised myasthenia gravis, but no approved therapies specifically target this system. Results from a phase 2 study suggested that eculizumab, a terminal complement inhibitor, produced clinically meaningful improvements in patients...... with anti-acetylcholine receptor antibody-positive refractory generalised myasthenia gravis. We further assessed the efficacy and safety of eculizumab in this patient population in a phase 3 trial. METHODS: We did a phase 3, randomised, double-blind, placebo-controlled, multicentre study (REGAIN) in 76...... hospitals and specialised clinics in 17 countries across North America, Latin America, Europe, and Asia. Eligible patients were aged at least 18 years, with a Myasthenia Gravis-Activities of Daily Living (MG-ADL) score of 6 or more, Myasthenia Gravis Foundation of America (MGFA) class II-IV disease...

Mutagenicity of pan residues and gravy from fried meat.

Science.gov (United States)

Overvik, E; Nilsson, L; Fredholm, L; Levin, O; Nord, C E; Gustafsson, J A

1987-02-01

Lean pork meat was fried with or without the addition of frying-fat at 200 or 250 degrees C. The pan residues were collected by washing the hot pan with boiling water. When producing thickened gravy the water was substituted by a mixture of water and flour, milk and flour or cream and flour. The basic extracts were tested for mutagenicity in Ames' Salmonella test on strain TA98 with the addition of S9 mix. High amounts of mutagenicity were found in all samples. The amounts of mutagenicity in the pan residues were at a comparable level of the amounts found in the meat crusts. Thickening of the gravy caused only small changes in the mutagenicity.

Survivin as a potential mediator to support autoreactive cell survival in myasthenia gravis: a human and animal model study.

Directory of Open Access Journals (Sweden)

Linda L Kusner

Full Text Available The mechanisms that underlie the development and maintenance of autoimmunity in myasthenia gravis are poorly understood. In this investigation, we evaluate the role of survivin, a member of the inhibitor of apoptosis protein family, in humans and in two animal models. We identified survivin expression in cells with B lymphocyte and plasma cells markers, and in the thymuses of patients with myasthenia gravis. A portion of survivin-expressing cells specifically bound a peptide derived from the alpha subunit of acetylcholine receptor indicating that they recognize the peptide. Thymuses of patients with myasthenia gravis had large numbers of survivin-positive cells with fewer cells in the thymuses of corticosteroid-treated patients. Application of a survivin vaccination strategy in mouse and rat models of myasthenia gravis demonstrated improved motor assessment, a reduction in acetylcholine receptor specific autoantibodies, and a retention of acetylcholine receptor at the neuromuscular junction, associated with marked reduction of survivin-expressing circulating CD20+ cells. These data strongly suggest that survivin expression in cells with lymphocyte and plasma cell markers occurs in patients with myasthenia gravis and in two animal models of myasthenia gravis. Survivin expression may be part of a mechanism that inhibits the apoptosis of autoreactive B cells in myasthenia gravis and other autoimmune disorders.

On the generalized gravi-magnetic hypothesis

International Nuclear Information System (INIS)

Massa, C.

1989-01-01

According to a generalization of the gravi-magnetic hypothesis (GMH) any neutral mass moving in a curvilinear path with respect to an inertial frame creates a magnetic field, dependent on the curvature radius of the path. A simple astrophysical consequence of the generalized GMH is suggested considering the special cases of binary pulsars and binary neutron stars

Thymectomy is a beneficial therapy for patients with non-thymomatous ocular myasthenia gravis: a systematic review and meta-analysis.

Science.gov (United States)

Zhu, Kai; Li, Jiaoxing; Huang, Xin; Xu, Wei; Liu, Weibin; Chen, Jiaxin; Chen, Pei; Feng, Huiyu

2017-10-01

Ocular myasthenia gravis, an autoimmune disease, is characterized by extraocular muscle weakness. Myasthenia gravis is closely associated with the functional status of the thymus gland. The efficacy of thymectomy for non-thymomatous ocular myasthenia gravis remains controversial. Here, we present the first systematic review and meta-analysis of studies assessing the outcome of thymectomy in patients with non-thymomatous ocular myasthenia gravis and found that the pooled rate of complete stable remission was 0.5074 with considerable heterogeneity. Furthermore, subgroup analysis showed that the efficacy of thymectomy differed according to geographical location. Furthermore, thymectomy outcomes are better in children than they are in adults. Thymectomy clearly represents an effective treatment for patients with non-thymomatous ocular myasthenia gravis. However, more multicenter, randomized, controlled clinical trials are now required to confirm these conclusions.

Suppression of CHRN endocytosis by carbonic anhydrase CAR3 in the pathogenesis of myasthenia gravis.

Science.gov (United States)

Du, Ailian; Huang, Shiqian; Zhao, Xiaonan; Feng, Kuan; Zhang, Shuangyan; Huang, Jiefang; Miao, Xiang; Baggi, Fulvio; Ostrom, Rennolds S; Zhang, Yanyun; Chen, Xiangjun; Xu, Congfeng

2017-01-01

Myasthenia gravis is an autoimmune disorder of the neuromuscular junction manifested as fatigable muscle weakness, which is typically caused by pathogenic autoantibodies against postsynaptic CHRN/AChR (cholinergic receptor nicotinic) in the endplate of skeletal muscle. Our previous studies have identified CA3 (carbonic anhydrase 3) as a specific protein insufficient in skeletal muscle from myasthenia gravis patients. In this study, we investigated the underlying mechanism of how CA3 insufficiency might contribute to myasthenia gravis. Using an experimental autoimmune myasthenia gravis animal model and the skeletal muscle cell C2C12, we find that inhibition of CAR3 (the mouse homolog of CA3) promotes CHRN internalization via a lipid raft-mediated pathway, leading to accelerated degradation of postsynaptic CHRN. Activation of CAR3 reduces CHRN degradation by suppressing receptor endocytosis. CAR3 exerts this effect by suppressing chaperone-assisted selective autophagy via interaction with BAG3 (BCL2-associated athanogene 3) and by dampening endoplasmic reticulum stress. Collectively, our study illustrates that skeletal muscle cell CAR3 is critical for CHRN homeostasis in the neuromuscular junction, and its deficiency leads to accelerated degradation of CHRN and development of myasthenia gravis, potentially revealing a novel therapeutic approach for this disorder.

Evento com aparente risco de morte recorrente como manifestação inicial de síndrome miastênica congênita Evento con aparente riesgo de muerte recurrente como manifestación inicial de síndrome miasténico congénito Recurrent apparent life-threatening event as the first manifestation of congenital myasthenia

Directory of Open Access Journals (Sweden)

Mariana Tresoldi das N. Romaneli

2013-03-01

Full Text Available OBJETIVO: Alertar os pediatras sobre a necessidade de investigar criteriosamente a etiologia de eventos com aparente risco de morte recorrente. Não foram encontrados relatos associando tais eventos à miastenia congênita. DESCRIÇÃO DO CASO: Lactente de sete meses apresentando história de eventos com aparente risco de morte recorrente foi internado para investigação. Durante a internação, apresentou cianose e dispneia progressiva, com necessidade de ventilação mecânica por três dias. Após a melhora clínica, e tendo sido descartadas as hipóteses de doença do refluxo gastroesofágico e aspiração pulmonar como desencadeantes, notou-se ptose palpebral bilateral, hipotonia apendicular e choro fraco, que conduziram à suspeita clínica de miastenia congênita. Após confirmação do diagnóstico, foi mantido tratamento ambulatorial com piridostigmina, com recuperação nutricional e neurológica, sem novos eventos com aparente risco de morte nos três anos seguintes. COMENTÁRIOS: A investigação minuciosa das causas de eventos com aparente risco de morte pode levar a diagnósticos menos frequentes que exigem tratamento específico, como a miastenia congênita.OBJETIVO: Alertar a los pediatras sobre la necesidad de investigar criteriosamente la etiología de eventos con aparente riesgo de muerte recurrente. No se encontraron relatos asociando tales eventos a la miastenia congénita. DESCRIPCIÓN DEL CASO: Lactante de siete meses presentando historia de eventos con aparente riesgo de muerte recurrente fue internado para investigación. Durante la internación, presentó cianosis y disnea progresiva, con necesidad de ventilación mecánica por tres días. Después de la mejora clínica, y habiendo sido rechazadas las hipótesis de enfermedad del reflujo gastroesofágico y aspiración pulmonar como desencadenantes, se notó ptosis palpebral bilateral, hipotonía apendicular y lloro débil, que condujeron a la sospecha clínica de

Myasthenia Gravis: a population-based epidemiological study.

LENUS (Irish Health Repository)

Rutledge, S

2016-02-01

Myasthenia Gravis (MG) is a disorder affecting components of the neuromuscular junction. Epidemiological studies show rising incidence and prevalence rates. The aim of this study was to determine the incidence and prevalence of MG in the Republic of Ireland. Data sources included patient lists from consultant neurologists and ophthalmologists, a neuroimmunology laboratory, general practitioners and the Myasthenia Gravis Association. A total of 1,715 cases were identified, of which 706 definite, probable or possible autoimmune and congenital MG cases were included. The overall prevalence rate from the data obtained is 15.38\\/100,000. The study demonstrated a female preponderance (female:male of 1.3: 1) and some geographical variation within Ireland. The average incidence rate for the years 2000 to 2009 was 11.3 per year; the rate for the current decade is 18 per year. The increasing number of diagnoses may be due to improved access to diagnostic investigations and increasing awareness of the clinical manifestations.

Acetylcholine receptor binding antibody-associated myasthenia gravis and rhabdomyolysis induced by nivolumab in a patient with melanoma.

Science.gov (United States)

Shirai, Takushi; Sano, Tasuku; Kamijo, Fuminao; Saito, Nana; Miyake, Tomomi; Kodaira, Minori; Katoh, Nagaaki; Nishie, Kenichi; Okuyama, Ryuhei; Uhara, Hisashi

2016-01-01

We reported an 81-year-old woman with metastatic melanoma, in whom myasthenia gravis and rhabdomyolysis developed after nivolumab monotherapy. The first symptom of myasthenia gravis was dyspnea. Ultrasonography detected hypokinesis of the bilateral diaphragm suggesting myasthenia gravis, although there was no abnormal finding of the lungs in computed tomography images. Acetylcholine receptor binding antibodies were low-titer positive in the preserved serum before administration of nivolumab, strongly suggesting that the myasthenia gravis was a nivolumab-related immune adverse event. Despite the remarkable clinical benefits of immune checkpoint inhibitors for patients with advanced melanoma, it is important to recognize unexpected immune-related adverse events. © The Author 2015. Published by Oxford University Press. All rights reserved. For Permissions, please email: journals.permissions@oup.com.

Vocal cord palsy: An uncommon presenting feature of myasthenia gravis

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Sethi Prahlad

2011-01-01

Full Text Available Vocal cord palsy can have myriad causes. Unilateral vocal cord palsy is common and frequently asymptomatic. Trauma, head, neck and mediastinal tumors as well as cerebrovascular accidents have been implicated in causing unilateral vocal cord palsy. Viral neuronitis accounts for most idiopathic cases. Bilateral vocal cord palsy, on the other hand, is much less common and is a potentially life-threatening condition. Myasthenia gravis, an autoimmune disorder caused by antibodies targeting the post-synaptic acetylcholine receptor, has been infrequently implicated in its causation. We report here a case of bilateral vocal cord palsy developing in a 68-year-old man with no prior history of myasthenia gravis 2 months after he was operated on for diverticulitis of the large intestine. Delay in considering the diagnosis led to endotracheal intubation and prolonged mechanical ventilation with attendant complications. Our case adds to the existing literature implicating myasthenia gravis as an infrequent cause of bilateral vocal cord palsy. Our case is unusual as, in our patient, acute-onset respiratory distress and stridor due to bilateral vocal cord palsy was the first manifestation of a myasthenic syndrome.

Factors affecting outcome in ocular myasthenia gravis.

Science.gov (United States)

Mazzoli, Marco; Ariatti, Alessandra; Valzania, Franco; Kaleci, Shaniko; Tondelli, Manuela; Nichelli, Paolo F; Galassi, Giuliana

2018-01-01

50%-60% of patients with ocular myasthenia gravis (OMG) progress to generalized myasthenia gravis (GMG) within two years. The aim of our study was to explore factors affecting prognosis of OMG and to test the predictive role of several independent clinical variables. We reviewed a cohort of 168 Caucasian patients followed from September 2000 to January 2016. Several independent variables were considered as prognostic factors: gender, age of onset, results on electrophysiological tests, presence and level of antibodies against acetylcholine receptors (AChR Abs), treatments, thymic abnormalities. The primary outcome was the progression to GMG and/or the presence of bulbar symptoms. Secondary outcomes were either achievement of sustained minimal manifestation status or worsening in ocular quantitative MG subscore (O-QMGS) or worsening in total QMG score (T-QMGS), assessed by Myasthenia Gravis Foundation of America (MGFA) quantitative scores. Changes in mental and physical subscores of health-related quality of life (HRQoL) were assessed with SF-36 questionnaire. Variance analysis was used to interpret the differences between AChR Ab titers at different times of follow up among the generalized and non-generalized patients. Conversion to GMG occurred in 18.4% of patients; it was significantly associated with sex, later onset of disease and anti-AChR Ab positivity. Antibody titer above the mean value of 25.8 pmol/mL showed no significant effect on generalization. Sex and late onset of disease significantly affected T-QMGS worsening. None of the other independent variables significantly affected O-QMGS and HRQoL. Sex, later onset and anti-AChR Ab positivity were significantly associated with clinical worsening.

PHYSICOCHEMICAL PROPERTIES OF INSTANT PUMPKIN JAVANESE NOODLE GRAVY [Sifat Fisikokimia Saus Bubuk Mi Jawa Instan dari Labu Kering

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Yusnita Hamzah*

2012-12-01

Full Text Available This research was carried out to study the physical and chemical characteristics of pumpkin Javanese noodle gravy prepared in powder form. The finished product is categorized as an instant product as it only needs to be rehydrated with warm water (60°C before consumption. Five gravy formulations were developed with the incorporation of two types of flour (tapioca and corn flours as the thickening agent. Physical characteristics including colour and swelling properties of the final products were evaluated. For chemical analysis, ash, moisture, protein, crude fiber, fat and carbohydrate compositions were also determined. For physical analysis, all formulations showed similar colour appearance of the gravy powder including lightness (L, redness (a* and yellowness (b*. Pertaining swelling properties of the dried gravy, the water absorption index (WAI values were low (2.0–3.0g/g, while the water solubility index (WSI were moderate (33.0–50.0% when evaluated at both hot (85°C and cold (25°C water. In termn of chemical compositions, carbohydrate and fat content increased and decreased, respectively with the addition of tapioca and corn flours. The moisture content of the instant gravy increased with the increased of corn flour addition. There were differences in fiber and ash content in all formulations, but no specific trend was observed. Finally, protein content remained the same in all formulations. This current finding provided information of the gravy powder produced. Further analysis on the characteristics of the rehydrated pumpkin Javanese noodle gravy will provide a more complete picture of the finished product.

RI scintigraphy in myasthenia gravis

International Nuclear Information System (INIS)

Kuroda, Yoshikazu; Miyamoto, Mariko; Maki, Masako; Yamazaki, Toshiro.

1982-01-01

35 cases of myasthenia gravis were studied with RI scintigraphy. 67 Ga-citrate was used in 34 patients and 76 Se-selenomethionine in 5 patients for thymus scintigraphy. RI scintigram was negative in non-tumorous thymus, regardless of the severity of illness and it was positive in seven of nine patients with thymomas. RI accumulation in thymus was found both in benign and malignant thymomas. RI seems to accumulated in lymphocytic and mixed thymus more than epithelial type. (author)

An occasional diagnosis of myasthenia gravis - a focus on thymus during cardiac surgery: a case report

Directory of Open Access Journals (Sweden)

Dainese Luca

2009-10-01

Full Text Available Abstract Background Myasthenia gravis, an uncommon autoimmune syndrome, is commonly associated with thymus abnormalities. Thymomatous myasthenia gravis is considered to have worst prognosis and thymectomy can reverse symptoms if precociously performed. Case report We describe a case of a patient who underwent mitral valve repair and was found to have an occasional thymomatous mass during the surgery. A total thymectomy was performed concomitantly to the mitral valve repair. Conclusion The diagnosis of thymomatous myasthenia gravis was confirmed postoperatively. Following the surgery this patient was strictly monitored and at 1-year follow-up a complete stable remission had been successfully achieved.

[Value of the albumin to globulin ratio in predicting severity and prognosis in myasthenia gravis patients].

Science.gov (United States)

Yang, D H; Su, Z Q; Chen, Y; Chen, Z B; Ding, Z N; Weng, Y Y; Li, J; Li, X; Tong, Q L; Han, Y X; Zhang, X

2016-03-08

To assess the predictive value of the albumin to globulin ratio (AGR) in evaluation of disease severity and prognosis in myasthenia gravis patients. A total of 135 myasthenia gravis (MG) patients were enrolled between February 2009 and March 2015. The AGR was detected on the first day of hospitalization and ranked from lowest to highest, and the patients were divided into three equal tertiles according to the AGR values, which were T1 (AGR 1.53). The Kaplan-Meier curve was used to evaluate the prognostic value of AGR. Cox model analysis was used to evaluate the relevant factors. Multivariate Logistic regression analysis was used to find the predictors of myasthenia crisis during hospitalization. The median length of hospital stay for each tertile was: for the T1 21 days (15-35.5), T2 18 days (14-27.5), and T3 16 days (12-22.5) (Pmyasthenia gravis. At the multivariate Cox regression analysis, the AGR (Pmyasthenia gravis patients. Respectively, the hazard ratio (HR) were 4.655 (95% CI: 2.355-9.202) and 0.596 (95% CI: 0.492-0.723). Multivariate Logistic regression analysis showed the AGR (Pmyasthenia crisis. The AGR may represent a simple, potentially useful predictive biomarker for evaluating the disease severity and prognosis of patients with myasthenia gravis.

Characterization of the key aroma compounds in beef and pork vegetable gravies á la chef by application of the aroma extract dilution analysis.

Science.gov (United States)

Christlbauer, Monika; Schieberle, Peter

2009-10-14

By application of the aroma extract dilution analysis (AEDA) on an aroma distillate isolated from a freshly prepared, stewed beef/vegetable gravy, 52 odor-active compounds were detected in the flavor dilution (FD) factor range of 4-4096. On the basis of high FD factors in combination with the results of the identification experiments, 3-(methylthio)propanal (cooked potato), 3-mercapto-2-methylpentan-1-ol (gravy-like), (E,E)-2,4-decadienal (deep-fried, fatty), 3-hydroxy-4,5-dimethyl-2(5H)-furanone (lovage-like), vanillin (vanilla-like), (E,E)-2,4-nonadienal (deep-fried), and (E)-2-undecenal (metallic) are suggested as key contributors to the aroma of the gravy. To get an insight into the role of the vegetables as sources of gravy odorants, a beef gravy was prepared without vegetables. The AEDA results revealed that, in particular, onions and leek are important sources of gravy aroma compounds, adding particularly the very potent, gravy-like smelling 3-mercapto-2-methylpentan-1-ol to the overall aroma profile. Further compounds that were clearly derived from the vegetables and, thus, are important modifiers of the overall aroma were 4-vinyl-2-methoxyphenol, (E)-beta-damascenone, beta-ionone, 2-isopropyl-3-methoxypyrazine, and 2-(sec-butyl)-3-methoxypyrazine. Interestingly, none of the key odorants detected in the gravy can be assumed to be formed from a reaction between beef and vegetable constituents. A comparison of the odorants in the beef/vegetable gravy with a gravy prepared according to the same procedure, but substituting beef by pork meat, indicated that most of the aroma compounds were identical-although different in FD factors-but the tallowy smelling 12-methyltridecanal was detected as key odorant only in the beef/vegetable gravy.

Effects of napping on neuromuscular fatigue in myasthenia gravis.

Science.gov (United States)

Kassardjian, Charles D; Murray, Brian J; Kokokyi, Seint; Jewell, Dana; Barnett, Carolina; Bril, Vera; Katzberg, Hans D

2013-11-01

The relationship between sleep and neuromuscular fatigue is understood poorly. The goal of this study was to evaluate the effects of napping on quantitative measures of neuromuscular fatigue in patients with myasthenia gravis (MG). Eight patients with mild to moderate MG were recruited. Patients underwent maintenance of wakefulness tests (MWT) and multiple sleep latency tests (MSLT). The Quantitative Myasthenia Gravis Score (QMGS) was measured before nap and after each nap to examine the effects of napping and sleep on neuromuscular weakness. Results showed that QMGS improves only after naps where patients slept more than 5 min but not where patients did not sleep or slept less than 5 min. Daytime napping mitigates neuromuscular fatigue in patients with MG, especially if patients slept for more than 5 min. Copyright © 2013 Wiley Periodicals, Inc.

Acquired generalised neuromyotonia, cutaneous lupus erythematosus and alopecia areata in a patient with myasthenia gravis.

LENUS (Irish Health Repository)

O'Sullivan, S S

2012-02-03

We describe a patient with the diagnoses of acquired neuromyotonia, cutaneous lupus erythematosus and alopecia areata, occurring many years after a thymectomy for myasthenia gravis associated with a thymoma. We review the current literature on autoimmune conditions associated with myasthenia gravis and thymectomy. To our knowledge, this combination of multiple autoimmune conditions has not been reported previously.

RI scintigraphy in myasthenia gravis

Energy Technology Data Exchange (ETDEWEB)

Kuroda, Yoshikazu; Miyamoto, Mariko (Tokyo Metropolitan Fuchu Hospital (Japan)); Maki, Masako; Yamazaki, Toshiro

1982-10-01

35 cases of myasthenia gravis were studied with RI scintigraphy. /sup 67/Ga-citrate was used in 34 patients and /sup 76/Se-selenomethionine in 5 patients for thymus scintigraphy. RI scintigram was negative in non-tumorous thymus, regardless of the severity of illness and it was positive in seven of nine patients with thymomas. RI accumulation in thymus was found both in benign and malignant thymomas. RI seems to accumulated in lymphocytic and mixed thymus more than epithelial type.

neuromuscular disease mimicking myasthenia gravis in a nigerian

African Journals Online (AJOL)

cardiomyopathy, may occur3,4 and cardiac and respiratory involvement have been documented as indices of worse ... The heart is not involved in myasthenia gravis and electrocardiographic findings remain normal .... transmission and of Motor neurons. In: Berhman. RE, Kliegman RM, Jenson HB eds. Nelson extbook of ...

The Anti-Acetylcholine Receptor Antibody Test in Suspected Ocular Myasthenia Gravis

Directory of Open Access Journals (Sweden)

Jung Jin Lee

2014-01-01

Full Text Available Aim. To estimate the clinical significance of anti-acetylcholine receptor antibody (anti-AChR-Ab levels in suspected ocular myasthenia gravis. Methods. In total, 144 patients complaining of fluctuating diplopia and ptosis were evaluated for serum levels of anti-acetylcholine receptor antibody and their medical charts were retrospectively reviewed. Subjects were classified into three groups: variable diplopia only, ptosis only, and both variable diplopia and ptosis. We investigated serum anti-AChR-Ab titer levels and performed thyroid autoantibody tests. Results. Patients’ chief complaints were diplopia (N=103, ptosis (N=12, and their concurrence (N=29. Abnormal anti-AChR-Ab was observed in 21 of 144 patients (14.1%. Between the three groups, mean age, number of seropositive patients, and mean anti-AChR-Ab level were not significantly different (P=0.224, 0.073, and 0.062, resp.. Overall, 27.5% of patients had abnormal thyroid autoantibodies. Conclusion. The sensitivity of anti-AChR-Ab was 14.1% in suspected ocular myasthenia gravis and seropositivity in myasthenia gravis patients showed a high correlation with the presence of thyroid autoantibodies.

Value of radiologic imaging of the thymus gland in myasthenia gravis

International Nuclear Information System (INIS)

Glathe, S.; Neufang, K.F.R.; Haupt, F.W.

1989-01-01

Radiologic imaging in myasthenia gravis is used for the evaluation of pathologic changes of the thymus gland. Computed tomography can demonstrate tumors of the anterior mediastinum in nearly 90% and is therefore superior to conventional radiography.Because of the variety of size and shape of the normal thymus gland, differentiation between normal thymus, follicular hyperplasia and thymoma is rarely possible especially in younger patients. In elderly patients with myasthenia gravis and involution of the thymus gland tumors of the thymus are reliably detected by computed tomography, whereas the ability of computed tomography to predict the histological diagnosis is poor even with intravenous administration of contrast media. (orig.) [de

A conceptual framework for evaluating impairments in myasthenia gravis.

Science.gov (United States)

Barnett, Carolina; Bril, Vera; Kapral, Moira; Kulkarni, Abhaya; Davis, Aileen M

2014-01-01

Myasthenia gravis is characterized by weakness and fatigability of different muscle groups, including ocular, bulbar and the limbs. Therefore, a measure of disease severity at the impairment level in myasthenia needs to reflect all the relevant impairments, as well as their variations with activity and fatigue. We conducted a qualitative study of patients with myasthenia, to explore their experiences and related impairments, aimed at developing a conceptual framework of disease severity at the impairment level in myasthenia gravis. Twenty patients representing the spectrum of disease participated in semi-structured interviews. Interviews were recorded and the transcripts were analyzed by content analysis using an inductive approach with line-by-line open coding. Themes were generated from these codes. Two main themes were identified: the severity of the impairments and fatigability (i.e., triggering or worsening of an impairment with activity). The impairments were further classified within body regions (ocular, bulbar and axial/limbs). Fatigability was described as a phenomenon affecting the whole body but also affecting specific impairments, and was associated with fluctuation of the symptoms. Patients were concerned that clinical examination at a single point in time might not reflect their true clinical state due to fatigability and fluctuations in severity. This conceptual framework reflects the relevance of both severity and fatigability in understanding impairment-based disease severity in myasthenia. This framework could inform the development of impairment measures in myasthenia gravis.

A conceptual framework for evaluating impairments in myasthenia gravis.

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Carolina Barnett

Full Text Available BACKGROUND: Myasthenia gravis is characterized by weakness and fatigability of different muscle groups, including ocular, bulbar and the limbs. Therefore, a measure of disease severity at the impairment level in myasthenia needs to reflect all the relevant impairments, as well as their variations with activity and fatigue. We conducted a qualitative study of patients with myasthenia, to explore their experiences and related impairments, aimed at developing a conceptual framework of disease severity at the impairment level in myasthenia gravis. METHODS: Twenty patients representing the spectrum of disease participated in semi-structured interviews. Interviews were recorded and the transcripts were analyzed by content analysis using an inductive approach with line-by-line open coding. Themes were generated from these codes. RESULTS: Two main themes were identified: the severity of the impairments and fatigability (i.e., triggering or worsening of an impairment with activity. The impairments were further classified within body regions (ocular, bulbar and axial/limbs. Fatigability was described as a phenomenon affecting the whole body but also affecting specific impairments, and was associated with fluctuation of the symptoms. Patients were concerned that clinical examination at a single point in time might not reflect their true clinical state due to fatigability and fluctuations in severity. CONCLUSIONS: This conceptual framework reflects the relevance of both severity and fatigability in understanding impairment-based disease severity in myasthenia. This framework could inform the development of impairment measures in myasthenia gravis.

Onset and Evolution of Clinically Apparent Myasthenia Gravis After Resection of Non-myasthenic Thymomas.

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Mineo, Tommaso Claudio; Tamburrini, Alessandro; Schillaci, Orazio; Ambrogi, Vincenzo

2018-03-06

Patients with thymoma and without clinical or electromyographical myasthenic signs may occasionally develop myasthenia several years after thymectomy. Hereby, we investigated the predictors and the evolution of this peculiar disease. We performed a retrospective analysis in 104 consecutive patients who underwent thymectomy between 1987 and 2013 for thymoma without clinical or electromyographic signs of myasthenia gravis. Predictors of post-thymectomy onset of myasthenia gravis were investigated with univariate time-to-disease analysis. Evolution of myasthenia was analyzed with time-to-regression analysis. Eight patients developed late myasthenia gravis after a median period of 33 months from thymectomy. No significant correlation was found for age, gender, Masaoka's stage, and World Health Organization histology. Only high preoperative serum acetylcholine-receptor antibodies titer (>0.3 nmol/L) was significantly associated with post-thymectomy myasthenia gravis at univariate time-to-disease (P = 0.003) analysis. Positron emission tomography was always performed in high-titer patients, and increased metabolic activity was detected in 4 of these patients. Surgical treatment through redo-sternotomy or video-assisted thoracoscopy was performed in these last cases with a remission in all patients after 12, 24, 32 and 48 months, respectively. No patient under medical treatment has yet developed a complete remission. In our study the presence of preoperative high-level serum acetylcholine receptor antibodies was the only factor significantly associated with the development of post-thymectomy myasthenia gravis. The persistence of residual islet of ectopic thymic tissue was one of the causes of the onset of myasthenia and its surgical removal was successful. Copyright © 2018 Elsevier Inc. All rights reserved.

QMG and MG-ADL correlations: Study of eculizumab treatment of myasthenia gravis.

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Howard, James F; Freimer, Miriam; O'Brien, Fanny; Wang, Jing Jing; Collins, Stephen R; Kissel, John T

2017-08-01

A phase 2 study of eculizumab for treating myasthenia gravis (MG) used the quantitative myasthenia gravis score (QMG) and myasthenia gravis activities of daily living profile (MG-ADL) to evaluate baseline disease severity and treatment response. Correlations were then analyzed between these assessments. Patients were given eculizumab or placebo during the first 16-week treatment period of the crossover study, with treatment assignments reversed for the second treatment period following a 5-week washout. QMG and MG-ADL scores at baseline and endpoint of each treatment period generated correlation coefficients for baseline status and treatment response during eculizumab therapy. Correlation strength between QMG and MG-ADL scores was higher for treatment response (R = 0.726; 95% confidence interval, 0.264-0.907; P = 0.0036) than for assessing baseline disease status (R = 0.552; 95% confidence interval, -0.022-0.839; P = 0.0495). MG-ADL may be more sensitive for assessing treatment response than point-in-time disease status. Muscle Nerve 56: 328-330, 2017. © 2016 Wiley Periodicals, Inc.

Exercise in myasthenia gravis: A feasibility study of aerobic and resistance training.

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Rahbek, Martin Amadeus; Mikkelsen, Erik Elgaard; Overgaard, Kristian; Vinge, Lotte; Andersen, Henning; Dalgas, Ulrik

2017-10-01

It has not been established whether progressive resistance training (PRT) and aerobic training (AT) are feasible and efficient in myasthenia gravis (MG). Fifteen subjects with generalized MG (Myasthenia Gravis Foundation of America (MGFA) clinical classification II-IV) were randomly assigned to 20 training sessions during 8 weeks of either PRT or AT. Feasibility was evaluated based on adherence, drop-out rate, adverse events, and Quantitative Myasthenia Gravis (QMG) score. Twelve subjects (MGFA II, n = 11; MGFA III, n=1) completed the intervention with a mean adherence of 95 % ± 8. One dropout (PRT) could potentially be related to PRT. Both groups reported adverse events, including bulbar symptoms (n = 2) and increased fatigue (n = 3), but no change in QMG score was observed in either group. The PRT group showed increases in maximal strength and functional capacity. Eight weeks of moderate to high intensity AT and PRT were feasible for most patients with mild MG. Maximal strength and functional capacity increased in the PRT group. Muscle Nerve 56: 700-709, 2017. © 2017 Wiley Periodicals, Inc.

Valence of acetylcholine-receptor-antibody-titers in myasthenia gravis

International Nuclear Information System (INIS)

Zeitlhofer, J.; Maida, E.M.; Mamoli, B.; Mayr, N.

1986-01-01

In a retrospective study in 47 patients with myasthenia gravis acetylcholine-receptor-antibody-titers (AChR-AB) were correlated with the severity of the disease. In 18 patients the course of titers was studied and two groups of patients could be differentiated: patients with relative constant and patients with fluctuating titers. Age, age of begin of myasthenia and sex did not influence the titers. Also the duration of the disease and the severity of symptoms did not influence the level of AChR-AB-titers. In this retrospective study the influence of immunsuppressive therapy on the intra-individual course of AB-titers and their correlation with the clinical symptoms could not be judged. Measurement of AChR-AB is of value for the diagnosis of myasthenia gravis and important for judging the clinical course and the effect of therapy. (Author)

Juvenile Myasthenia Gravis in Korea: Subgroup Analysis According to Sex and Onset Age.

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Lee, Ha Neul; Kang, Hoon-Chul; Lee, Joon Soo; Kim, Heung Dong; Shin, Ha Young; Kim, Seung Min; Sunwoo, Il Nam; Lee, Young-Mock

2016-12-01

Juvenile myasthenia gravis presents before 18 years of age with different characteristics according to racial background and pubertal development. The authors aimed to determine the clinical characteristics of children and adolescents of Korean ethnicity with myasthenia gravis, and evaluate the presentation and clinical outcomes according to the sex and onset age of the patients. The authors recruited 88 Korean juvenile myasthenia gravis patients between September 2005 and August 2015. Worse clinical severity from presentation, more aggressive treatment strategies, and worse final treatment outcomes were noted in girls with postpubertal onset than in the other patients. The symptoms were milder (pure ocular presentation in 96.6% [85/88]) and the disease course was more benign (94.3% [83/88]) in this study than in the literature. The homogenous racial background might have contributed to these results. These findings highlight the influence of pubertal development and the need for timely and appropriate active treatment, including thymectomy, to improve prognosis. © The Author(s) 2016.

Effect of marination in gravy on the radio frequency and microwave processing properties of beef.

Science.gov (United States)

Basaran-Akgul, Nese; Rasco, Barbara A

2015-02-01

Dielectric properties (the dielectric constant (ε') and the dielectric loss factor (ε″)) and the penetration depth of raw eye of round beef Semitendinosus muscle, raw beef marinated in gravy, raw beef cooked in gravy, and gravy alone were determined as a function of the temperature (20-130 °C) and frequency (27-1,800 MHz). Both ε' and ε″ values increased as the temperature increased at low frequencies (27 and 40 MHz). At high frequencies (915 and 1,800 MHz), ε' showed a 50 % decrease while ε″ increased nearly three fold with increasing temperature in the range from 20 to 130 °C. ε' increased gradually while ε″ increased five fold when the temperature increased from 20 to 130 °C. Both ε' and ε″ of all samples decreased with increase in frequency. Marinating the beef in gravy dramatically increased the ε″ values, particularly at the lower frequencies. Power penetration depth of all samples decreased with increase temperature and frequency. These results are expected to provide useful data for modeling dielectric heating processes of marinated muscle food.

Intensive Care Management of Myasthenia Gravis After Thymectomy ...

African Journals Online (AJOL)

Aim:To evaluate the management of post thymectomy myasthenia gravis (MG) patient in our centre; to highlight those aspects of patient care that could improve outcome and to serve as a bench mark for developing a standardized protocol for management. Method: A retrospective study of 5 cases of post thymectomy MG ...

Is thymectomy in non-thymomatous myasthenia gravis of any benefit?

Science.gov (United States)

Diaz, Andres; Black, Edward; Dunning, Joel

2014-03-01

A best evidence topic in thoracic surgery was written according to a structured protocol. The question addressed was if thymectomy in non-thymomatous myasthenia gravis was of any benefit? Overall, 137 papers were found using the reported search, of which 16 represented the best evidence to answer the clinical question. The authors, journal, date and country of publication, patient group studied, study type, relevant outcomes and results of these papers are tabulated. The outcome variables were similar in all of the papers, including complete stable remission (CSR), pharmacological remission, age at presentation, gender, duration of symptoms, preoperative classification (Oosterhius, Osserman or myasthenia gravis Foundation of America (MGFA)), thymic pathology, preoperative medications (steroids, immunosuppressants), mortality and morbidity. We conclude that evidence-based reviews have shown that relative rates of thymectomy patients compared with non-thymectomy patients attaining outcome indicate that the former group of patients is more likely to achieve medication-free remission, become asymptomatic and clinically improve (54%, P benefit rate (BR), P benefits has not been established due to factors such as the confounding differences between myasthenia gravis patients receiving and not receiving thymectomy, the non-randomized nature of class II studies and the lack of Class I evidence to support its use. There is currently a randomized trial ongoing looking at thymectomy by sternotomy vs controls and the results are eagerly awaited.

Inferior oblique muscle paresis as a sign of myasthenia gravis.

Science.gov (United States)

Almog, Yehoshua; Ben-David, Merav; Nemet, Arie Y

2016-03-01

Myasthenia gravis may affect any of the six extra-ocular muscles, masquerading as any type of ocular motor pathology. The frequency of involvement of each muscle is not well established in the medical literature. This study was designed to determine whether a specific muscle or combination of muscles tends to be predominantly affected. This retrospective review included 30 patients with a clinical diagnosis of myasthenia gravis who had extra-ocular muscle involvement with diplopia at presentation. The diagnosis was confirmed by at least one of the following tests: Tensilon test, acetylcholine receptor antibodies, thymoma on chest CT scan, or suggestive electromyography. Frequency of involvement of each muscle in this cohort was inferior oblique 19 (63.3%), lateral rectus nine (30%), superior rectus four (13.3%), inferior rectus six (20%), medial rectus four (13.3%), and superior oblique three (10%). The inferior oblique was involved more often than any other muscle (pmyasthenia gravis can be difficult, because the disease may mimic every pupil-sparing pattern of ocular misalignment. In addition diplopia caused by paresis of the inferior oblique muscle is rarely encountered (other than as a part of oculomotor nerve palsy). Hence, when a patient presents with vertical diplopia resulting from an isolated inferior oblique palsy, myasthenic etiology should be highly suspected. Copyright © 2015 Elsevier Ltd. All rights reserved.

Miastenia grave: avaliação clínica de 153 pacientes Myasthenia gravis: clinical evaluation of 153 patients

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FRANCISCO MARCOS B. CUNHA

1999-06-01

Full Text Available São analisados, retrospectivamente, os prontuários dos doentes miastênicos diagnosticados e seguidos no período de fevereiro de 1973 a março de l995. Os principais achados clínicos foram ptose palpebral, diplopia, disfagia, disfonia ou disatria, dificuldade de mastigação, dispnéia, astenia, paresia da musculutura cervical e das extremidades. A partir das informações coletadas, os pacientes foram classificados clinicamente pela escala de Osserman-Genkins modificada, mas considerando o aspecto não dinâmico dessa escala, adotou-se a escala funcional de Niakan modificada, na qual os doentes são colocados nas seguintes condições: remissão, controlado, compensado, controle parcial, controle precário, sem resposta. Na casuística foram estudados 153 pacientes: 104 (68,0% do sexo feminino e 49 (32,0% do sexo masculino, numa proporção de 2,1:1. O tempo de doença variou de sete dias a 27 anos, com média de 6,26 anos (± 5,44. A idade dos primeiros sintomas variou entre 24 horas a 80 anos, com média de idade de 32,13 anos (±19,48. Até os 15 anos foram observados 30 doentes; entre 15 e 50 anos, 91 doentes; com idade acima de 50 anos, 32 doentes; após os 60 anos, a doença tem nítido predomínio entre os homens (1,5:1. Na amostra estudada, a forma auto-imunoadquirida foi a mais frequente, com comprometimento muscular generalizado, porém o envolvimento da musculatura ocular com ptose e diplopia se constituiu nas manifestações clínicas mais frequentes.We have retrospectively analysed the records of patients diagnosed as having myasthenia gravis and followed up in our departament from February 1973 to March 1995. The main clinical findings were ptosis, diplopia, dysphagia, disphonia or dysarthria, mastigatory impairment, dyspnea, asthenia, weakness of the cervical muscles and of the extremities, as well as findings of the physical and neurological examination. Based on the information collected, the patients were classified

Psychiatric disorders in myasthenia gravis

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Mariana Inés Ybarra

2011-04-01

Full Text Available OBJECTIVE: To investigate the prevalence of psychiatric disorders in patients with myasthenia gravis (MG. METHOD: Forty-one patients with MG answered to a structured psychiatric interview (MINI-Plus. RESULTS: Eleven (26.1% patients were diagnosed with a depressive disorder and 19 (46.3% were diagnosed with an anxiety disorder. Patients with dysthymia were older (p=0.029 and had longer disease duration (p=0.006. Patients with social phobia also had longer disease duration (p=0.039. CONCLUSION: Psychiatric disorders in MG are common, especially depressive and anxiety disorders.

Von Hippel-Lindau disease associated with myasthenia gravis not related to thymoma

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Paolo Pozzato

2013-04-01

Full Text Available BACKGROUND Von Hippel-Lindau disease (VHL is a rare autosomal dominant inherited disorder characterized by an increased risk of tumours in a number of locations (eyes, brain, adrenal gland, pancreas, liver, kidneys, or other areas of the body. It is caused by germline mutation in the VHL gene. The VHL gene is a tumour suppressor gene that has been identified on the short arm of chromosome 3. CASE REPORT We report a case of a 60 year-old female with the clinical diagnosis of VHL type 1 (cerebellar haemangioblastoma, pancreatic cysts with subsequent steatorrhoea, and bilateral renal carcinoma who developed weakness and fatigability of skeletal muscles, left lid ptosis, snarling expression and nasal timbre speech. Acetylcholine receptor antibodies were negative in serum, while the electrodiagnostic test demonstrated an alteration of neuromuscolar junction which was consistent with the diagnosis of myasthenia gravis. Contrast-enhanced TC scan of the anterior mediastinum was performed, which excluded thymus enlargement. VHL gene evaluation in this patient identified a new mutation (c279delC9 and polymorphism c291C>G. At present the patient still suffers from ataxia and dysmetria due to cerebellar involvement in VHL, while fatigue and lid ptosis improved after the treatment with oral pyridostigmine 60 mg tid. DISCUSSION AND CONCLUSIONS To our knowledge this is the first report of a case of VHL associated with myasthenia gravis without thymoma. A case of VHL associated with a form of myasthenia gravis related to thymoma has been recently reported. In our case the absence of acetylcholine receptor antibodies may suggest a genetic origin also for the myasthenia gravis.

Clinical Profile and Outcome of Postthymectomy versus Non-Thymectomy Myasthenia Gravis Patients in the Philippine General Hospital: A 6-Year Retrospective Study.

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De Roxas, Ranhel C; Bagnas, Marjorie Anne C; Baldonado, Jobelle Joyce Anne R; Rivera, Jonathan P; Roxas, Artemio A

2016-01-01

Myasthenia gravis is an autoimmune neuromuscular disorder characterized by the production of abnormal autoantibodies directed against the receptors present in the neuromuscular junction. It has been the standard practice to offer thymectomy in all generalized myasthenia gravis patients despite the lack of robust evidence. The objectives of this study are to describe the clinical profile and differentiate the clinical outcomes of thymectomy versus non-thymectomy and thymomatous versus non-thymomatous myasthenia gravis patients in the Philippine General Hospital. Between 2009 and 2014, a total of 69 postthymectomy and 16 non-thymectomy patient records were successfully retrieved. The demographic characteristics, surgical approach, and histopathologic results were obtained. The clinical outcome after 6 months or 1 year-follow-up was also determined and grouped according to the following: (1) complete remission, (2) pharmacological remission, (3) no clinical change, (4) worsening symptoms, and (5) mortality. Majority of the patients were females (68.0%) with a mean age of 39.8 years and a mean duration of myasthenic symptoms of 21 months. Using the Myasthenia Gravis Foundation of America classification, 54.1% of patients fell under Class II and 48.2% of them presented with generalized weakness. In this study, 60.8% of postthymectomy myasthenia gravis patients had either complete remission or pharmacologic remission compared with 12.5% among non-thymectomy patients (p-value myasthenia gravis after thymectomy (p-value = 0.29). This study showed that both thymomatous and non-thymomatous myasthenia gravis patients who underwent thymectomy had a higher incidence of complete stable remission and pharmacologic remission as compared with myasthenia gravis patients who did not undergo thymectomy.

Myasthenia gravis and preeclampsia: Dot all the I's and cross all the T's

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Ali Hassan, FCPS

2017-10-01

Full Text Available Although rare, the co-occurrence of myasthenia gravis and preeclampsia during pregnancy is responsible for considerable maternal and foetal morbidity and mortality. Both careful selection of medications and a multidisciplinary approach are required for treating such cases. This study presents a case report of a patient with a known history of generalized myasthenia gravis who presented with preeclampsia at 33 weeks' gestation. Subsequently, the patient developed recurrent seizures that necessitated the use of multiple medications, including phenytoin, valproic acid, levetiracetam, and propofol. Magnesium sulphate was not administered due to its blocking effect on calcium channels at the neuromuscular junction. The patient underwent a caesarean section under spinal anaesthesia and gave birth to a baby with intrauterine growth restriction (IUGR. Blood pressure control was achieved with the administration of methyldopa and parenteral hydralazine, an increased dose of pyridostigmine, and intravenous immunoglobulin therapy. The status of the patient's myasthenia gravis remained stable. This case serves to highlight the conflicts in the management of these two disorders and suggests strategies to resolve these conflicts in clinical management.

Relato autobiográfico e identidad personal: Un modelo de análisis narrativo

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Dante Duero

2007-05-01

Full Text Available En el siguiente trabajo analizamos cómo las estrategias narrativas que las personas emplean para construir relatos autobiográficos condiciona sus formas de pensar acerca de sí mismas, el mundo y sus propias acciones. Hemos partido del supuesto de que la organización y la coherencia de esta clase de relatos constituyen un aspecto clave para entender el problema de la identidad personal. Analizamos los relatos autobiográficos de dos muestras de estudiantes universitarios, pertenecientes a la Universidad Nacional de Córdoba (Argentina a la Universidad Autónoma de México (México. Nos hemos propuesto, en primer lugar, describir y analizar los diferentes relatos autobiográficos que obtuvimos en ambas muestras a través de entrevistas en profundidad. A través de dicho análisis, hemos procurado caracterizar el proceso de construcción narrativa, estableciendo la estructura y las funciones que los elementos del relato cumplen en su organización, contribuyendo a su coherencia y composición. Teniendo en cuenta esto, y teniendo en cuenta los datos de ambos grupos, intentamos, por una parte, identificar diferentes tipos de relatos. Simultáneamente, analizamos cómo las características de las historias condicionaba el modo en que las personas construyen su propia identidad, en tanto personajes de tales relatos.

Effects of nisin and temperature on survival, growth, and enterotoxin production characteristics of psychrotrophic Bacillus cereus in beef gravy.

Science.gov (United States)

Beuchat, L R; Clavero, M R; Jaquette, C B

1997-05-01

The presence of psychrotrophic enterotoxigenic Bacillus cereus in ready-to-serve meats and meat products that have not been subjected to sterilization treatment is a public health concern. A study was undertaken to determine the survival, growth, and diarrheal enterotoxin production characteristics of four strains of psychrotrophic B. cereus in brain heart infusion (BHI) broth and beef gravy as affected by temperature and supplementation with nisin. A portion of unheated vegetative cells from 24-h BHI broth cultures was sensitive to nisin as evidenced by an inability to form colonies on BHI agar containing 10 micrograms of nisin/ml. Heat-stressed cells exhibited increased sensitivity to nisin. At concentrations as low as 1 microgram/ml, nisin was lethal to B. cereus, the effect being more pronounced in BHI broth than in beef gravy. The inhibitory effect of nisin (1 microgram/ml) was greater on vegetative cells than on spores inoculated into beef gravy and was more pronounced at 8 degrees C than at 15 degrees C. Nisin, at a concentration of 5 or 50 micrograms/ml, inhibited growth in gravy inoculated with vegetative cells and stored at 8 or 15 degrees C, respectively, for 14 days. Growth of vegetative cells and spores of B. cereus after an initial period of inhibition is attributed to loss of activity of nisin. One of two test strains produced diarrheal enterotoxin in gravy stored at 8 or 15 degrees C within 9 or 3 days, respectively. Enterotoxin production was inhibited in gravy supplemented with 1 microgram of nisin/ml and stored at 8 degrees C for 14 days; 5 micrograms of nisin/ml was required for inhibition at 15 degrees C. Enterotoxin was not detected in gravy in which less than 5.85 log10 CFU of B. cereus/ml had grown. Results indicate that as little as 1 microgram of nisin/ml may be effective in inhibiting or retarding growth of and diarrheal enterotoxin production by vegetative cells and spores of psychrotrophic B. cereus in beef gravy at 8 degrees C, a

Auto-relato e relato de informante secundário na avaliação da saúde em idosos

OpenAIRE

Jardim, Renata; Barreto, Sandhi Maria; Giatti, Luana

2010-01-01

OBJETIVO: Analisar se o modelo explicativo para a avaliação da saúde do idoso com base no auto-relato é comparável com o modelo de relato do informante secundário e se a auto-avaliação de saúde do informante secundário influencia a avaliação da saúde do idoso. MÉTODOS: Estudo transversal com 230 pares idoso-informante secundário realizado em Belo Horizonte, MG, em 2007. Foram investigadas variáveis sociodemográficas e de saúde dos idosos por meio de entrevista estruturada. Utilizou-se regress...

Repercusión perinatal de los trastornos inmunológicos del embarazo Perintal effect of immunological disorders during pregnancy

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Rafael J. Manotas Cabarcas

1993-03-01

Full Text Available

En esta revisión se analizan varios trastornos de índole inmunológica, comprobada o supuesta, que ocurren durante el embarazo, a saber: hipertensión, diabetes mellitus, hipertiroidismo, hipotiroidismo, trombocitopenia aloinmune y autoinmune, lupus eritematoso sistémico y miastenia gravis. En cada caso se describen la patogénesis y los efectos perinatales y se hacen consideraciones diagnósticas y terapéuticas para la orientación del obstetra y del pediatra.

Several disorders of proven or supposed immunological nature, that occur during pregnancy are described, namely: arterial hypertension, diabetes mellitus, hyperthyroidism, hypothyroidism, alloimmune and autoimmune thrombocytopenia, erythematous systemic lupus and myastenia gravis. The pathogenesis and perinatal effects of each one are described and diagnostic and therapeutic considerations are made for orientation of obstetricians and pediatricians.

Ocular myasthenia gravis: A review

Science.gov (United States)

Nair, Akshay Gopinathan; Patil-Chhablani, Preeti; Venkatramani, Devendra V; Gandhi, Rashmin Anilkumar

2014-01-01

Myasthenia gravis (MG) is a disease that affects the neuro-muscular junction resulting in classical symptoms of variable muscle weakness and fatigability. It is called the great masquerader owing to its varied clinical presentations. Very often, a patient of MG may present to the ophthalmologist given that a large proportion of patients with systemic myasthenia have ocular involvement either at presentation or during the later course of the disease. The treatment of ocular MG involves both the neurologist and ophthalmologist. Thus, the aim of this review was to highlight the current diagnosis, investigations, and treatment of ocular MG. PMID:25449931

Incidence of seropositive myasthenia gravis in Cape Town and ...

African Journals Online (AJOL)

Background. Myasthenia gravis (MG) is a treatable autoimmune disease characterised by fatiguable weakness of skeletal muscles. More than 85% of MG patients have antibodies to the acetylcholine receptor (AChR) at the neuromuscular junction or are seropositive for MG (SPMG). In the developed world the incidence of ...

Myasthenia gravis exacerbation and diarrhea associated with erythromycin treatment

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Sora Yasri

2017-01-01

Full Text Available An important problem in management of the case with myasthenia gravis (MG is the control of exacerbation. There are several possible causes of exacerbation of MG including the use of drug. Here, the authors report a case of MG exacerbation and diarrhea associated with erythromycin treatment.

Detection of myasthenia gravis using electrooculography signals.

Science.gov (United States)

Liang, T; Boulos, M I; Murray, B J; Krishnan, S; Katzberg, H; Umapathy, K

2016-08-01

Myasthenia gravis (MG) is an autoimmune neuromuscular disorder resulting from skeletal muscle weakness and fatigue. An early common symptom is fatigable weakness of the extrinsic ocular muscles; if symptoms remain confined to the ocular muscles after a few years, this is classified as ocular myasthenia gravis (OMG). Diagnosis of MG when there are mild, isolated ocular symptoms can be difficult, and currently available diagnostic techniques are insensitive, non-specific or technically cumbersome. In addition, there are no accurate biomarkers to follow severity of ocular dysfunction in MG over time. Single-fiber electromyography (SFEMG) and repetitive nerve stimulation (RNS) offers a way of detecting and measuring ocular muscle dysfunction in MG, however, challenges of these methods include a poor signal to noise ratio in quantifying eye muscle weakness especially in mild cases. This paper presents one of the attempts to use the electric potentials from the eyes or electrooculography (EOG) signals but obtained from three different forms of sleep testing to differentiate MG patients from age- and gender-matched controls. We analyzed 8 MG patients and 8 control patients and demonstrated a difference in the average eye movements detected between the groups. A classification accuracy as high as 68.8% was achieved using a linear discriminant analysis based classifier.

Myasthenia gravis on the Dutch antilles : an epidemiological study

NARCIS (Netherlands)

Holtsema, H; Mourik, J; Rico, RE; Falconi, [No Value; Kuks, JBM; Oosterhuis, HJGH

2000-01-01

We carried out an epidemiological study on thr prevalence and annual incidence of myasthenia gravis on tropical islands Curacao and Aruba in the period 1980 1995. Twenty-one patients (seven men and 14 women) were identified. The point prevalence increased from 29 per million in 1980 to about 70 per

Ten-year results of thoracoscopic unilateral extended thymectomy performed in nonthymomatous myasthenia gravis.

Science.gov (United States)

Tomulescu, Victor; Sgarbura, Olivia; Stanescu, Codrut; Valciu, Crisanda; Campeanu, Ana; Herlea, Vlad; Popescu, Irinel

2011-11-01

The aim of this study was to analyze the 10-year results of thoracoscopic unilateral extended thymectomy (TUET) performed in nontumoral myasthenia gravis according to the Myasthenia Gravis Foundation of America recommendations. Thoracoscopic unilateral extended thymectomy has the benefits of a minimally invasive approach. Previous data have shown promising midterm results but long-term results were lacking. Two hundred forty patients with nontumoral myasthenia gravis who underwent surgery between 1999 and 2009 were eligible for the study. The mean follow-up was of 67 months (range: 12-125), 134 patients completed follow-up assessments more than 60 months after TUET. There were 39 males (16.3%) and 201 females (83.7%), with an age range from 8 to 60 years. The mean preoperative disease duration was 21.5 months. All patients underwent preoperative steroid therapy. Anticholinesterase drugs were required for 123 patients (51.3%), and immunosuppressive drugs were required for 87 (36.3%) patients. The pathologic findings were as follows: normal thymus in 13 patients (5.5%), involuted thymus in 65 patients (27%), and hyperplastic thymus in 162 patients (67.5%). The average weight of the thymus was 110 ± 45 g. Ectopic thymic tissue was found in 147 patients (61.3%). There was no mortality, and morbidity consisted of 12 patients (5%). Complete stable remission was achieved in 61% of the patients, and the cumulative probability of achieving complete stable remission was 0.88 at 10 years. With zero mortality, low morbidity, and comparable long-term results to open surgery, TUET can be regarded as the best treatment option for patients undergoing surgery for myasthenia gravis.

Sensory aspects in myasthenia gravis: A translational approach.

Science.gov (United States)

Leon-Sarmiento, Fidias E; Leon-Ariza, Juan S; Prada, Diddier; Leon-Ariza, Daniel S; Rizzo-Sierra, Carlos V

2016-09-15

Myasthenia gravis is a paradigmatic muscle disorder characterized by abnormal fatigue and muscle weakness that worsens with activities and improves with rest. Clinical and research studies done on nicotinic acetylcholine receptors have advanced our knowledge of the muscle involvement in myasthenia. Current views still state that sensory deficits are not "features of myasthenia gravis". This article discusses the gap that exists on sensory neural transmission in myasthenia that has remained after >300years of research in this neurological disorder. We outline the neurobiological characteristics of sensory and motor synapses, reinterpret the nanocholinergic commonalities that exist in both sensory and motor pathways, discuss the clinical findings on altered sensory pathways in myasthenia, and propose a novel way to score anomalies resulting from multineuronal inability associated sensory troubles due to eugenic nanocholinergic instability and autoimmunity. This medicine-based evidence could serve as a template to further identify novel targets for studying new medications that may offer a better therapeutic benefit in both sensory and motor dysfunction for patients. Importantly, this review may help to re-orient current practices in myasthenia. Copyright © 2016 Elsevier B.V. All rights reserved.

The Effect of Acupuncture on Blepharoptosis and Diplopia in Ocular Myasthenia Gravis: A Report of Three Cases

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Mohammad Etezad Razavi

2015-07-01

Full Text Available Introduction: The aim of this study was to evaluate the efficacy of acupuncture therapy on relieving ocular symptoms in resistant Ocular Myasthenia Gravis (OMG. Case: In this pilot study, three patients with OMG were offered ten sessions of acupuncture therapy, twice weekly, while they received their routine medical treatment. Their symptoms included ptosis and diplopia. Subjective impression of changes was assessed based on Ocular-quantitative Myasthenia Gravis (QMG scoring before and after treatment sessions. Conclusion:All three patients enrolled in this study showed resolution of diplopia. Their subjective impression of symptoms was improved. However, the score of ptosis did not improve considerably. Acupuncture is another choice for reducing ocular symptoms besides conventional treatment in resistant Ocular Myasthenia Gravis (OMG. However, future randomized controlled trial studies are needed to approve this issue.

Clinical Characteristics of Juvenile Myasthenia Gravis in Southern China

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Xin Huang

2018-02-01

Full Text Available ObjectivesTo describe the clinical profile, clinical outcomes and factors that may affect the outcome of juvenile myasthenia gravis (JMG patients in southern China.MethodsWe reviewed information relating to JMG patients treated and evaluated at the First Affiliated Hospital, Sun Yat-sen University, between 1998 and 2015. The study involved 327 JMG patients who had been followed up for ≥1 year.ResultsOverall, 77.4% patients showed initial symptoms in the prepubertal period (<12 years. 306 patients showed only ocular symptoms at onset. By the final follow-up, 61 ocular myasthenia gravis (OMG patients (61/306, 19.9% had developed generalized myasthenia gravis (GMG. Anti-acetylcholine receptor antibodies (AChR-Ab titer was an independent risk factor for generalization. Eleven patients (3.4% experienced spontaneous remission, but four relapsed. Low-dose oral prednisone (0.25 mg/kg was administered when symptoms did not significantly improve after pyridostigmine treatment. Immunosuppressants were administered when prednisone was unsatisfactory. Optimal outcome was achieved in 59.6% of patients. Specifically, 60 patients (18.3% attained complete stable remission (CSR, 12 (3.7% attained pharmaceutical remission (PR, and 123 (37.6% attained minimal manifestation (MM. In total, 53 OMG patients (21.5% attained CSR, a significantly higher proportion than among the GMG patients (8.6%, P = 0.009. Moreover, 67.2% of patients with duration <2 years showed significant clinical improvement compared with 46.3% of those with duration >2 years (P < 0.001. Thymectomy did not exhibit definite efficacy for JMG patients.ConclusionThere was a low frequency of cases positive for AChR-Ab in the Chinese population. AChR-Ab titer was revealed as an independent risk factor for generalization. Low doses of prednisone could treat JMG effectively with few side effects.

Treatment of experimental myasthenia gravis with total lymphoid irradiation

International Nuclear Information System (INIS)

de Silva, S.; Blum, J.E.; McIntosh, K.R.; Order, S.; Drachman, D.B.

1988-01-01

Total lymphoid irradiation (TLI) has been reported to be effective in the immunosuppressive treatment of certain human and experimental autoimmune disorders. We have investigated the effects of TLI in Lewis rats with experimental autoimmune myasthenia gravis (EAMG) produced by immunization with purified torpedo acetylcholine receptor (AChR). The radiation is given in 17 divided fractions of 200 rad each, and nonlymphoid tissues are protected by lead shielding. This technique suppresses the immune system, while minimizing side effects, and permits the repopulation of the immune system by the patient's own bone marrow cells. Our results show that TLI treatment completely prevented the primary antibody response to immunization with torpedo AChR, it rapidly abolished the ongoing antibody response in established EAMG, and it suppressed the secondary (anamnestic) response to a boost of AChR. No EAMG animals died during TLI treatment, compared with six control animals that died of EAMG. TLI produces powerful and prompt immunosuppression and may eventually prove useful in the treatment of refractory human myasthenia gravis

Gravi-Burst: Super-GZK Cosmic Rays from Localized Gravity

International Nuclear Information System (INIS)

Davoudiasl, Hooman

2000-01-01

The flux of cosmic rays beyond the GZK cutoff (∼ 10 20 eV) may be explained through their production by ultra high energy cosmic neutrinos, annihilating on the relic neutrino background, in the vicinity of our galaxy. This process is mediated through the production of a Z boson at resonance, and is generally known as the Z-Burst mechanism. We show that a similar mechanism can also contribute to the super-GZK spectrum at even higher, ultra-GZK energies, where the particles produced at resonance are the Kaluza-Klein gravitons of weak scale mass and coupling from the Randall-Sundrum (RS) hierarchy model of localized gravity model. We call this mechanism Gravi-Burst. We discuss the parameter space of relevance to Gravi-Bursts, and comment on the possibility of its contribution to the present and future super-GZK cosmic ray data and place bounds on the RS model parameters. Under certain assumptions about the energy spectrum of the primary neutrinos we find that cosmic ray data could be potentially as powerful as the LHC in probing the RS model

Treatment of experimental myasthenia gravis with total lymphoid irradiation

Energy Technology Data Exchange (ETDEWEB)

de Silva, S.; Blum, J.E.; McIntosh, K.R.; Order, S.; Drachman, D.B.

1988-07-01

Total lymphoid irradiation (TLI) has been reported to be effective in the immunosuppressive treatment of certain human and experimental autoimmune disorders. We have investigated the effects of TLI in Lewis rats with experimental autoimmune myasthenia gravis (EAMG) produced by immunization with purified torpedo acetylcholine receptor (AChR). The radiation is given in 17 divided fractions of 200 rad each, and nonlymphoid tissues are protected by lead shielding. This technique suppresses the immune system, while minimizing side effects, and permits the repopulation of the immune system by the patient's own bone marrow cells. Our results show that TLI treatment completely prevented the primary antibody response to immunization with torpedo AChR, it rapidly abolished the ongoing antibody response in established EAMG, and it suppressed the secondary (anamnestic) response to a boost of AChR. No EAMG animals died during TLI treatment, compared with six control animals that died of EAMG. TLI produces powerful and prompt immunosuppression and may eventually prove useful in the treatment of refractory human myasthenia gravis.

Reseña de publicaciones

Directory of Open Access Journals (Sweden)

Facultad de Medicina Revista

1994-07-01

Full Text Available Insuficiencia renal crónica, patología digestiva alta y Helicobacter Pylori / Histerectomía en el manejo del NIC en el IMI / Miastenia gravis Ila. Timectoria vs. tratamiento médico / Estudio comparativo de tres alternativas de valoración pre-quirúrgica en pacientes asa Il y IIl / Abordaje endoscópico de la silla turca a través del seno esfenoidal / Evolución de los trastornos específicos del aprendizaje / Estudio del tiempo de tránsito colónico con marcadores radiopacos en pacientes con constipación idiopática crónica / Seguimiento de pacientes asintomáticos colonizados por H pylori / Estudio sobre el comportamiento radiol6gico del cuello femoral y la fisis de crecimiento proximal del fémur.

Diagnostic imaging and treatment of an invasive thymoma in myasthenia gravis. Bildgebende Diagnostik und Therapie eines invasiven Thymoms mit Myasthenia gravis

Energy Technology Data Exchange (ETDEWEB)

Soetje, G.; Brinkmann, G. (Kiel Univ. (Germany, F.R.). Abt. Radiologie); Striepling, E.; Engemann, R. (Kiel Univ. (Germany, F.R.). Abt. Allgemeine Chirurgie)

1991-05-01

A 40-year old woman with an invasive thymoma and myasthenia gravis is described in this article. Chest-x-ray, CT and MRI of the mediastinum could not offer definite results on tumour malignancy. Radical surgical removal was the consequence. This revealed a tumour infiltration of the pleura and pericardium; hence an adjuvant irradiation must have been performed. Mestinon-treatment was afterwards gradually reduced. (orig.).

Myasthenia gravis: an update for the clinician

Science.gov (United States)

Sieb, J P

2014-01-01

This paper provides a thorough overview of the current advances in diagnosis and therapy of myasthenia gravis (MG). Nowadays the term ‘myasthenia gravis’ includes heterogeneous autoimmune diseases, with a postsynaptic defect of neuromuscular transmission as the common feature. Myasthenia gravis should be classified according to the antibody specificity [acetylcholine, muscle-specific receptor tyrosine kinase (MuSK), low-density lipoprotein receptor-related protein 4 (LRP4), seronegative], thymus histology (thymitis, thymoma, atrophy), age at onset (in children; aged less than or more than 50 years) and type of course (ocular or generalized). With optimal treatment, the prognosis is good in terms of daily functions, quality of life and survival. Symptomatic treatment with acetylcholine esterase inhibition is usually combined with immunosuppression. Azathioprine still remains the first choice for long-term immunosuppressive therapy. Alternative immunosuppressive options to azathioprine include cyclosporin, cyclophosphamide, methotrexate, mycophenolate mofetil and tacrolimus. Rituximab is a promising new drug for severe generalized MG. Emerging therapy options include belimumab, eculizumab and the granulocyte– macrophage colony-stimulating factor. One pilot study on etanercept has given disappointing results. For decades, thymectomy has been performed in younger adults to improve non-paraneoplastic MG. However, controlled prospective studies on the suspected benefit of this surgical procedure are still lacking. In acute exacerbations, including myasthenic crisis, intravenous immunoglobulin, plasmapheresis and immunoadsorption are similarly effective. PMID:24117026

Analysis of TNF-related apoptosis-inducing ligand and receptors and implications in thymus biology and myasthenia gravis.

Science.gov (United States)

Kanatli, Irem; Akkaya, Bahar; Uysal, Hilmi; Kahraman, Sevim; Sanlioglu, Ahter Dilsad

2017-02-01

Myasthenia Gravis is an autoantibody-mediated, neuromuscular junction disease, and is usually associated with thymic abnormalities presented as thymic tumors (~10%) or hyperplastic thymus (~65%). The exact role of thymus in Myasthenia Gravis development is not clear, yet many patients benefit from thymectomy. The apoptotic ligand TNF-Related Apoptosis-Inducing Ligand is thought to be involved in the regulation of thymocyte counts, although conflicting results are reported. We investigated differential expression profiles of TNF-Related Apoptosis-Inducing Ligand and its transmembrane receptors, Nuclear Factor-kB activation status, and apoptotic cell counts in healthy thymic tissue and pathological thymus from Myasthenia Gravis patients. All tissues expressed TNF-Related Apoptosis-Inducing Ligand and its receptors, with hyperplastic tissue having the highest expression levels of death receptors DR4 and DR5. No detectable Nuclear Factor-kB activation, at least via the canonical Protein Kinase A-mediated p65 Ser276 phosphorylation, was evident in any of the tissues studied. Apoptotic cell counts were higher in MG-associated tissue compared to the normal thymus. Possible use of the TNF-Related Apoptosis-Inducing Ligand within the concept of an apoptotic ligand-mediated medical thymectomy in thymoma- or thymic hyperplasia-associated Myasthenia Gravis is also discussed. Copyright © 2016 Elsevier B.V. All rights reserved.

Clinical and pathological aspects of microscopic thymoma with myasthenia gravis and review of published reports.

Science.gov (United States)

Fukuhara, Mitsuro; Higuchi, Mitsunori; Owada, Yuki; Inoue, Takuya; Watanabe, Yuzuru; Yamaura, Takumi; Muto, Satoshi; Hasegawa, Takeo; Suzuki, Hiroyuki

2017-06-01

Microscopic thymomas, defined as epithelial proliferations smaller than 1 mm in diameter, characteristically occur in patients with myasthenia gravis without macroscopic thymic epithelial tumors. However, some clinical and pathological aspects of this entity are still unclear. This retrospective study includes five consecutive patients who had undergone extended thymectomy for myasthenia gravis at our institution from April 2007 to March 2016 and in whom microscopic thymomas were diagnosed by histopathological examination of the resected specimens. During the same period, we performed 32 extended transsternal thymothymectomies/thymectomies in patients with myasthenia gravis, including the above five cases. We here review 18 cases of microscopic thymoma, including our five cases and 13 previously reported cases. The incidence of previously undiagnosed microscopic thymoma in patients undergoing thymectomy for myasthenia gravis in our institution is 15.2%. Serum preoperative anti-acetylcholine receptor antibody (anti-AchR Ab) titers were abnormally high in all of our five cases h (74.4±53.3 nmol/L) and decreased significantly after surgery (11.7±13.5 nmol/L, P=0.037). We divided our cases into the following three groups: microscopic thymoma group (Group M), thymoma group (Group T) and non-thymic tumor group (Group N). The mean preoperative anti-AchR Ab titers of these groups were 74.4, 26.5, and 368 nmol/L, respectively. All these values decreased postoperatively. The mean anti-AchR Ab titer was significantly higher in Group M than in Group T (P=0.034). All five cases in Group M were found by post-operative pathological examination to have multifocal type A thymomas. Microscopic thymomas tend to be multifocal type A thymomas. Anti-AchR Ab titers decreased significantly in all groups. It is very important to both perform complete extended thymectomies in patients with myasthenia gravis and pathological examination of thin slices of thymic tissue to maximize detection

Evaluation of the key aroma compounds in beef and pork vegetable gravies a la chef by stable isotope dilution assays and aroma recombination experiments.

Science.gov (United States)

Christlbauer, Monika; Schieberle, Peter

2011-12-28

Although the aroma compounds of meat processed as such have been studied previously, data on complete homemade dishes containing beef and pork meat were scarcely studied. Recently, 38 odor-active compounds were characterized in beef and pork vegetable gravies using GC-olfactometry. In the present investigation, the most odor-active compounds were quantitated in a freshly prepared stewed beef vegetable gravy (BVG) as well as a stewed pork vegetable gravy (PVG) by means of stable isotope dilution assays. Calculation of odor activity values (OAVs; ratio of concentration to odor threshold) revealed 3-mercapto-2-methylpentan-1-ol, (E,E)-2,4-decadienal, (E,Z)-2,6-nonadienal, (E)-2-decenal, (E)-2-undecanal, and 3-hydroxy-4,5-dimethyl-2(5H)-furanone as the most potent odorants in both gravies. However, significantly different OAVs were found for 12-methyltridecanal, which was much higher in the BVG, whereas (E,Z)-2,4-decadienal showed a clearly higher OAV in the PVG. Aroma recombination experiments performed on the basis of the actual concentrations of the odorants in both gravies revealed a good similarity of the aromas of both model mixtures containing all odorants with OAVs > 1 with those of the original gravies.

[Therapeutic strategies against myasthenia gravis].

Science.gov (United States)

Utsugisawa, Kimiaki; Nagane, Yuriko

2013-05-01

Many patients with myasthenia gravis (MG) still find it difficult to maintain daily activities due to chronic residual fatigability and long-term side effects of oral corticosteroids, since full remission is not common. Our analysis demonstrated that disease severity, oral corticosteroids, and depressive state are the major factors negatively associated with QOL, and that QOL of MM status patients taking CSR and is a target of treatment. In order to achieve early MM or better status with prednisolne strategy that can achieve early improvement by performing an aggressive therapy using combined treatment with plasmapheresis and high-dose intravenous methylprednisolone and then maintain an improved status using low-dose oral corticosteroids and calcineurin inhibitors.

Ectopic cervical thymoma in a patient with Myasthenia gravis

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Chang Hung

2011-07-01

Full Text Available Abstract Ectopic cervical thymoma is rare and is often misdiagnosed as a thyroid tumor or other malignancy. Ectopic thymic tissue can be found along the entire thymic descent path during embryogenesis. However, a thymoma arising from such ectopic thymic tissue is extremely rare. Herein we report a patient with ectopic cervical thymoma and myasthenia gravis (MG and discuss the management.

Pathogenesis of myasthenia gravis: update on disease types, models, and mechanisms

Science.gov (United States)

Phillips, William D.; Vincent, Angela

2016-01-01

Myasthenia gravis is an autoimmune disease of the neuromuscular junction (NMJ) caused by antibodies that attack components of the postsynaptic membrane, impair neuromuscular transmission, and lead to weakness and fatigue of skeletal muscle. This can be generalised or localised to certain muscle groups, and involvement of the bulbar and respiratory muscles can be life threatening. The pathogenesis of myasthenia gravis depends upon the target and isotype of the autoantibodies. Most cases are caused by immunoglobulin (Ig)G1 and IgG3 antibodies to the acetylcholine receptor (AChR). They produce complement-mediated damage and increase the rate of AChR turnover, both mechanisms causing loss of AChR from the postsynaptic membrane. The thymus gland is involved in many patients, and there are experimental and genetic approaches to understand the failure of immune tolerance to the AChR. In a proportion of those patients without AChR antibodies, antibodies to muscle-specific kinase (MuSK), or related proteins such as agrin and low-density lipoprotein receptor-related protein 4 (LRP4), are present. MuSK antibodies are predominantly IgG4 and cause disassembly of the neuromuscular junction by disrupting the physiological function of MuSK in synapse maintenance and adaptation. Here we discuss how knowledge of neuromuscular junction structure and function has fed into understanding the mechanisms of AChR and MuSK antibodies. Myasthenia gravis remains a paradigm for autoantibody-mediated conditions and these observations show how much there is still to learn about synaptic function and pathological mechanisms. PMID:27408701

Pathogenesis of myasthenia gravis: update on disease types, models, and mechanisms.

Science.gov (United States)

Phillips, William D; Vincent, Angela

2016-01-01

Myasthenia gravis is an autoimmune disease of the neuromuscular junction (NMJ) caused by antibodies that attack components of the postsynaptic membrane, impair neuromuscular transmission, and lead to weakness and fatigue of skeletal muscle. This can be generalised or localised to certain muscle groups, and involvement of the bulbar and respiratory muscles can be life threatening. The pathogenesis of myasthenia gravis depends upon the target and isotype of the autoantibodies. Most cases are caused by immunoglobulin (Ig)G1 and IgG3 antibodies to the acetylcholine receptor (AChR). They produce complement-mediated damage and increase the rate of AChR turnover, both mechanisms causing loss of AChR from the postsynaptic membrane. The thymus gland is involved in many patients, and there are experimental and genetic approaches to understand the failure of immune tolerance to the AChR. In a proportion of those patients without AChR antibodies, antibodies to muscle-specific kinase (MuSK), or related proteins such as agrin and low-density lipoprotein receptor-related protein 4 (LRP4), are present. MuSK antibodies are predominantly IgG4 and cause disassembly of the neuromuscular junction by disrupting the physiological function of MuSK in synapse maintenance and adaptation. Here we discuss how knowledge of neuromuscular junction structure and function has fed into understanding the mechanisms of AChR and MuSK antibodies. Myasthenia gravis remains a paradigm for autoantibody-mediated conditions and these observations show how much there is still to learn about synaptic function and pathological mechanisms.

[The muscular lesion in myasthenia gravis: study of 17 cases with muscular histochemistry].

Science.gov (United States)

Werneck, L C

1982-03-01

A study of 17 muscle biopsies from patients with myasthenia gravis was done, using freshfrozen section and histochemistry tecnics. It was found 15 abnormal muscle biopsies. The most common abnormality were small dark angular fibers, excess of lipids droplets outside the muscle membrane, changes in fiber size and type II fiber atrophy. These findings suggested denervation in 11 biopsies, type II fiber atrophy in 7, linfocyte infiltration in 4, fiber necrosis with fagocitosis in 1 and 2 were normal. Was noted a direct correlation between the disease duration and the severity of the histological abnormality. Two patients had tymoma. Congenital myasthenia gravis, rheumatoid arthritis, intersticial hypertrophic neuritis, Hashimoto tireoiditis and concomitance of myasthenic syndrome was found once in different patients.

Influence of temperature shifts on survival, growth, and toxin production by psychrotrophic and mesophilic strains of Bacillus cereus in potatoes and chicken gravy.

Science.gov (United States)

Mahakarnchanakul, W; Beuchat, L R

1999-03-15

A study was done to determine the influence of temperature on growth and toxin production characteristics of psychrotrophic and mesophilic strains of Bacillus cereus when inoculated into mashed potatoes and chicken gravy containing various concentrations of sodium chloride and held at temperatures different from those at which cells had been cultured. Logarithmic growth phase cells (10 h, 30 degrees C) of psychrotrophic (F3802A/84) and mesophilic (B4ac-1) strains of Bacillus cereus were inoculated into rehydrated commercially processed instant mashed potatoes and chicken gravy supplemented with 0, 2, or 4% sodium chloride. Growth, survival, and diarrheal toxin production in potatoes and gravy held at 30, 37, and 10 degrees C (strain F3802A/84) or 30, 40, and 10 degrees C (strain B4ac-1) were monitored. Both strains grew in both foods containing no added sodium chloride or 2% sodium chloride when held at 30, 37, or 40 degrees C for 2 days. Strain B4ac-1 grew better than strain F3802A/84 in foods containing 4% sodium chloride. Maximum amounts of enterotoxin (1024 ng/g) were produced by strain B4ac-1 in chicken gravy held at 30 and 40 degrees C. Strain F3802A/84 grew to populations of 7 log10 CFU/g in foods containing no added sodium chloride or 2% sodium chloride at 10 degrees C. Strain F3802A/84 produced the highest amount of enterotoxin (1024 ng/g) at 30 degrees C in chicken gravy containing 0.7 or 2% sodium chloride; however, little or low amounts of toxin (4-16 ng/g) were produced in chicken gravy at 10 degrees C. Compared to strain B4ac-1, cells of strain F3802A/84 subjected to a downward shift in incubation temperature (10 degrees C) grew more rapidly in chicken gravy. Strain B4ac-1 produced the highest amount of toxin (1024 ng/g) at 30 degrees C in gravy containing 4% sodium chloride and at 40 degrees C in gravy containing 0.7% sodium chloride. Toxin was not detected in inoculated mashed potatoes. Results of this study indicate that shifts in incubation

Relationship between hearing function and myasthenia gravis: A contemporary review.

Science.gov (United States)

Ralli, Massimo; Altissimi, Giancarlo; Di Stadio, A; Mazzei, Filippo; Turchetta, Rosaria; Cianfrone, Giancarlo

2017-10-01

There is increasing evidence of a connection between hearing function and myasthenia gravis (MG). Studies of the pathophysiological basis of this relationship suggest that acetylcholine receptors (AChRs) on outer hair cells (OHCs) play a central role. In patients with MG, autoantibodies against AChRs induce a progressive loss of AChRs on OHCs, decreasing their electromotility. The stapedial reflex decay test can be altered in MG patients, and can be used as an additional tool for diagnosis and monitoring. Transient evoked and distortion product otoacoustic emissions are the main diagnostic tool for monitoring OHC functionality in MG patients, and can be used to record subclinical hearing alterations before the onset of clinically evident hearing loss. Understanding the association between MG and hearing dysfunction requires a multidisciplinary approach. Otolaryngologists should take this relationship into account when approaching patients with a diagnosis of myasthenia gravis and "in patients with MG" with ण128;œin MG patients, and the progress of hearing alterations should always be monitored in patients with MG.

“Myasthenia Gravis and Anesthesia – A Review of the Literature ...

African Journals Online (AJOL)

Myasthenia gravis (MG) is an immunological disorder characterized by damaged acetylcholine receptors (AchR) due to antibody, which not only blocks the receptor site but also causes degenerations of the receptors.. It is closely associated with disorders of similar pathogenesis, such as pernicious anaemia, thyrotoxicosis, ...

Headache Associated with Myasthenia Gravis: The Impact of Mild Ocular Symptoms

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Yoshinori Nishimoto

2011-01-01

Full Text Available Myasthenia gravis (MG patients visiting outpatient clinics frequently complain of headache. However, there have been few reports on the relation between chronic headache and myasthenia gravis (MG. We aimed to investigate whether MG symptoms affect the development or worsening of chronic headache. Among the 184 MG patients who were followed at the MG clinics, tension-type headache was observed in 71 (38.6% patients and 9 (4.9% complained of migraine. Twenty-five (13.6% complained that headache appeared or was exacerbated after the MG onset. The investigation into differences in the clinical characteristics of the MG patients showed that women tended to suffer from MG-associated headache more often than men. Logistic regression analyses revealed that female gender and mild ocular symptoms were independently predictive of headache associated with MG. Our results suggest that treatment of chronic headache should be required to improve the quality of life in MG patients.

IFNA-AS1 regulates CD4+ T cell activation in myasthenia gravis though HLA-DRB1.

Science.gov (United States)

Luo, Mengchuan; Liu, Xiaofang; Meng, Huanyu; Xu, Liqun; Li, Yi; Li, Zhibin; Liu, Chang; Luo, Yue-Bei; Hu, Bo; Xue, Yuanyuan; Liu, Yu; Luo, Zhaohui; Yang, Huan

2017-10-01

Abnormal CD4 + T cell activation is known to play roles in the pathogenesis of myasthenia gravis (MG). However, little is known about the mechanisms underlying the roles of lncRNAs in regulating CD4 + T cell. In this study, we discovered that the lncRNA IFNG-AS1 is abnormally expressed in MG patients associated with quantitative myasthenia gravis (QMG) and the positive anti-AchR Ab levels patients. IFNG-AS1 influenced Th1/Treg cell proliferation and regulated the expression levels of their transcription factors in an experimental autoimmune myasthenia gravis (EAMG)model. IFNG-AS1 could reduce the expression of HLA-DRB and HLA-DOB and they had a negative correlation in MG. Furthermore IFNG-AS1 influenced the expression levels of CD40L and CD4 + T cells activation in MG patient partly depend on effecting the HLA-DRB1 expression. It suggests that IFNG-AS1 may be involved in CD4 + T cell-mediated immune responses in MG. Copyright © 2017 The Authors. Published by Elsevier Inc. All rights reserved.

Autoimmune Myasthenia Gravis after Sternal Fracture

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Jens A. Petersen

2012-01-01

Full Text Available We report a 54-year-old woman who suffered a commotio cerebri, whiplash injury and a chest trauma with sternal fracture due to a high-velocity car accident. Two months later, she developed unilateral ptosis and blurred vision, which worsened during the day. Multiple diagnoses were suggested, ranging from thoracic outlet syndrome towards depression. Symptoms persisted and five years later, the patient consulted a neurologist. Laboratory analysis revealed significantly elevated levels of antibodies to acetylcholine receptors, and the diagnosis of myasthenia gravis was made. Speculatively, the damage of retrosternal thymic remnants due to a sternal fracture might have precipitated the condition or exacerbated subclinical disease.

PHYSICOCHEMICAL PROPERTIES OF INSTANT PUMPKIN JAVANESE NOODLE GRAVY [Sifat Fisikokimia Saus Bubuk Mi Jawa Instan dari Labu Kering

OpenAIRE

Yusnita Hamzah*; Ng Kim Yee

2012-01-01

This research was carried out to study the physical and chemical characteristics of pumpkin Javanese noodle gravy prepared in powder form. The finished product is categorized as an instant product as it only needs to be rehydrated with warm water (60°C) before consumption. Five gravy formulations were developed with the incorporation of two types of flour (tapioca and corn flours) as the thickening agent. Physical characteristics including colour and swelling properties of the final products ...

Edén: relato, imagen y proyecto. El concepto de paraíso terrenal como generador de arquitecturas

OpenAIRE

Casha Vida, Stephanie

2016-01-01

Esta tesis, Edén: relato, imagen y proyecto. El concepto de Paraíso terrenal como generador de arquitecturas se realiza con el objetivo de estudiar los vínculos entre la idea de Edén, o Paraíso y la arquitectura. Siempre trabajando desde los tres niveles de representación, relato, imagen y proyecto. En la aproximación al objeto de estudio, se procede a estudiar el relato en sí, y se hallan, en la forma misma del relato, unas implicaciones relacionadas con el mundo mitológico y arquetípico. E...

Computed tomography of the mediastinum in myasthenia gravis

Energy Technology Data Exchange (ETDEWEB)

Guertler, K.F.; Janzen, R.W.C.; Hagemann, J.; Otto, H.F.

1982-01-01

Computed tomography of the mediastinum was performed in 45 patients with myasthenia gravis. Surgery was carried out in fourteen. Amongst these, there were four thymomas, one thymolipoma, eight thymic hyperplasias and one normal thymus gland. A further patient, who did not have surgery, probably also had a thymic tumour. The normal thymus and thymic hyperplasia cannot be distinguished on computed tomography. Differentiation of small thymomas from normal thymus is not always possible. Invasion by thymomas can only be appreciated with large tumours.

Millora en el tractament contra la miastènia gravis

OpenAIRE

Ponseti, J.M.

2006-01-01

La miastènia gravis és una malaltia autoimmune i crónica, caracteritzada per una gran debilitat muscular. Un assaig clínic realitzat per la Unitat de Miastènia de l'Hospital General Universitari Vall d'Hebron durant cinc anys acaba de demostrar els beneficis d'incloure un fàrmac, el tacrolimus, per guarir aquesta malaltia.

Current and emerging treatments for the management of myasthenia gravis

Science.gov (United States)

Sathasivam, Sivakumar

2011-01-01

Myasthenia gravis is an autoimmune neuromuscular disorder. There are several treatment options, including symptomatic treatment (acetylcholinesterase inhibitors), short-term immunosuppression (corticosteroids), long-term immunosuppression (azathioprine, cyclosporine, cyclophosphamide, methotrexate, mycophenolate mofetil, rituximab, tacrolimus), rapid acting short-term immunomodulation (intravenous immunoglobulin, plasma exchange), and long-term immunomodulation (thymectomy). This review explores in detail these different treatment options. Potential future treatments are also discussed. PMID:21845054

Worsening of myasthenia gravis after administration of injectable long-acting risperidone for treatment of schizophrenia; first case report and a call for caution.

Science.gov (United States)

Al-Hashel, Jasem Y; Ismail, Ismail Ibrahim; John, John K; Ibrahim, Mohammed; Ali, Mahmoud

2016-01-01

Myasthenia gravis is an autoimmune disease characterized by muscle weakness due to autoantibodies affecting the neuromuscular junction. Co-occurrence of myasthenia gravis and schizophrenia is very rare and raises a challenge in management of both diseases. Antipsychotic drugs exhibit anticholinergic side effects and have the potentials of worsening myasthenia. Long-acting risperidone is an injectable atypical antipsychotic drug that has not been previously reported to worsen myasthenia gravis in literature. We report the first case report of worsening of myasthenia after receiving long-acting risperidone injection for schizophrenia in a 29-year-old female with both diseases. She started to have worsening 2 weeks following the first injection and her symptoms persisted despite receiving plasma exchange. This could be explained by the pharmacokinetics of the drug. We recommend that long-acting risperidone should be used with caution in patients with myasthenia gravis, and clinicians must be aware of the potential risks of this therapy. Copyright © 2016 Elsevier B.V. All rights reserved.

Thymectomy in Myasthenia Gravis

Science.gov (United States)

Aydin, Yener; Ulas, Ali Bilal; Mutlu, Vahit; Colak, Abdurrahim; Eroglu, Atilla

2017-01-01

In recent years, thymectomy has become a widespread procedure in the treatment of myasthenia gravis (MG). Likelihood of remission was highest in preoperative mild disease classification (Osserman classification 1, 2A). In absence of thymoma or hyperplasia, there was no relationship between age and gender in remission with thymectomy. In MG treatment, randomized trials that compare conservative treatment with thymectomy have started, recently. As with non-randomized trials, remission with thymectomy in MG treatment was better than conservative treatment with only medication. There are four major methods for the surgical approach: transcervical, minimally invasive, transsternal, and combined transcervical transsternal thymectomy. Transsternal approach with thymectomy is the accepted standard surgical approach for many years. In recent years, the incidence of thymectomy has been increasing with minimally invasive techniques using thoracoscopic and robotic methods. There are not any randomized, controlled studies which are comparing surgical techniques. However, when comparing non-randomized trials, it is seen that minimally invasive thymectomy approaches give similar results to more aggressive approaches. PMID:28416933

Computed tomography of the mediastinum in myasthenia gravis

International Nuclear Information System (INIS)

Guertler, K.F.; Janzen, R.W.C.; Hagemann, J.; Otto, H.F.; Hamburg Univ.; Hamburg Univ.

1982-01-01

Computed tomography of the mediastinum was performed in 45 patients with myasthenia gravis. Surgery was carried out in fourteen. Amongst these, there were four thymomas, one thymolipoma, eight thymic hyperplasias and one normal thymus gland. A further patient, who did not have surgery, probably also had a thymic tumour. The normal thymus and thymic hyperplasia cannot be distinguished on computed tomography. Differentiation of small thymomas from normal thymus is not always possible. Invasion by thymomas can only be appreciated with large tumours. (orig.) [de

Malignant thymona with symptoms of myasthenia gravis; Grasiczak zlosliwy z objawami nuzliwosci miesni

Energy Technology Data Exchange (ETDEWEB)

Miarzynska, M.; Szlezak, L.; Fibak, J.; Wolski, M.; Lis-Podrzycka, E.; Miarzynski, K. [Szpital im. F. Raszei, Poznan (Poland)

1994-12-31

The rare case of malignant tumor of thymus - Thymoma malignum was described. The initial diagnosis was difficult, because of the irregular symptoms of myasthenia gravis. The diagnostic difficulties, treatment and clinical features of this neoplasm were also discussed. (author)

A case of late-onset, thymoma-associated myasthenia gravis with ryanodine receptor and titin antibodies and concomitant granulomatous myositis.

Science.gov (United States)

Stefanou, M I; Komorowski, L; Kade, S; Bornemann, A; Ziemann, U; Synofzik, M

2016-09-13

Myasthenia gravis is an autoimmune neuromuscular disorder, which has only rarely been reported to co-manifest with myositis. The diagnosis of concomitant myositis in patients with myasthenia gravis is clinically challenging, and requires targeted investigations for the differential diagnosis, including EMG, autoantibody assays, muscle biopsy and, importantly, imaging of the mediastinum for thymoma screening. This report presents a case-vignette of a 72-year-old woman with progressive proximal muscle weakness and myalgias, diagnosed with thymoma-associated myasthenia and bioptically verified granulomatous myositis, with positive autoantibody status for ryanodine receptor and titin antibodies. The diagnosis of concurrent myositis and myasthenia gravis, especially in the presence of ryanodine receptor and titin antibodies, should lead neurologists to adopt different treatment strategies compared to those applied in myasthenia or myositis alone. Moreover, further evidence is warranted that titin and, particularly, ryanodine receptor antibodies may co-occur or be pathophysiologically involved in myasthenia-myositis cases.

Clinical features of patients with Myasthenia gravis from the Henan province, China.

Science.gov (United States)

Gao, Feng; Zhao, Xue; Zhang, Jing; Cui, Xinzheng; Zhang, Yingna; Li, Qianru; Liu, Pingping; Fang, Hua; Du, Ying; Zhang, Qingyong; Yang, Junhong; Zhang, Yunke

2016-05-01

Myasthenia gravis (MG) occurs globally, and many studies have indicated that there are regional differences in epidemiology, biomarkers, and prognosis of MG. A total of 478 patients with MG who visited hospitals in the Henan Province between January 2010 and February 2014 were included. Age, gender, age at onset, serum antibody, thymus pathology, treatment information, and Myasthenia Gravis Foundation of America (MGFA) classification were assessed. Compared with previous reports from other areas, we found some differences in MG patients from Henan. The proportion of childhood MG in our study was lower than other reports of Oriental patients with MG. There seems to be an association between onset age, muscle involvement, and acetylcholine receptor antibody levels. Thymectomy improved the symptoms in 78.5% of thymectomy-treated patients. These differences are likely related to geographical, environmental, and ethnic differences. Understanding these differences will help us to define more specific treatment. © 2016 Wiley Periodicals, Inc.

A patient with myasthenia gravis and a large arachnoid cyst - report of a case.

Science.gov (United States)

Bucuk, Mira; Gasparovic, Iva; Sonnenschein, Ivan; Perkovic, Olivio

2017-05-01

Myasthenia gravis is a chronic autoimmune disease characterized by weakening of voluntary muscles during the day and a marked restitution of function during the night and after rest. The symptoms may worsen over days or weeks, sometimes even in a few hours, and are usually well controlled by appropriate therapy. Arachnoid cysts are congenital or acquired deformities of the arachnoid membrane and are usually too small to cause distinct clinical symptomatology. We describe a case of a 76-year-old myasthenia gravis patient with an arachnoid cyst. To the best of our knowledge this is the first reported case of these two comorbidities together.

Reflexão Jornalística sobre o Relato Desportivo

OpenAIRE

Nunes, João Miguel Santos

2013-01-01

Relatório de Estágio apresentado para cumprimento dos requisitos necessários à obtenção do grau de Mestre em Ciências da Comunicação – Estudos dos Media e de Jornalismo O presente trabalho tem como objetivo refletir academicamente sobre o relato desportivo. O tema foi até hoje pouco estudado, embora a narração de acontecimentos desportivos seja presença assídua nas grelhas de programação das estações de rádio. Se na prática parece existir um consenso em relação à forma como o relato des...

Emergency Department Management of a Myasthenia Gravis Patient with Community-Acquired Pneumonia: Does Initial Antibiotic Choice Lead to Cure or Crisis?

Science.gov (United States)

Van Berkel, Megan A; Twilla, Jennifer D; England, Bryan S

2016-02-01

Myasthenic crisis is a rare, yet serious condition that carries a 3%-8% mortality rate. Although infection is a common cause of decompensation in myasthenia gravis, several antibiotics classes have also been associated with an exacerbation. Selecting antibiotics can be a daunting clinical task and, if chosen inappropriately, can carry significant deleterious consequences. Not only do clinicians have to focus on treating the underlying infection appropriately, but avoiding antibiotics that may potentiate a myasthenic crisis is also vital. An 85-year-old female with a history of myasthenia gravis presented to the emergency department (ED) with increasing generalized weakness and shortness of breath. Clinical work-up was consistent with a community-acquired pneumonia (CAP) diagnosis. Her medical history included a myasthenia gravis exacerbation shortly after receiving moxifloxacin for CAP. After reviewing the patient's allergies, as well as potential antibiotic triggers, the decision was made to treat with tigecycline. The patient responded well to tigecycline therapy and was deemed stable for discharge on day 4 of hospitalization. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: Evaluation of the myasthenia gravis patient frequently originates in the ED. It is important for clinicians to be able to distinguish between an underlying illness and a myasthenic crisis. In the event of an infectious process causing clinical deterioration in a myasthenia patient, optimal antibiotic selection becomes paramount. This patient case highlights the addition of tigecycline to the armamentarium of therapies available to treat myasthenia gravis patients presenting to the emergency department with CAP. Copyright © 2016 Elsevier Inc. All rights reserved.

Pressure resistance of cold-shocked Escherichia coli O157:H7 in ground beef, beef gravy and peptone water.

Science.gov (United States)

Baccus-Taylor, G S H; Falloon, O C; Henry, N

2015-06-01

(i) To study the effects of cold shock on Escherichia coli O157:H7 cells. (ii) To determine if cold-shocked E. coli O157:H7 cells at stationary and exponential phases are more pressure-resistant than their non-cold-shocked counterparts. (iii) To investigate the baro-protective role of growth media (0·1% peptone water, beef gravy and ground beef). Quantitative estimates of lethality and sublethal injury were made using the differential plating method. There were no significant differences (P > 0·05) in the number of cells killed; cold-shocked or non-cold-shocked. Cells grown in ground beef (stationary and exponential phases) experienced lowest death compared with peptone water and beef gravy. Cold-shock treatment increased the sublethal injury to cells cultured in peptone water (stationary and exponential phases) and ground beef (exponential phase), but decreased the sublethal injury to cells in beef gravy (stationary phase). Cold shock did not confer greater resistance to stationary or exponential phase cells pressurized in peptone water, beef gravy or ground beef. Ground beef had the greatest baro-protective effect. Real food systems should be used in establishing food safety parameters for high-pressure treatments; micro-organisms are less resistant in model food systems, the use of which may underestimate the organisms' resistance. © 2015 The Society for Applied Microbiology.

[Juvenile myasthenia gravis in sub-Saharan Africa: a case study of two consanguine sisters born from consanguinity in Togo].

Science.gov (United States)

Maneh, Nidain; Apetse, Kossivi; Diatewa, Bénédicte Marèbe; Domingo, Sidik Abou-Bakr; Agba, Aidé Isabelle; Ayena, Koffi Didier; Balogou, Koffi Agnon; Balo, Komi Patrice

2017-01-01

Myasthenia gravis is a rare acquired autoimmune pathology causing neuromuscular transmission impairment. Juvenile onset of myasthenia gravis is often characterized by ocular involvement. We report two cases of ocular juvenile myasthenia gravis (JMG) in two siblings. They were two young girls, XA and XB, aged 11 and 9 years, of Malian origin, residing in Togo, born from first-degree of consanguinity presenting to Ophthalmology due to progressive decrease in visual acuity. XA showed visual acuity 8/10 on both eyes while XB showed improvement in visual acuity from 3/10 to 7/10 using a pinhole occluder, suggesting ametropia. XA had a 2-year history of bilateral ptosis lifting the upper eyelid of 7 mm, while XB had a 3-year history of bilateral ptosis with no lifting of the upper eyelid. Ice pack test was strongly positive in both patients. They had Cogan's lid twitch with paresis of the oculomotor nerve without diplopia. The dosage of acetylcholine receptor autoantibodies was normal. The diagnosis of JMG associated with ametropia was suspected. Ametropia was corrected by glasses and a specific treatment with pyridostigmine was initiated, but both patients were lost to follow-up. Autoimmune myasthenia gravis with inaugural ophthalmologic manifestation is rare but it can occur among children living in sub-Saharan Africa. Studies should be conducted to establish the features of this disease.

Recurrence of thymoma with pleural invasion in a patient with myasthenia gravis and pure red blood cell aplasia: a case report

International Nuclear Information System (INIS)

Rodriguez, Sonia Pilar; Zuluaga, Claudia Patricia; Uriza, Luis Felipe C; Sanchez M, Jully Mariana

2008-01-01

Thymoma are thymic tumors that arise from epithelial cells, they have different morphological characteristics. It is known for its association with autoimmune diseases such as myasthenia gravis, pure red cell aplasia, systemic lupus erythematosus, or hipogamaglobulinemia pemphigus foliaceus. The association thymoma-myasthenia gravis-pure red cell aplasia is a rare one; there will be a case report with the corresponding discussion and review of the literature

Nonmelanoma skin cancer risk awareness in azathioprine-treated myasthenia gravis patients.

LENUS (Irish Health Repository)

McGurgan, Iain J

2015-10-01

Increased rates of NMSC (nonmelanoma skin cancer) have recently been reported in people with MG (myasthenia gravis) receiving azathioprine treatment. Guidelines on azathioprine for patients with dermatological and gastrointestinal disorders stress the importance of NMSC risk awareness and prevention. The aim of this study is to assess whether MG patients are being informed of this risk.

Prophylactic Effect of Probiotics on the Development of Experimental Autoimmune Myasthenia Gravis

Science.gov (United States)

Chae, Chang-Suk; Kwon, Ho-Keun; Hwang, Ji-Sun; Kim, Jung-Eun; Im, Sin-Hyeog

2012-01-01

Probiotics are live bacteria that confer health benefits to the host physiology. Although protective role of probiotics have been reported in diverse diseases, no information is available whether probiotics can modulate neuromuscular immune disorders. We have recently demonstrated that IRT5 probiotics, a mixture of 5 probiotics, could suppress diverse experimental disorders in mice model. In this study we further investigated whether IRT5 probiotics could modulate the progression of experimental autoimmune myasthenia gravis (EAMG). Myasthenia gravis (MG) is a T cell dependent antibody mediated autoimmune disorder in which acetylcholine receptor (AChR) at the neuromuscular junction is the major auto-antigen. Oral administration of IRT5 probiotics significantly reduced clinical symptoms of EAMG such as weight loss, body trembling and grip strength. Prophylactic effect of IRT5 probiotics on EMAG is mediated by down-regulation of effector function of AChR-reactive T cells and B cells. Administration of IRT5 probiotics decreased AChR-reactive lymphocyte proliferation, anti-AChR reactive IgG levels and inflammatory cytokine levels such as IFN-γ, TNF-α, IL-6 and IL-17. Down-regulation of inflammatory mediators in AChR-reactive lymphocytes by IRT5 probiotics is mediated by the generation of regulatory dendritic cells (rDCs) that express increased levels of IL-10, TGF-β, arginase 1 and aldh1a2. Furthermore, DCs isolated from IRT5 probiotics-fed group effectively converted CD4+ T cells into CD4+Foxp3+ regulatory T cells compared with control DCs. Our data suggest that IRT5 probiotics could be applicable to modulate antibody mediated autoimmune diseases including myasthenia gravis. PMID:23284891

Angioceratoma conjuntival canino: relato de caso

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J.O.T. Souza

Full Text Available RESUMO O angioceratoma é um tumor de origem vascular, semelhante ao hemangioma, que se diferencia deste por alterações histológicas epiteliais. A apresentação ocular do angioceratoma é pouco frequente em cães, sendo mais comum a ocorrência de hemangioma e hemangiossarcoma. Neste relato, é descrito o caso de um cão, macho, da raça Border Collie, que apresentava uma massa localizada, hiperêmica, bem vascularizada e protrusa, na região temporal da conjuntiva bulbar do olho direito (OD. Foi realizado exame oftalmológico completo e ultrassonografia ocular, tendo sido possível observar que a massa não envolvia outras estruturas oculares além da conjuntiva. Dessa forma, foi realizada a conjuntivectomia parcial, e o material foi encaminhado para análise histológica. O exame histopatológico foi conclusivo para um angioceratoma e mostrou que as margens da amostra estavam livres. O procedimento cirúrgico com margem de segurança foi eficiente no tratamento da neoplasia, sem recorrência até o momento do presente relato.

The value of computed tomography in myasthenia gravis

International Nuclear Information System (INIS)

Brown, L.R.; Muhm, J.R.; Sheedy, P.F. II; Unni, K.K.; Bernatz, P.E.; Hermann, R.C. Jr.

1983-01-01

In a 5 year study, 19 patients with myasthenia gravis were studied by computed tomography (CT) and underwent thymectomy. CT was accurate in detecting the nine true thymic masses but could not differentiate thymomas from nonthymomatous masses, including thymic cysts. No thymoma was found in a patient under 25 years of age. In one case, the 18 sec scanner could not differentiate a large gland from a thymoma. In eight cases, glands with histologic thymic hyperplasia and histologically normal thymus appeared to be similar and could not be differentiated by CT

The value of computed tomography in myasthenia gravis

Energy Technology Data Exchange (ETDEWEB)

Brown, L.R.; Muhm, J.R.; Sheedy, P.F. II; Unni, K.K.; Bernatz, P.E.; Hermann, R.C. Jr.

1983-01-01

In a 5 year study, 19 patients with myasthenia gravis were studied by computed tomography (CT) and underwent thymectomy. CT was accurate in detecting the nine true thymic masses but could not differentiate thymomas from nonthymomatous masses, including thymic cysts. No thymoma was found in a patient under 25 years of age. In one case, the 18 sec scanner could not differentiate a large gland from a thymoma. In eight cases, glands with histologic thymic hyperplasia and histologically normal thymus appeared to be similar and could not be differentiated by CT.

Relatos audiovisuales de ficción sobre la identidad adolescente en contextos escolares

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Laia Falcón Díaz-Aguado

2014-01-01

Full Text Available Los relatos audiovisuales que tienen a los adolescentes como audiencia y protagonistas son utilizados por éstos para buscar referencias con las que construir su identidad. Esta investigación busca comprender cómo es la identidad de los estudiantes adolescentes en distintos tipos de relatos audiovisuales de ficción, como punto de partida para la elaboración de materiales de alfabetización mediática que les ayude en dicho proceso. Para ello se seleccionaron tres narraciones europeas recientes sobre la vida escolar de adolescentes, con las que poder contrastar entre géneros, códigos y valores: la serie televisiva «Física o Química» y las películas «Harry Potter y la Orden del Fénix» y «La clase». El método ha sido el análisis textual de tipo cualitativo, utilizando como categorías las de la morfología del relato clásico. Los resultados reflejan que dicho esquema, familiar para el profesorado de Literatura, permite detectar importantes coincidencias entre relatos (como la importancia de las relaciones de pareja y amistad…, que hacen más significativas las diferencias del contraste (como la orientación hacia el futuro o el valor del pensamiento y del profesorado en dicho proceso. Se concluye que la comparación entre los tres tipos de relatos representa un óptimo recurso para la alfabetización mediática así como para ayudarles a tomar conciencia de cuáles son los valores con los que quieren identificarse y los problemas que pueden obstaculizarlos.

Relato de experiência em educação musical: questões básicas

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José Nunes Fernandes

2015-12-01

Full Text Available Pretende-se discutir a divulgação, cada vez maior, de relatos de experiência pelos educadores em todas as áreas do conhecimento relacionadas ao ensino, considerando-os, muitas vezes, como pesquisa, e, no nosso caso, como pesquisa em educação musical. Com isso, questiona-se: os relatos de experiência não são trabalhos considerados científicos? Qual a sua contribuição para a área? Quais são as diferenças e semelhanças entre relato de experiência, pesquisa-ação e estudo de caso? Como podemos estruturar um relato de experiência? Para responder tais questões foi necessário iniciarmos com a verificação da situação do campo através da análise do quadro de teses e dissertações (de 1970 a 2010, dos Anais dos Congressos Nacionais da ABEM e da Revista da ABEM (de 2001 a 2011. Discutimos em seguida alguns aspectos do estudo de caso e da pesquisa ação, e finalizamos com uma reflexão sobre relato de experiência, incluindo sua definição, usos, indicações de estruturação, cuidados a serem tomados e redação.

Effect of Gender, Disease Duration and Treatment on Muscle Strength in Myasthenia Gravis

DEFF Research Database (Denmark)

Citirak, Gülsenay; Cejvanovic, Sanja; Andersen, Henning

2016-01-01

INTRODUCTION: The aim of this observational, cross-sectional study was to quantify the potential presence of muscle weakness among patients with generalized myasthenia gravis (gMG). The influence of gender, treatment intensity and disease duration on muscle strength and disease progression was also...

Prolonged Dyspnea after Interscalene Block: Attributed to Undiagnosed Addison's Disease and Myasthenia Gravis.

Science.gov (United States)

Skedros, John G; Kiser, Casey J; Mendenhall, Shaun D

2011-01-01

This report describes a patient who had a series of daily interscalene nerve blocks to treat pain following a shoulder manipulation for postsurgical stiffness. She experienced acute respiratory compromise that persisted for many weeks. All typical and unusual causes of these symptoms were ruled out. Her treating pulmonologist theorized that the ipsilateral carotid body had been injured. However, it was subsequently determined that the constellation of symptoms and their prolonged duration were best explained by a poor stress response from Addison's disease coupled with exacerbation of early onset myasthenia gravis. This patient's case is not a typical reaction to interscalene nerve blocks, and thus preoperative testing would not be recommended for myasthenia gravis or Addison's disease without underlying suspicion. We describe this report to inform physicians to consider a workup for these diagnoses if a typical workup rules out all usual causes of complications from an interscalene block.

Prolonged Dyspnea after Interscalene Block: Attributed to Undiagnosed Addison's Disease and Myasthenia Gravis

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John G. Skedros

2011-01-01

Full Text Available This report describes a patient who had a series of daily interscalene nerve blocks to treat pain following a shoulder manipulation for postsurgical stiffness. She experienced acute respiratory compromise that persisted for many weeks. All typical and unusual causes of these symptoms were ruled out. Her treating pulmonologist theorized that the ipsilateral carotid body had been injured. However, it was subsequently determined that the constellation of symptoms and their prolonged duration were best explained by a poor stress response from Addison's disease coupled with exacerbation of early onset myasthenia gravis. This patient's case is not a typical reaction to interscalene nerve blocks, and thus preoperative testing would not be recommended for myasthenia gravis or Addison's disease without underlying suspicion. We describe this report to inform physicians to consider a workup for these diagnoses if a typical workup rules out all usual causes of complications from an interscalene block.

Myeloid-derived suppressor cells as a potential therapy for experimental autoimmune myasthenia gravis.

Science.gov (United States)

Li, Yan; Tu, Zhidan; Qian, Shiguang; Fung, John J; Markowitz, Sanford D; Kusner, Linda L; Kaminski, Henry J; Lu, Lina; Lin, Feng

2014-09-01

We recently demonstrated that hepatic stellate cells induce the differentiation of myeloid-derived suppressor cells (MDSCs) from myeloid progenitors. In this study, we found that adoptive transfer of these MDSCs effectively reversed disease progression in experimental autoimmune myasthenia gravis (EAMG), a T cell-dependent and B cell-mediated model for myasthenia gravis. In addition to ameliorated disease severity, MDSC-treated EAMG mice showed suppressed acetylcholine receptor (AChR)-specific T cell responses, decreased levels of serum anti-AChR IgGs, and reduced complement activation at the neuromuscular junctions. Incubating MDSCs with B cells activated by anti-IgM or anti-CD40 Abs inhibited the proliferation of these in vitro-activated B cells. Administering MDSCs into mice immunized with a T cell-independent Ag inhibited the Ag-specific Ab production in vivo. MDSCs directly inhibit B cells through multiple mechanisms, including PGE2, inducible NO synthase, and arginase. Interestingly, MDSC treatment in EAMG mice does not appear to significantly inhibit their immune response to a nonrelevant Ag, OVA. These results demonstrated that hepatic stellate cell-induced MDSCs concurrently suppress both T and B cell autoimmunity, leading to effective treatment of established EAMG, and that the MDSCs inhibit AChR-specific immune responses at least partially in an Ag-specific manner. These data suggest that MDSCs could be further developed as a novel approach to treating myasthenia gravis and, even more broadly, other diseases in which T and B cells are involved in pathogenesis. Copyright © 2014 by The American Association of Immunologists, Inc.

Mortality in myasthenia gravis: A nationwide population-based follow-up study in Denmark

DEFF Research Database (Denmark)

Hansen, Julie S; Danielsen, Ditte H; Somnier, Finn E

2016-01-01

INTRODUCTION: In previous studies of myasthenia gravis (MG), increased mortality has been reported. The aim of this study was to estimate mortality in patients with acetylcholine receptor antibody-positive (AChR-Ab-seropositive) MG in a nationwide population-based, long-term follow-up study...

Diurnal and day-to-day variation of isometric muscle strength in myasthenia gravis

DEFF Research Database (Denmark)

Vinge, Lotte; Jakobsen, Johannes; Pedersen, Asger Roer

2016-01-01

INTRODUCTION: In patients with myasthenia gravis (MG), muscle strength is expected to decrease gradually during the day due to physical activities. METHODS: Isometric muscle strength at the shoulder, knee, and ankle was determined in 10 MG patients (MGFA class II-IV) who were receiving usual...

[Symptoms of myasthenia gravis in a patient with a history of thymectomy for invasive thymoma].

Science.gov (United States)

Giraldo, Lilliana María; Duque, Camilo; Uribe, Carlos Santiago; Hernández, Olga Helena

2015-01-01

Myasthenia gravis is an antibody-mediated autoimmune disease. Approximately 10-15% of patients present with a thymoma, the presence of which is associated with greater severity of symptoms, myasthenic crisis, and irresponsiveness to front-line therapy. A thymectomy is recommended in young patients with generalized myasthenia gravis and in all patients presenting with thymoma. The patient was a 43-year-old woman, who first showed symptoms of myasthenic crisis in 2005 and presented with invasive thymoma managed with thymectomy and radiotherapy. In the subsequent three years, the patient presented with severe symptoms and two myasthenic crises that required mechanical ventilation and immunoglobulin treatment. Contrast chest computed tomography examinations showed no recurrence. Between 2009 and 2012, the patient experienced decreased symptom severity. In 2013, the patient presented with an exacerbation of symptoms; a contrast chest magnetic resonance scan showed a lesion in the anterior mediastinum, previously observed in 2011, suggestive of residual tissue as opposed to fibrosis. Regular management was started with immunoglobulins; a positron emission tomography scan was inconclusive, requiring a new resection, which showed no evidence of tumor recurrence. Patients with myasthenia gravis and those with myasthenia-related thymoma both share thymectomy as an element of treatment. However, following the procedure, exacerbation or reappearance of symptoms does not necessarily represent new alterations in the thymus.

[Clinical and neurologic characteristic and principles of therapy of late-onset Myasthenia gravis].

Science.gov (United States)

Kosachev, V D; Alekseeva, T M; Khalmurzina, A N

2016-01-01

In the present work the results of the clinic-epidemiological analysis of 223 patients with the onset of the myasthenia at 60 y. o. and later, admitted and treated in the clinic of neurology for the passed 25years are represented. A dynamic growth of incidence of the late-onset myasthenia through the passed 10 years was administered. We administered a prevalence of the generalized form of the myasthenia gravis (61,5 %). The whole clinical table of the myasthenia was developed during an year in 76,7 % of the cases. A wide range of the concomitant somatic pathology in this group of the patients (especially, with a cardio-vascular pathology - 93,3 %) was found to worsen the course of the myasthenia itself. We found that the set of the therapeutic measures in myasthenia in the elderly is determined by the course of the myasthenia and the multiple organ failure due to the concomitant diseases. The scheme of complex corrective therapy of myasthenia gravis in elderly was developed.

Reconstrução do sulco interglúteo: relato de caso

OpenAIRE

Almeida Junior, Genes Lopes de

2011-01-01

INTRODUÇÃO: O conceito de beleza feminina tem mudado ao longo dos anos, porém a forma e a projeção das nádegas permanecem como símbolos de máxima feminilidade. A exposição corporal, especialmente da região glútea, tem proporcionado aumento da procura pela cirurgia de gluteoplastia. OBJETIVO: O propósito deste relato é demonstrar a correção cirúrgica de uma deformidade do sulco interglúteo, utilizando a preservação do ligamento sacrocutâneo. RELATO DO CASO: Paciente do sexo feminino, com queix...

Relatos de vivências educacionais

Directory of Open Access Journals (Sweden)

Congresso Internacional de Dislexia

2011-05-01

Full Text Available Relatos de experiências em educação, abrangendo a descrição de projeto ou vivência específica em sala de aula ou outro ambiente educacional, que envolva o trabalho com escolares com dislexia e outros transtornos da aprendizagem da leitura e escrita.Os trabalhos foram apresentados na terceira edição do Congresso Internacional de Dislexia (CID, realizado nas dependências do Centro Universitário Metodista Izabela Hendrix, em maio de 2011. 

Disability and Functional Profiles of Patients with Myasthenia Gravis Measured with ICF Classification

Science.gov (United States)

Leonardi, Matilde; Raggi, Alberto; Antozzi, Carlo; Confalonieri, Paolo; Maggi, Lorenzo; Cornelio, Ferdinando; Mantegazza, Renato

2009-01-01

The objective of this study is to describe functional profiles of patients with myasthenia gravis (MG), and the relationships among symptoms, activities and environmental factors (EF), by using WHO's International Classification of Functioning Disability and Health (ICF). Patients were consecutively enrolled at the Besta Institute of Milan, Italy.…

Diagnostic imaging and treatment of an invasive thymoma in myasthenia gravis

International Nuclear Information System (INIS)

Soetje, G.; Brinkmann, G.; Striepling, E.; Engemann, R.

1991-01-01

A 40-year old woman with an invasive thymoma and myasthenia gravis is described in this article. Chest-x-ray, CT and MRI of the mediastinum could not offer definite results on tumour malignancy. Radical surgical removal was the consequence. This revealed a tumour infiltration of the pleura and pericardium; hence an adjuvant irradiation must have been performed. Mestinon-treatment was afterwards gradually reduced. (orig.) [de

Comparison of the growth of Listeria monocytogenes in unirradiated and irradiated cook-chill roast beef and gravy at refrigeration temperatures

International Nuclear Information System (INIS)

Grant, I.R.; Nixon, C.R.; Patterson, M.F.

1993-01-01

Specific growth rates of two strains of Listeria monocytogenes in unirradiated and irradiated (2 kGy) roast beef and gravy stored at 5° and 10°C were found to be similar. However, exponential growth of L. monocytogenes after irradiation was preceded by an extended lag period of 6–9 d at 5°C and 3–4 d at 10°C, compared with lag periods of 1–2 d and <0.1 d in unirradiated beef and gravy stored similarly

Acute Respiratory Failure Induced by Magnesium Replacement in a 62-Year-Old Woman with Myasthenia Gravis.

Science.gov (United States)

Singh, Paramveer; Idowu, Olakunle; Malik, Imrana; Nates, Joseph L

2015-10-01

Magnesium is known to act at the neuromuscular junction by inhibiting the presynaptic release of acetylcholine and desensitizing the postsynaptic membrane. Because of these effects, magnesium has been postulated to potentiate neuromuscular weakness. We describe the case of a 62-year-old woman with myasthenia gravis and a metastatic thymoma who was admitted to our intensive care unit for management of a myasthenic crisis. The patient's neuromuscular weakness worsened in association with standard intravenous magnesium replacement, and the exacerbated respiratory failure necessitated intubation, mechanical ventilation, and an extended stay in the intensive care unit. The effect of magnesium replacement on myasthenia gravis patients has not been well documented, and we present this case to increase awareness and stimulate research. In addition, we discuss the relevant medical literature.

Combined short-term immunotherapy for experimental autoimmune myasthenia gravis

International Nuclear Information System (INIS)

Pestronk, A.; Drachman, D.B.; Teoh, R.; Adams, R.N.

1983-01-01

A therapeutic strategy was designed to eliminate the humoral immune response to acetylcholine receptor (AChR) in ongoing experimental autoimmune myasthenia gravis (EAMG). Rats with EAMG were treated with a protocol consisting of three components: (1) A single high dose of cyclophosphamide (200 mg/kg) was used to produce a rapid and sustained fall in the anti-AChR antibody levels by preferential destruction of antibody-producing B-lymphocytes. ''Memory'' lymphocytes were not eliminated by cyclophosphamide. (2) Irradiation (600 rads) was used to eliminate the ''memory'' cells. It eliminated the anamnestic response to a challenge with the antigen AChR. (3) Bone marrow transplantation was used to repopulate the hematopoietic system after the otherwise lethal dose of cyclophosphamide. We used bone marrow from syngeneic rats with active EAMG to simulate an autologous transplant. Rats with EAMG treated with this combined protocol showed a prompt and sustained fall in the anti-AChR antibody levels and had no anamnestic response to a challenge with AChR. Thus, an affected animal's own marrow could be stored and used later for repopulation after cyclophosphamide-irradiation treatment. This treatment eliminates the animal's ongoing immune responses and reconstitutes the immune system in its original state. The success of this approach suggests that, if their safety could be established, similar ''curative'' strategies might be developed for the treatment of patients with severe antibody-mediated autoimmune disorders, such as myasthenia gravis

Computed tomography of the thymus gland in myasthenia gravis

International Nuclear Information System (INIS)

Thorvinger, B.; Lyttkens, K.; Samuelsson, L.

1987-01-01

Sixteen consecutive patients with myasthenia gravis were examined by computed tomography (CT) before thymectomy. Surgical and histologic findings were compared with those obtained at CT. The results, like those of most other recent studies, indicate that CT is a reliable method for identifying thymoma. In all three patients with thymoma, and in five out of eight patients with hyperplasia, the lesion was observed at CT, giving an overall accuracy of 80%. Although tymic hyperplasia is a basically histological diagnosis an enlarged gland may give a correct diagnosis of this abnormality. (orig.)

Computed tomography of the thymus gland in myasthenia gravis

Energy Technology Data Exchange (ETDEWEB)

Thorvinger, B.; Lyttkens, K.; Samuelsson, L.

Sixteen consecutive patients with myasthenia gravis were examined by computed tomography (CT) before thymectomy. Surgical and histologic findings were compared with those obtained at CT. The results, like those of most other recent studies, indicate that CT is a reliable method for identifying thymoma. In all three patients with thymoma, and in five out of eight patients with hyperplasia, the lesion was observed at CT, giving an overall accuracy of 80%. Although tymic hyperplasia is a basically histological diagnosis an enlarged gland may give a correct diagnosis of this abnormality.

El envejecimiento como relato: Una invitación a la gerontología narrativa

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Feliciano Villar

2015-07-01

Full Text Available El presente artículo tiene como objetivo presentar una panorámica actual de la aplicación de un enfoque narrativo al estudio del envejecimiento. En primer lugar se delinea cuál es el objeto de conocimiento de la gerontología narrativa, que se extiende en un doble nivel interrelacionado: la identidad narrativa en la vejez por una parte, y los relatos culturales sobre el envejecimiento por otra. En segundo lugar, se describen las principales metodologías utilizadas en la gerontología narrativa, diferenciando entre aquellas interesadas en el contenido de los relatos (qué se narra de las interesadas en el proceso de elaboración dialogada de los relatos (cómo se narra. El artículo finaliza con algunas reflexiones sobre el futuro y desafíos a los que se enfrenta la gerontología narrativa.  

AChR-specific immunosuppressive therapy of myasthenia gravis.

Science.gov (United States)

Luo, Jie; Lindstrom, Jon

2015-10-15

Myasthenia gravis (MG) is an organ-specific autoimmune disease characterized by muscle fatigability. In most cases, it is mediated by autoantibodies targeting muscle nicotinic acetylcholine receptors (AChRs) at the neuromuscular junction. Experimental autoimmune myasthenia gravis (EAMG) is an animal model for MG, which is usually induced by immunization with AChR purified from fish electric organ. Pathological autoantibodies to AChRs are directed at the extracellular surface, especially the main immunogenic region (MIR). Current treatments for MG can help many but not all patients. Antigen-specific immunosuppressive therapy for MG that specifically suppresses the autoimmune response without affecting the entire immune system and avoids side effects of general immunosuppression is currently unavailable. Early attempts at antigen-specific immunosuppression for EAMG using AChR extracellular domain sequences that form epitopes for pathological autoantibodies risked provoking autoimmunity rather than suppressing it. We discovered a novel approach to specific immunosuppression of EAMG with a therapeutic vaccine consisting of bacterially-expressed human AChR cytoplasmic domains, which has the potential to specifically suppress MG without danger of causing exacerbation. This approach prevents development of chronic EAMG when initiated immediately after the acute phase of EAMG, and rapidly reverses established chronic EAMG when started during the chronic phase of EAMG. Successfully treated rats exhibited long-term resistance to re-induction of EAMG. In this review we also discuss the current understanding of the mechanisms by which the therapy works. Vaccination with AChR cytoplasmic domains in adjuvant is promising as a safe, antigen-specific, potent, effective, rapidly acting, and long lasting approach to therapy of MG. Copyright © 2015 Elsevier Inc. All rights reserved.

¿Fútbol de verdad? : Observaciones narratológicas sobre los relatos en vivo

OpenAIRE

Martínez, Matías

2011-01-01

En el relato periodístico retrospectivo de un partido de futbol –el de Chile contra México por la Copa América, el 30 de junio de 1999- se puede observar el uso de diversos elementos narratológicos que lo vuelven interesante. Los reportajes de distintos diarios chilenos contienen crónicas, historias y esquemas narrativos construidos a partir del antagonismo entre adversarios, el protagonismo de ciertos personajes y los puntos culminantes que ellos viven. Además, hay en los relatos una identif...

Diphyllobothrium latum: relato de caso no Brasil

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Emmel Vanessa Erichsen

2006-01-01

Full Text Available Difilobotriose é causada em humanos pela infecção com vermes adultos do gênero Diphyllobothrium adquiridos pelo consumo de peixe cru ou mal cozido. Diphyllobothrium latum foi confirmado pelo exame dos proglotes grávidos e típicos ovos operculados nas fezes. O paciente havia comido crustáceos e peixes. É o relato do primeiro brasileiro infectado.

Palpebral portion of the orbicularis oculi muscle to repetitive nerve stimulation testing: A potential assessment indicator in patients with generalized myasthenia gravis.

Science.gov (United States)

Yan, Chong; Song, Jie; Pang, Song; Yi, Fangfang; Xi, Jianying; Zhou, Lei; Ding, Ding; Wang, Weifeng; Qiao, Kai; Zhao, Chongbo

2018-02-01

Repetitive nerve stimulation (RNS) is a valuable diagnostic method for myasthenia gravis (MG). However, its association with clinical severity was scarcely studied. We reviewed medical records and retrospectively enrolled 121 generalized MG patients. Sensitivity of different muscles to RNS and clinical scoring systems was evaluated. RNS testing revealed facial muscles have the highest positive rate, followed by proximal muscles and distal muscles, with the palpebral portion of the orbicularis oculi muscle most sensitive. Amplitude decrement of compound muscle action potential (CMAP) in the palpebral portion of the orbicularis oculi muscle is related to quantitative myasthenia gravis (QMG) scores, MG-specific manual muscle testing (MMT) scores and myasthenia gravis-related activities of daily living (MG-ADL) scores. We suggest that RNS testing of the palpebral portion of the orbicularis oculi muscle is a potential assessment indicator in patients with generalized MG. Copyright © 2017 Elsevier Ltd. All rights reserved.

Complicating autoimmune diseases in myasthenia gravis: a review

Science.gov (United States)

Nacu, Aliona; Andersen, Jintana Bunpan; Lisnic, Vitalie; Owe, Jone Furlund; Gilhus, Nils Erik

2015-01-01

Abstract Myasthenia gravis (MG) is a rare autoimmune disease of skeletal muscle endplates. MG subgroup is relevant for comorbidity, but usually not accounted for. MG patients have an increased risk for complicating autoimmune diseases, most commonly autoimmune thyroid disease, systemic lupus erythematosus and rheumatoid arthritis. In this review, we present concomitant autoimmune disorders associated with the different MG subgroups, and show how this influences treatment and prognosis. Concomitant MG should always be considered in patients with an autoimmune disorder and developing new neuromuscular weakness, fatigue or respiratory failure. When a second autoimmune disorder is suspected, MG should be included as a differential diagnosis. PMID:25915571

Relato policial y el crimen que lo habita

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Francisco de Undurraga

2011-05-01

Full Text Available La aparición de publicidad y de novelas en serie en las portadas de periódicos franceses, apartir de 1836, marca la relación entre el género policial y los nuevos modos de visibilidad de la información en las sociedades industrializadas. Algunos ejemplos, tomados de un periódico de la época, la "Gazette des Tribunaux", permiten demostrar que la prensa estuvo desde sus comienzos vinculada a la literatura, si acaso no es una derivación de ella. E.A. Poe, creador del relato policial, conduce la retórica del morbo trabajada por Dante, Swift, Lautréamont y Baudelaire, de una apelación al lector, al orden del argumento y del procedimiento que lo estructura. En el relato policial conviven el discurso de lo que se cuenta, característico de la literatura, y el de la ciencia, pero solo en apariencia éste viene a suplantar a aquél, configurando de este modo su retórica.

"Warming yang and invigorating qi" acupuncture alters acetylcholine receptor expression in the neuromuscular junction of rats with experimental autoimmune myasthenia gravis

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Hai-peng Huang

2016-01-01

Full Text Available Myasthenia gravis is an autoimmune disorder in which antibodies have been shown to form against the nicotinic acetylcholine nicotinic postsynaptic receptors located at the neuromuscular junction. "Warming yang and invigorating qi" acupuncture treatment has been shown to reduce serum inflammatory cytokine expression and increase transforming growth factor beta expression in rats with experimental autoimmune myasthenia gravis. However, few studies have addressed the effects of this type of acupuncture on the acetylcholine receptors at the neuromuscular junction. Here, we used confocal laser scanning microscopy to examine the area and density of immunoreactivity for an antibody to the nicotinic acetylcholine receptor at the neuromuscular junction in the phrenic nerve of rats with experimental autoimmune myasthenia gravis following "warming yang and invigorating qi" acupuncture therapy. Needles were inserted at acupressure points Shousanli (LI10, Zusanli (ST36, Pishu (BL20, and Shenshu (BL23 once daily for 7 consecutive days. The treatment was repeated after 1 day of rest. We found that area and the integrated optical density of the immunoreactivity for the acetylcholine receptor at the neuromuscular junction of the phrenic nerve was significantly increased following acupuncture treatment. This outcome of the acupuncture therapy was similar to that of the cholinesterase inhibitor pyridostigmine bromide. These findings suggest that "warming yang and invigorating qi" acupuncture treatment increases acetylcholine receptor expression at the neuromuscular junction in a rat model of autoimmune myasthenia gravis.

VAV1 and BAFF, via NFκB pathway, are genetic risk factors for myasthenia gravis

DEFF Research Database (Denmark)

Avidan, Nili; Le Panse, Rozen; Harbo, Hanne F

2014-01-01

OBJECTIVE: To identify novel genetic loci that predispose to early-onset myasthenia gravis (EOMG) applying a two-stage association study, exploration, and replication strategy. METHODS: Thirty-four loci and one confirmation loci, human leukocyte antigen (HLA)-DRA, were selected as candidate genes...

Combined short-term immunotherapy for experimental autoimmune myasthenia gravis

Energy Technology Data Exchange (ETDEWEB)

Pestronk, A.; Drachman, D.B.; Teoh, R.; Adams, R.N.

1983-08-01

A therapeutic strategy was designed to eliminate the humoral immune response to acetylcholine receptor (AChR) in ongoing experimental autoimmune myasthenia gravis (EAMG). Rats with EAMG were treated with a protocol consisting of three components: (1) A single high dose of cyclophosphamide (200 mg/kg) was used to produce a rapid and sustained fall in the anti-AChR antibody levels by preferential destruction of antibody-producing B-lymphocytes. ''Memory'' lymphocytes were not eliminated by cyclophosphamide. (2) Irradiation (600 rads) was used to eliminate the ''memory'' cells. It eliminated the anamnestic response to a challenge with the antigen AChR. (3) Bone marrow transplantation was used to repopulate the hematopoietic system after the otherwise lethal dose of cyclophosphamide. We used bone marrow from syngeneic rats with active EAMG to simulate an autologous transplant. Rats with EAMG treated with this combined protocol showed a prompt and sustained fall in the anti-AChR antibody levels and had no anamnestic response to a challenge with AChR. Thus, an affected animal's own marrow could be stored and used later for repopulation after cyclophosphamide-irradiation treatment. This treatment eliminates the animal's ongoing immune responses and reconstitutes the immune system in its original state. The success of this approach suggests that, if their safety could be established, similar ''curative'' strategies might be developed for the treatment of patients with severe antibody-mediated autoimmune disorders, such as myasthenia gravis.

Raquitismo carencial: relato de caso

OpenAIRE

Porto, Juliana Antola et al.

2005-01-01

Introdução: O raquitismo é uma doença do tecido ósseo em formação, onde há falha na calcificação e conseguinte acúmulo de tecido osteóide. O raquitismo carencial é decorrente da deficiência dos precursores da vitamina D, sendo a causa de raquitismo mais facilmente evitável e tratável. Objetivo: Relatar um caso, revisar a etiopatogenia e o tratamento do raquitismo carencial. Relato de caso: Paciente masculino com 2 anos de idade, previamente hígido, consulta com dificuldade de deambular progre...

Thoracic computer tomography for the evaluation of the thymus gland in cases of myasthenia gravis

International Nuclear Information System (INIS)

Druschky, K.F.; Stadler, H.W.; Daun, H.; Erlangen-Nuernberg Univ., Erlangen

1981-01-01

Hyperplasia of the thymus gland is observed in 65% of all patients with myasthenia gravis, while the incidence of thymus tumor is reported to be 8.5-28%. Conventional radiological techniques provide little information in the diagnosis of mediastinal lesions. Even a clearly developed thymus tumor can escape clinical detection. Since March 1978 thoracic computer tomography has been performed in addition to X-rays of the chest in a series of 19 patients with myasthenia ravis, 10 women and 9 men ranging in age from 15-71 years and in 3 patients with suspected thymomas but without myasthenia gravis. These examinations were carried out with a Somatom II (Siemens) since September 1979. On the average 15-20 scans were made at the level of the upper two-third of the sternum. The chest X-rays in 2 planes revealed signs of a thymus tumor in 3 female patients. Thoracic computer tomography showed definite signs of a space-occupying lesion in the anterior mediastinum in 11 cases. At thymectomy 6 patients were found to have hyperplasia of the thymus, 2 patients had a benign thymoma and 3 patients a malignant thymoma. In 6 cases computer tomography showed only slight changes and in 5 patients no pathological findings could be demonstrated in the thymus gland. Thoracic computer tomography is a relatively harmless diagnostic measure without any risk for the patient. It has a high resolution and great accuracy in the evaluation of the thymus gland and is therefore the method of choice for the diagnosis of patients with myasthenia gravis. (orig.) [de

Thoracic computer tomography for the evaluation of the thymus gland in cases of myasthenia gravis

Energy Technology Data Exchange (ETDEWEB)

Druschky, K.F.; Stadler, H.W.; Daun, H.

1981-01-01

Hyperplasia of the thymus gland is observed in 65% of all patients with myasthenia gravis, while the incidence of thymus tumor is reported to be 8.5-28%. Conventional radiological techniques provide little information in the diagnosis of mediastinal lesions. Even a clearly developed thymus tumor can escape clinical detection. Since March 1978 thoracic computer tomography has been performed in addition to X-rays of the chest in a series of 19 patients with myasthenia ravis, 10 women and 9 men ranging in age from 15-71 years and in 3 patients with suspected thymomas but without myasthenia gravis. These examinations were carried out with a Somatom II (Siemens) since September 1979. On the average 15-20 scans were made at the level of the upper two-third of the sternum. The chest X-rays in 2 planes revealed signs of a thymus tumor in 3 female patients. Thoracic computer tomography showed definite signs of a space-occupying lesion in the anterior mediastinum in 11 cases. At thymectomy 6 patients were found to have hyperplasia of the thymus, 2 patients had a benign thymoma and 3 patients a malignant thymoma. In 6 cases computer tomography showed only slight changes and in 5 patients no pathological findings could be demonstrated in the thymus gland. Thoracic computer tomography is a relatively harmless diagnostic measure without any risk for the patient. It has a high resolution and great accuracy in the evaluation of the thymus gland and is therefore the method of choice for the diagnosis of patients with myasthenia gravis.

Anestesia em paciente com síndrome de Rubinstein-Taybi: relato de caso

OpenAIRE

Oliveira, Carlos Rogério Degrandi; Elias, Luciana

2005-01-01

JUSTIFICATIVA E OBJETIVOS: A síndrome de Rubinstein-Taybi (SRT) é uma doença genética causada por uma mutação ou apagamento do cromossomo 16, caracterizada por retardo físico e mental, anormalidades craniofaciais e hálux e polegares largos. Há pouca informação sobre esta síndrome na literatura anestésica. O objetivo deste relato foi apresentar a conduta anestésica em paciente submetido à cirurgia odontológica e discutir as características de interesse para a anestesia nesses pacientes. RELATO...

Auto-relato de erros em tarefas de leitura: efeitos de um treino de correspondência.

OpenAIRE

Camila Domeniconi

2006-01-01

Quando crianças com história de fracasso escolar relatam seus resultados em leitura de palavras, a maioria delas tende a relatar a maior parte as respostas como corretas, mesmo que tenham cometido erros. Este estudo investigou as variáveis que influenciam a fidedignidade desses relatos e tentou treinar a correspondência para assegurar relatos correspondentes de erros, bem como de acertos. As sessões experimentais apresentaram diversas palavras em uma tela do computador. O compu...

Current and emerging therapies for the treatment of myasthenia gravis

Directory of Open Access Journals (Sweden)

Renato Mantegazza

2011-03-01

Full Text Available Renato Mantegazza, Silvia Bonanno, Giorgia Camera, Carlo AntozziDepartment of Neuromuscular Diseases and Neuroimmunology, Fondazione Istituto Neurologico Carlo Besta, Milan, ItalyAbstract: Myasthenia gravis (MG is an autoimmmune disease in which autoantibodies to different antigens of the neuromuscular junction cause the typical weakness and fatigability. Treatment includes anticholinesterase drugs, immunosuppression, immunomodulation, and thymectomy. The autoimmune response is maintained under control by corticosteroids frequently associated with immunosuppressive drugs, with improvement in the majority of patients. In case of acute exacerbations with bulbar symptoms or repeated relapses, modulation of autoantibody activity by plasmapheresis or intravenous immunoglobulins provides rapid improvement. Recently, techniques removing only circulating immunoglobulins have been developed for the chronic management of treatment-resistant patients. The rationale for thymectomy relies on the central role of the thymus. Despite the lack of controlled studies, thymectomy is recommended as an option to improve the clinical outcome or promote complete remission. New videothoracoscopic techniques have been developed to offer the maximal surgical approach with the minimal invasiveness and hence patient tolerability. The use of biological drugs such as anti-CD20 antibodies is still limited but promising. Studies performed in the animal model of MG demonstrated that several more selective or antigen-specific approaches, ranging from mucosal tolerization to inhibition of complement activity or cellular therapy, might be feasible. Investigation of the transfer of these therapeutic approaches to the human disease will be the challenge for the future.Keywords: myasthenia gravis, therapy, immunosuppression, thymectomy, plasmapheresis

Dificuldade de intubação traqueal em paciente com craniossinostose: relato de caso

OpenAIRE

Gifoni, Cláudia Luisa; Nascimento, Henrique S; Mizumoto, Nelson

2001-01-01

JUSTIFICATIVA E OBJETIVOS: Existem formas complexas de craniossinostose acompanhadas de malformações da face e das vias aéreas, que podem levar a dificuldade de intubação traqueal (IOT). O objetivo deste relato é apresentar um caso de intubação traqueal difícil, em criança submetida à cirurgia para correção da craniossinostose. RELATO DO CASO: Criança com 57 dias, 3700 gramas, perímetro cefálico 39 cm, ASA II, com craniossinostose, retrognatia, macroglosia e exoftalmo bilateral, dificuldade i...

Thermal inactivation of ileal loop-reactive Clostridium perfringens type A strains in phosphate buffer and beef gravy.

Science.gov (United States)

Bradshaw, J G; Peeler, J T; Twedt, R M

1977-09-01

The thermal resistance of spore crops produced from each of two ileal loop-reactive strains of Clostridium perfringens type A was determined in two suspending vehicles consisting of 0.067 M (pH 7.0) phosphate buffer and a commercial beef gravy. D115.6 values obtained in buffer and enumerated after pretreatment with sodium ethylenediaminetetraacetate and recovery in plating medium containing lysozyme were two- to threefold greater than those obtained without this treatment. D115.6 values obtained with beef gravy were less than those obtained in buffer with or without lysozyme; however, the D98.9 and D104.4 values were 1.3 to 2 times greater than those obtained in buffer with lysozyme. The z values were within the ranges reported by previous investigators.

Alcoolismo após cirurgia bariátrica: relato de caso

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Juliana Garbayo dos Santos

Full Text Available RESUMO Objetivo Discutir, a partir do relato de um caso de alcoolismo iniciado após tratamento cirúrgico de obesidade mórbida, a hipótese da “transferência de compulsão”. Segundo essa teoria, obesos submetidos à cirurgia bariátrica tendem a transferir sua “compulsão” dos alimentos para o álcool ou outras drogas, aumentando o risco de desenvolvimento de transtornos relacionados a substâncias. Evidências de que a alimentação hedônica (caracterizada pelo consumo exagerado de alimentos palatáveis na ausência de fome é regulada pelos mesmos sistemas cerebrais envolvidos no alcoolismo, aliadas a relatos anedóticos de alcoolismo após cirurgia bariátrica, alimentam essa hipótese. Métodos Relato de caso individual. O paciente concordou com a publicação do relato de caso e assinou um Termo de Consentimento Livre e Esclarecido. Resultados Homem de 25 anos, sem histórico prévio de abuso de álcool, evoluiu com alcoolismo grave quatro anos após a cirurgia bariátrica. Havia antecedentes familiares de transtornos relacionados a substâncias. Conclusão A cirurgia bariátrica (particularmente com derivação em Y de Roux pode alterar o metabolismo do etanol e implicar mudanças no estilo de vida e no nível de estresse. Somados, esses fatores podem contribuir para o surgimento de padrões nocivos de consumo de álcool; entretanto, é incontestável que a correção da obesidade traz enormes benefícios para as diversas áreas da vida do paciente. A detecção de fatores de risco para abuso e dependência alcoólica, como história familiar positiva, é essencial para identificar pacientes que necessitarão maior aconselhamento pré-operatório e seguimento pós-operatório cuidadoso.

Cognitive fatigue in patients with myasthenia gravis.

Science.gov (United States)

Jordan, Berit; Schweden, Tabea L K; Mehl, Theresa; Menge, Uwe; Zierz, Stephan

2017-09-01

Cognitive fatigue has frequently been reported in myasthenia gravis (MG). However, objective assessment of cognitive fatigability has never been evaluated. Thirty-three MG patients with stable generalized disease and 17 healthy controls underwent a test battery including repeated testing of attention and concentration (d2-R) and Paced Auditory Serial Addition Test. Fatigability was based on calculation of linear trend (LT) reflecting dynamic performance within subsequent constant time intervals. Additionally, fatigue questionnaires were used. MG patients showed a negative LT in second d2-R testing, indicating cognitive fatigability. This finding significantly differed from stable cognitive performance in controls (P fatigue was significantly higher in MG patients compared with controls (P fatigue is not correlated with objective findings. Muscle Nerve 56: 449-457, 2017. © 2016 Wiley Periodicals, Inc.

Recombinant human acetylcholine receptor alpha-subunit induces chronic experimental autoimmune myasthenia gravis.

Science.gov (United States)

Lennon, V A; Lambert, E H; Leiby, K R; Okarma, T B; Talib, S

1991-04-01

A synthetic gene encoding the 210 N-terminal residues of the alpha-subunit of the nicotinic acetylcholine receptor (AChR) of human skeletal muscle was cloned into an inducible expression plasmid to produce a fusion protein in high yield in Escherichia coli. Like native human AChR, the recombinant human alpha 1-210 protein induced AChR-binding, AChR-modulating, and AChR-blocking autoantibodies in rats when injected once intradermally as an emulsion in CFA, with Bordetella pertussis vaccine as supplementary adjuvant. The minimum dose of recombinant protein required to induce biochemical signs of experimental autoimmune myasthenia gravis (EAMG) with 100% incidence was 2.2 micrograms. With 6.6 to 22 micrograms, serum levels of autoantibodies were persistent, and clinically apparent EAMG lasted more than a month. Clinical, electrophysiological, and biochemical indices of EAMG induced by doses of 66 micrograms or more were more uniformly severe and persistent, with 33% fatality. Rats receiving a control extract of E. coli containing plasmid without the alpha 1-210 codon insert, with adjuvants, did not develop autoantibodies or signs of EAMG. This highly reproducible new model of EAMG induced by a recombinant human autoantigen should be valuable for testing Ag-specific immunotherapeutic strategies that might be applicable to treating acquired myasthenia gravis in humans.

Pathogenesis of myasthenia gravis: update on disease types, models, and mechanisms [version 1; referees: 2 approved

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William D. Phillips

2016-06-01

Full Text Available Myasthenia gravis is an autoimmune disease of the neuromuscular junction (NMJ caused by antibodies that attack components of the postsynaptic membrane, impair neuromuscular transmission, and lead to weakness and fatigue of skeletal muscle. This can be generalised or localised to certain muscle groups, and involvement of the bulbar and respiratory muscles can be life threatening. The pathogenesis of myasthenia gravis depends upon the target and isotype of the autoantibodies. Most cases are caused by immunoglobulin (IgG1 and IgG3 antibodies to the acetylcholine receptor (AChR. They produce complement-mediated damage and increase the rate of AChR turnover, both mechanisms causing loss of AChR from the postsynaptic membrane. The thymus gland is involved in many patients, and there are experimental and genetic approaches to understand the failure of immune tolerance to the AChR. In a proportion of those patients without AChR antibodies, antibodies to muscle-specific kinase (MuSK, or related proteins such as agrin and low-density lipoprotein receptor-related protein 4 (LRP4, are present. MuSK antibodies are predominantly IgG4 and cause disassembly of the neuromuscular junction by disrupting the physiological function of MuSK in synapse maintenance and adaptation. Here we discuss how knowledge of neuromuscular junction structure and function has fed into understanding the mechanisms of AChR and MuSK antibodies. Myasthenia gravis remains a paradigm for autoantibody-mediated conditions and these observations show how much there is still to learn about synaptic function and pathological mechanisms.

Additional neutral vector boson in the 7-dimensional theory of gravy-electro-weak interactions

International Nuclear Information System (INIS)

Gavrilov, V.R.

1988-01-01

Possibilities of manifestation of an additional neutron vector boson, the existence of which is predicted by the 7-dimensional theory of gravy-electro-weak interactions, are analyzed. A particular case of muon neutrino scattering on a muon is considered. In this case additional neutral current manifests both at high and at relatively low energies of particle collisions

Factors affecting outcome in myasthenia gravis.

Science.gov (United States)

Andersen, Jintana B; Gilhus, Nils Erik; Sanders, Donald B

2016-12-01

Information from myasthenia gravis (MG) patients treated and evaluated for at least 2 years between 1980 and 2014 was reviewed to assess the effect of demographics, antibody status and titer, thymus histology, and clinical severity on outcome after 2, 5, and 10 years of treatment. Among 268 patients, 74% had acetylcholine receptor antibodies, 5% had muscle specific tyrosine kinase-antibodies, and 22% had neither. Optimal outcome was achieved by 64% of patients at 2 years of follow-up, 73% at 5 years, and 75% after 10 years. Optimal outcome was achieved more often in patients with late onset, in those who had thymectomy, and in those with ocular-only disease at maximum severity. The only consistent independent predictor of optimal outcome was onset after age 50 years on multivariate analysis. Prognosis is favorable for the majority of MG patients, regardless of age, maximum disease severity, or antibody status. Muscle Nerve, 2016 Muscle Nerve 54: 1041-1049, 2016. © 2016 Wiley Periodicals, Inc.

Bloqueio bilateral do nervo pudendo para hemorroidectomia em paciente acondroplásico: relato de caso

OpenAIRE

Morais, Bruno Salomé de; Cruvinel, Marcos Guilherme Cunha; Silva, Yerkes Pereira; Diniz, Dener Augusto; Castro, Carlos Henrique Viana de

2006-01-01

JUSTIFICATIVA E OBJETIVOS: O nanismo acondroplásico ou acondroplasia é a forma mais comum de nanismo e ocorre na maioria dos casos por alteração genética espontânea. A anestesia desses pacientes apresenta várias particularidades. O objetivo do presente relato foi descrever um caso de paciente acondroplásico, com história prévia de intervenção cirúrgica da coluna para descompressão medular, submetido a hemorroidectomia através de bloqueio bilateral dos nervos pudendos. RELATO DO CASO: Paciente...

Raquianestesia total após bloqueio do plexo lombar por via posterior: relato de caso

OpenAIRE

Duarte, Leonardo Teixeira Domingues; Saraiva, Renato Ângelo

2006-01-01

JUSTIFICATIVA E OBJETIVOS: O bloqueio do plexo lombar pode ser bastante útil em procedimentos cirúrgicos do quadril, da coxa e do joelho, mas exige prática do anestesiologista tendo em vista suas potenciais complicações. O presente relato tem por objetivo apresentar um caso de raquianestesia total após bloqueio do plexo lombar pela via posterior e discutir o provável mecanismo fisiopatológico. RELATO DO CASO: Paciente do sexo masculino, 34 anos, 97 kg, estado físico ASA I, programado para art...

Tratamento dos casos graves de miastenia: ação do ACTH e cortisona. Considerações a propósito de três casos

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José Lamartine de Assis

1956-03-01

Full Text Available São apresentados 3 casos de miastenia grave em que os recursos terapêuticos clássicos não deram resultado favorável. Os 3 pacientes foram, então, submetidos ao tratamento pelo ACTH e cortisona; em todos houve remissão da sintomatologia, remissão que perdurou até cêrca de um mês após a suspensão da hormonioterapia. O tratamento foi repetido várias vêzes de acôrdo com a evolução da doença. É de notar que em 2 pacientes (casos 1 e 2, foram estabelecidas doses mínimas de manutenção controladas durante cerca de 40 dias. O ACTH foi usado por via intravenosa em doses variáveis de 2,5 a 25 mg, diluído em 250 a 1000 ml de sôro glicosado isotônico, na velocidade de 20 a 25 gotas por minuto. Em todos os casos, durante a hormonioterapia, foi administrada prostigmina em doses muito pequenas.

RAQUITISMO HIPOFOSFATÊMICO: RELATO DE CASO

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Marta Liliane de Almeida Maia

2018-03-01

Full Text Available RESUMO Objetivo: O raquitismo hipofosfatêmico precisa ser precocemente diagnosticado porque seu tratamento previne sequelas incapacitantes. Este relato alerta para a doença. Relato de caso: Relato de perfil metabólico, depuração de creatinina, estado nutricional e desenvolvimento pôndero-estatural de paciente com características clínico-laboratoriais de raquitismo hipofosfatêmico, atendido em ambulatório de tubulopatias por período de 12 meses. Chegou ao serviço após tempo prolongado acamado, dependente de ventilação mecânica e com perfil metabólico ósseo alterado. Terapêutica consistiu na administração de fósforo (inicial: 65 mg/kg/dia, final: 24,2 mg/kg/dia, cálcio (inicial: 127 mg/kg/dia, final: 48,4 mg/kg/dia e calcitriol (inicial: 0,06 mcg/kg/dia, final: 0,03 mcg/kg/dia, e a análise constou da descrição das consultas, utilizando-se mediana de exames laboratoriais e dados antropométricos. Observou-se nítida melhora inicial do padrão respiratório do paciente, que evoluiu com ventilação espontânea e deambulação autônoma; com exames laboratoriais: cálcio (mg/dL inicial 7,1, final 10,1; fósforo (mg/dL inicial 1,7, final 3,2; magnésio (mg/dL inicial 1,5, final 2,1; paratormônio (pg/L inicial 85,8, final 52,7; fosfatase alcalina (UI/L inicial 12660, final 938; e melhora do desenvolvimento pôndero-estatural (escore Z: E/I inicial: -6,05, final -3,64; P/I: inicial -2,92, final -1,57 com presença de litíase transitória. A depuração de creatinina (mL/min/1,73 m2sc foi constante durante o seguimento. O tratamento propiciou benefícios clínicos, bioquímicos e nutricionais, mas, apesar da boa resposta inicial, a família abandonou o seguimento por dois anos, apresentando o paciente piora da deambulação e das deformidades esqueléticas. Comentários: Não apenas diagnóstico precoce é necessário, como também a adesão ao tratamento é fundamental para o sucesso na condução dessa patologia.

46,XY hypergonadotropic hypogonadism and myasthenia gravis.

Science.gov (United States)

Lichiardopol, Corina; Herlea, V; Ioan, Virginia; Tomulescu, V; Mixich, F

2006-01-01

Both hypergonadotropic hypogonadism and myasthenia gravis can be parts of type II autoimmune polyendocrine syndrome and association between the two disorders has been reported in few cases. A 14 year old male patient with a personal history of bilateral cryptorchidism and ptosis was referred for delayed puberty. Clinical examination revealed eunuchoid habitus, small, soft testes, gynecomastia, ptosis, a myasthenic deficit score of 22.5 points and an IQ of 84 points. Decreased testosterone (0.064 ng/mL) and elevated LH (64.5 mUI/mL) were consistent with hypergonadotropic hypogonadism and karyotype was normal: 46,XY. Thyroid function, haematologic evaluation, BUN, electrolytes, and glycemia were in the normal range. Therapy consisted of anticholinesterase inhibitors, immunosuppressants, corticotherapy, testosterone; thoracoscopic thymectomy was performed showing thymic lymphoid hyperplasia on histopathologic examination. Myasthenic score improved (12.5 points), progressive virilization occurred, and a year later the patient presented with cushingoid features and obesity.

Plasmapheresis in the treatment of myasthenia gravis: retrospective study of 26 patients Plasmaférese no tratamento da miastenia grave: estudo retrospectivo de 26 pacientes

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Rosana Carandina-Maffeis

2004-06-01

Full Text Available We analyzed the experience of Unicamp Clinical Hospital with plasma exchange (PE therapy in myasthenia gravis (MG. About 17.8 % of a totality of MG patients had PE performed: 26 cases, 19 women and seven men. The mean age-onset of MG was 28 years, extremes 11 and 69. Minimum deficit observed in the group was graded IIb (O & G or IIIa (MGFA scale. One patient had prethymectomy PE. In seven the procedures were performed due to myasthenic crisis and in 18 patients due to severe myasthenic symptoms or exacerbation of previous motor deficit. Two patients were also submitted to chronic PE considering refractoriness to other treatments. Twenty-six patients had 44 cycles of PE and 171 sessions. The mean number of sessions was 3.9 (SD ± 1.4 each cycle; median 5, extremes 2 and 6. The mean time by session was 106,5 minutes (SD ± 35.2; median 100.5 (extremes of 55 and 215. The mean volume of plasma exchanged in each session was 2396 ml (SD ± 561; median 2225 (extremes 1512 and 4500. Side effects occurred: reversible hypotension (seven cases, mild tremor or paresthesias (seven cases. Infection and mortality rates due to PE were zero. All patients had immediate benefit of each PE cycle and usually they also received prednisone or other immunosuppressors. Good acceptance of the procedure was observed in 80.7% of patients.Analisamos a experiência do Hospital das Clínicas da Unicamp com plasmaferese: (PF na miastania grave (MG. 17,8 % do total dos casos de MG submeteu-se a PF, 26 casos, 19 mulheres e sete homens. A idade média de início da MG foi 28 anos (extremos 11 e 69. O menor déficit clínico foi IIb (O & G e IIIa (MGFA. A PF foi indicada no pré-operatório de timectomia em um caso e em sete devido a crise miastênica. Em 18 casos, com MG generalizada e sintomas bulbares ou com exacerbação de déficit prévio, a PF foi indicada como intervenção aguda. Em dois pacientes desse grupo ela foi indicada também em regime crônico de ciclos mensais

[Case of awake intubation in semi-sitting position for a patient with myasthenia gravis combined with cervical disc hernia utilizing Pentax-AWS Airwayscope].

Science.gov (United States)

Komasawa, Nobuyasu; Ueki, Ryusuke; Tomita, Yukihiko; Kaminoh, Yoshiroh; Tashiro, Chikara

2011-01-01

We report a case of awake intubation utilizing Pentax-AWS Airwayscope in semi-sitting position. A 74-year-old man with myasthenia gravis and cervical disc hernia was scheduled for distal gastrectomy under general anesthesia. He could not move his head due to severe cervical disc hernia and also could not sufficiently breathe due to the fatigue of respiratory muscles by myasthenia gravis in supine position. With fentanyl bolus administration and lidocaine spray for laryngotracheal anesthesia, we performed awake intubation in semi-sitting position with AWS from cranial side. The patient did not buck during intubation and no hemodynamic change was observed.

Auto-relato e relato de informante secundário na avaliação da saúde em idosos

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Renata Jardim

2010-12-01

Full Text Available OBJETIVO: Analisar se o modelo explicativo para a avaliação da saúde do idoso com base no auto-relato é comparável com o modelo de relato do informante secundário e se a auto-avaliação de saúde do informante secundário influencia a avaliação da saúde do idoso. MÉTODOS: Estudo transversal com 230 pares idoso-informante secundário realizado em Belo Horizonte, MG, em 2007. Foram investigadas variáveis sociodemográficas e de saúde dos idosos por meio de entrevista estruturada. Utilizou-se regressão logística múltipla para analisar associação com auto-avaliação da saúde do idoso como ruim e com as informações prestadas pelo informante secundário. RESULTADOS: No modelo com base no auto-relato, a variável mais fortemente associada à avaliação da saúde do idoso como ruim foi a presença de restrições ou incapacidade para realizar atividades relacionadas à vida diária e/ou à mobilidade. No modelo baseado no informante secundário, a variável explicativa mais relevante foi o número de doenças crônicas apresentadas pelo idoso. Além disso, a chance de o informante secundário avaliar a saúde do idoso como ruim foi três vezes maior quando ele auto-avaliou sua saúde da mesma forma. CONCLUSÕES: Os resultados mostram diferenças importantes entre o modelo da avaliação da saúde do idoso com base nas respostas do próprio indivíduo e nas do informante secundário. O idoso tende a valorizar suas restrições ou incapacidade de realizar atividades da vida diária/mobilidade, enquanto o informante secundário tende a valorizar o diagnóstico de doenças crônicas. O informante secundário com pior auto-avaliação da saúde apresenta chance quase três vezes maior de relatar a saúde do idoso da mesma forma. Assim, informações auto-relatadas refletem melhor a condição de saúde do indivíduo do que se relatadas por informantes secundários.

Heat shock and thermotolerance of Escherichia coli O157:H7 in a model beef gravy system and ground beef.

Science.gov (United States)

Juneja, V K; Klein, P G; Marmer, B S

1998-04-01

Duplicate beef gravy or ground beef samples inoculated with a suspension of a four-strain cocktail of Escherichia coli O157:H7 were subjected to sublethal heating at 46 degrees C for 15-30 min, and then heated to a final internal temperature of 60 degrees C. Survivor curves were fitted using a linear model that incorporated a lag period (TL), and D-values and 'time to a 4D inactivation' (T4D) were calculated. Heat-shocking allowed the organism to survive longer than non-heat-shocked cells; the T4D values at 60 degrees C increased 1.56- and 1.50-fold in beef gravy and ground beef, respectively. In ground beef stored at 4 degrees C, thermotolerance was lost after storage for 14 h. However, heat-shocked cells appeared to maintain their thermotolerance for at least 24 h in ground beef held to 15 or 28 degrees C. A 25 min heat shock at 46 degrees C in beef gravy resulted in an increase in the levels of two proteins with apparent molecular masses of 60 and 69 kDa. These two proteins were shown to be immunologically related to GroEL and DnaK, respectively. Increased heat resistance due to heat shock must be considered while designing thermal processes to assure the microbiological safety of thermally processed foods.

Video-assisted thoracoscopic surgery or transsternal thymectomy in the treatment of myasthenia gravis?

Science.gov (United States)

Zahid, Imran; Sharif, Sumera; Routledge, Tom; Scarci, Marco

2011-01-01

A best evidence topic in thoracic surgery was written according to a structured protocol. The question addressed was how video-assisted thoracoscopic surgery (VATS) compares to median sternotomy in the surgical management of patients with myasthenia gravis (MG)? Overall 74 papers were found using the reported search, of which 15 represented the best evidence to answer the clinical question. The authors, journal, date and country of publication, patient group studied, study type, relevant outcomes and results are tabulated. We conclude that VATS produces equivalent postoperative mortality and complete stable remission (CSR) rates, with superior results in terms of hospital stay, operative blood loss and patient satisfaction at the expense of a doubling of operative time. Six studies comparing VATS and transsternal sternotomy in non-thymomatous myasthenia gravis (NTMG) patients found VATS to have lower operative blood loss (73.8±70.7 vs. 155.3±91.7 ml; P0.05). One study comparing video-assisted thoracoscopic extended thymectomy to transsternal thymectomy in only thymoma-associated myasthenia gravis (T-MG) patients found equivalent CSR (11.3 vs. 8.7%, P=0.1090) at six-year follow-up. Thymoma recurrence rate (9.64%) was not significantly different (P=0.1523) between the two groups. Eight studies comparing VATS and transsternal approach in mixed T-MG and NTMG patients found a lower hospital stay (1.9±2.6 vs. 4.6±4.2 days, P<0.001), reduced need for postoperative medication (76.5 vs. 35.7%, P=0.022), lower intensive care unit stay (1.5 vs. 3.2 days, P=0.018), greater symptom improvement (100 vs. 77.9%, P=0.019) and better cosmetic satisfaction (100 vs. 83, P=0.042) with VATS. In concordance with NTMG and T-MG alone patient groups, VATS and transsternal methods had equivalent complication rates (23 vs. 19%, P=0.765) with no mortalities in either group. Even though VATS has a longer operative time (268±51 vs. 177±92 min, P<0.05), its improved cosmesis, reduced need

Clinical characteristics of patients with myasthenia gravis associated with other autoimmune diseases

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Li-li WANG

2014-10-01

Full Text Available Objective To analyze the clinical characteristics, drug efficacy and prognosis of patients with myasthenia gravis (MG associated with other autoimmune diseases. Methods Eighty-three MG patients were divided into 2 groups. One group included MG patients with autoimmune diseases (AIDMG, N = 24, and the other included MG patients without autoimmune diseases (NAIDMG, N = 59. Firstly, clinical features such as sex, age of onset, initial symptoms and thymus abnormalities were compared between patients with AIDMG and NAIDMG. Secondly, effect of different therapies, including pyridostigmine, corticosteroids, immunoglobulin, immunosuppressants and thymectomy was compared between 2 groups. Finally, prognosis including relapse rate and recurrence time during the first 2 years after MG onset was compared. Whether and when ocular myasthenia gravis (OMG progressing to general myasthenia gravis (GMG and the first onset of GMG symptoms during the first 2 years were also compared between 2 groups.  Results The difference of gender predominance (χ2 = 8.467, P = 0.004, ptosis affecting left or right or both sides (χ2 = 9.830, P = 0.007 and disease course within 2 years after onset (χ2 = 15.255, P = 0.001 between AIDMG group and NAIDMG group were statistically significant. Other clinical features such as age of onset (χ2 = 1.728, P = 0.228, initial symptoms (χ2 = 0.252, P = 0.791, thymus abnormalities (χ2 = 3.200, P = 0.202 were not significantly different between 2 groups. Differences of therapeutical effect such as pyridostigmine (χ2 = 0.411, P = 0.395, corticosteroids (χ2 = 0.156, P = 0.513, immunoglobulin (χ2 = 0.359, P = 0.462, immunosuppressants (χ2 = 0.081, P = 0.526 and thymectomy (χ2 = 0.337, P = 0.391 between 2 groups were not statistically significant. The ratio of OMG progressing to GMG (χ2 = 1.826, P = 0.148, time of progressing (Fisher's exact test: P = 0.639, first onset symptom (Fisher's exact test: P = 0.196 and recurrence

Autoimmune Thyroiditis and Myasthenia Gravis

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Angela Lopomo

2017-07-01

Full Text Available Autoimmune diseases (AIDs are the result of specific immune responses directed against structures of the self. In normal conditions, the molecules recognized as “self” are tolerated by immune system, but when the self-tolerance is lost, the immune system could react against molecules from the body, causing the loss of self-tolerance, and subsequently the onset of AID that differs for organ target and etiology. Autoimmune thyroid disease (ATD is caused by the development of autoimmunity against thyroid antigens and comprises Hashimoto’s thyroiditis and Graves disease. They are frequently associated with other organ or non-organ specific AIDs, such as myasthenia gravis (MG. In fact, ATD seems to be the most associated pathology to MG. The etiology of both diseases is multifactorial and it is due to genetic and environmental factors, and each of them has specific characteristics. The two pathologies show many commonalities, such as the organ-specificity with a clear pathogenic effect of antibodies, the pathological mechanisms, such as deregulation of the immune system and the implication of the genetic predisposition. They also show some differences, such as the mode of action of the antibodies and therapies. In this review that focuses on ATD and MG, the common features and the differences between the two diseases are discussed.

Autoimmune Thyroiditis and Myasthenia Gravis

Science.gov (United States)

Lopomo, Angela; Berrih-Aknin, Sonia

2017-01-01

Autoimmune diseases (AIDs) are the result of specific immune responses directed against structures of the self. In normal conditions, the molecules recognized as “self” are tolerated by immune system, but when the self-tolerance is lost, the immune system could react against molecules from the body, causing the loss of self-tolerance, and subsequently the onset of AID that differs for organ target and etiology. Autoimmune thyroid disease (ATD) is caused by the development of autoimmunity against thyroid antigens and comprises Hashimoto’s thyroiditis and Graves disease. They are frequently associated with other organ or non-organ specific AIDs, such as myasthenia gravis (MG). In fact, ATD seems to be the most associated pathology to MG. The etiology of both diseases is multifactorial and it is due to genetic and environmental factors, and each of them has specific characteristics. The two pathologies show many commonalities, such as the organ-specificity with a clear pathogenic effect of antibodies, the pathological mechanisms, such as deregulation of the immune system and the implication of the genetic predisposition. They also show some differences, such as the mode of action of the antibodies and therapies. In this review that focuses on ATD and MG, the common features and the differences between the two diseases are discussed. PMID:28751878

Dissecção da artéria basilar: relato de caso

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TOGNOLA WALDIR ANTONIO

2000-01-01

Full Text Available Relato de um caso de dissecção da artéria basilar, documentado com neuroimagem (tomografia computadorizada do crânio, ressonância magnética e angiografia digital com subtração de imagem.

Relatos digitales: activando las competencias comunicativa, narrativa y digital en la formación inicial del profesorado

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María Esther del Moral

2016-01-01

Full Text Available Los relatos digitales constituyen una técnica narrativa con gran potencial educativo al adoptar fórmulas creativas que integran información multiformato e instrumentos tecnológicos para comunicar ideas. Esta investigación evalúa el nivel de competencia comunicativa, narrativa y digital ligado al diseño de relatos en entornos digitales, alcanzado por estudiantes del Grado de Maestro de Primaria (N=143 -tras participar en una experiencia de creación colaborativa-, utilizando una rúbrica integrada por 28 indicadores: 12 relacionados con la competencia comunicativa (comunicación escrita y oral, 6 con la narrativa, y otros 10 específicos con la digital. Los resultados evidencian que si bien presentan gran destreza con las tecnologías, casi un tercio registra niveles medio-bajos en la competencia comunicativa. Se detectan dificultades en la elaboración y adaptación del guión escrito de los relatos al registro elegido -pobre vocabulario y precaria puntuación-, junto a limitaciones en la dicción al locutar los diálogos, no logrando imprimir expresividad al relato. Respecto a la competencia narrativa, solo un 40% muestra originalidad al resolver las tramas y construir personajes. Sin duda, este tipo de experiencias innovadoras ofrece un escenario idóneo para desarrollar habilidades narrativas y creativas en contextos digitales, y brinda numerosas oportunidades para la formación de los futuros docentes.

Anestesia venosa total (AVT) em lactente com doença de Werdnig-Hoffmann: relato de caso

OpenAIRE

Resende, Marco Antonio Cardoso de; Silva, Elizabeth Vaz da; Nascimento, Osvaldo José Moreira; Gemal, Alberto Esteves; Quintanilha, Giseli; Vasconcelos, Eliana Maria

2010-01-01

JUSTIFICATIVA E OBJETIVOS: A doença de Werdnig-Hoffmann é a causa mais comum de hipotonia no lactente e quando presente logo após o nascimento tem pior prognóstico. Fraqueza muscular simétrica, arreflexia e fasciculações da língua são característicos. A maioria dos lactentes morre antes dos dois anos por insuficiência respiratória. O presente relato apresenta um caso com técnica venosa total durante anestesia. RELATO DO CASO: Paciente feminina, branca, um ano, 10 kg, estado físico ASA III, co...

The Co-Existence of Myasthenia Gravis in Patients with Myositis: A Case Series

Science.gov (United States)

Paik, Julie J.; Corse, Andrea M.; Mammen, Andrew L.

2014-01-01

Objective Myositis and myasthenia gravis (MG) are both autoimmune disorders presenting with muscle weakness. Rarely, they occur simultaneously in the same patient. Since the management of myasthenia gravis differs from that of myositis, it is important to recognize when patients have both diseases. We reviewed the cases of 6 patients with both myositis and MG to identify clinical features that suggest the possibility of co-existing MG in myositis patients. Methods We identified 6 patients with dermatomyositis or polymyositis and MG. We reviewed their medical records to assess their clinical presentations, laboratory findings, and electrophysiological features. Results All 6 patients had definite dermatomyositis or polymyositis by the criteria of Bohan and Peter as well as electrophysiologic and/or serologic confirmation of MG. Among overlap patients, 5/6 (83%) had bulbar weakness, 2/6 (33%) had ptosis, and 1/6 (17%) had diplopia. Fatigable weakness was noted by 5/6 (83%) patients. Treatment with pyridostigmine improved symptoms in 5/6 (83%). High dose steroids were associated with worsening weakness in 2/6 (33%) patients. Conclusions Prominent bulbar symptoms, ptosis, diplopia, and fatigable weakness should suggest the possibility of MG in patients with myositis. A suspicion of MG may be confirmed through appropriate electrophysiologic and laboratory testing. In those with myositis-MG overlap, high dose steroids may exacerbate symptoms and pryidostigmine may play an important therapeutic role. PMID:24412588

Maintenance plasma exchange treatment for muscle specific kinase antibody positive myasthenia gravis patients.

Science.gov (United States)

Yamada, Chisa; Teener, James W; Davenport, Robertson D; Cooling, Laura

2015-10-01

Anti-muscle specific kinase antibody positive myasthenia gravis (MuSK MG) is often characterized by a relatively severe and progressive course, refractoriness to standard myasthenia gravis (MG) medications, and an increased risk of myasthenic crisis. We report here successful management of three MuSK MG patients using maintenance therapeutic plasma exchange (TPE) treatment for up to 4.5 years. The study was a 5-year retrospective review of all MG patients treated with TPE between 2008 and 2013 at University of Michigan. Inclusion criteria of MuSK MG were positive for anti-MuSK antibodies and a diagnosis of MuSK MG by staff neurologists. Patient data included age, gender, diagnostic testing results, medications, and the dates and response to TPE treatments. A total of 153 MG patients underwent at least one course of TPE between 2008 and 2013. A total of 12 patients (7.8%) were positive for anti-MuSK antibodies. Patients were predominantly female (83.3%) and a median age of onset was 46-years old. Three MuSK MG patients were successfully managed with maintenance TPE. Maintenance TPE may be an effective option for MuSK MG patients. The key of successful maintenance treatment at our institution has been to tailor the TPE frequency for each individual, and to modify the treatment interval in conjunction with medical management. © 2014 Wiley Periodicals, Inc.

Púrpura em paciente com estrongiloidíase disseminada Purpura in patient with disseminated strongiloidiasis

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Luciano C. Ribeiro

2005-05-01

Full Text Available A infecção pelo Strongyloides stercoralis em associação com imunosupressão pode manifestar-se com lesões em múltiplos órgãos e sistemas, caracterizando a forma disseminada da doença. Lesões cutâneas não são freqüentemente relatadas e, se presentes, manifestam-se como rash e petéquias. Púrpuras bem definidas são pouco descritas. No presente trabalho é descrito um caso de estrongiloidíase disseminada, com acometimento cutâneo em forma de púrpura, que se desenvolveu em um paciente timectomizado e usuário crônico de corticosteróide devido à miastenia gravis.The association of systemic corticosteroid therapy and dissemination of Strongyloides stercoralis has been increasingly documented in the literature. Skin involvement in disseminated strongyloidiasis has been reported and the most commonly described cutaneous manifestations are rash and petechial eruptions. We present a case of an immunosuppressed man that developed disseminated strongyloidiasis with extensive purpura.

Doença de Moyamoya e anestesia com sevoflurano fora do centro cirúrgico: relato de caso

OpenAIRE

Machado, Sheila Braga; Mendes, Florentino Fernandes; Angelini, Adriana de Campos

2002-01-01

JUSTIFICATIVA E OBJETIVOS: A doença de Moyamoya é uma desordem cerebrovascular progressiva que representa um desafio anestésico em virtude da precária circulação cerebral destes pacientes, constituindo-se numa importante causa de acidente vascular cerebral em indivíduos jovens. O objetivo deste relato é apresentar o caso de um paciente com doença de Moyamoya que foi submetido à anestesia geral com sevoflurano para procedimento diagnóstico fora do centro cirúrgico. RELATO DO CASO: Criança com ...

Criteria for Postoperative Mechanical Ventilation After Thymectomy in Patients With Myasthenia Gravis: A Retrospective Analysis.

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Chigurupati, Keerthi; Gadhinglajkar, Shrinivas; Sreedhar, Rupa; Nair, Muraleedharan; Unnikrishnan, Madathipat; Pillai, Manjusha

2018-02-01

To determine the criteria for postoperative mechanical ventilation after thymectomy in patients with Myasthenia Gravis. Retrospective study. Teritiary care centre. 77 Myasthenia gravis patients operated for thymectomy were studied. After obtaining clearance from Institutional ethics committee, medical records of 77 patients with MG, who were operated for thymectomy between January 2005 and December 2015 were reviewed in a retrospective manner. Perioperative variables collected from the patient records were demographic data, duration of the disease, Osserman and Genkin classification, Anti-acetylcholine antibody (AChR) positivity, preoperative daily dose of drug, history of preoperative myasthenic crisis, preoperative vital capacity, technique of anesthesia, drugs used for anesthesia, perioperative complications, and duration of postoperative mechanical ventilation. The patients were divided into two groups, group I and group II consisting of those who required postoperative ventilation for 300 minutes, respectively. The determinants of prolonged postoperative ventilation were studied. The requirement of mechanical ventilation was higher in patients with higher Osserman's grade of myasthenia gravis. Duration of the disease had no effect on the duration of mechanical ventilation in myasthenic patients post thymectomy (p = 0.89). The patients with a preoperative history of myasthenic crisis had a requirement for prolonged mechanical ventilation (p=0.03). Patients with preoperative vital capacity mechanical ventilation with p values mechanical ventilation (p=0.026). Preoperative dose of pyridostigmine and the choice of continuation or discontinuation of antcholinesterases on the day of surgery had no influence on the duration of mechanical ventilation (p value of 0.19 and 0.36 respectively). Epidural analgesia intra and postoperatively significantly reduced the requirement of mechanical ventilation (p=0.006). The predictors of postoperative ventilation in myasthenic

Hemangioma de úvula: relato de um caso

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G. Sobrinho Fernando P.

2003-01-01

Full Text Available O hemangioma é o mais comum tumor de origem vascular, benigno, freqüentemente diagnosticado em crianças, e com predileção pela cabeça e pescoço, mas que raramente tem origem na úvula. Neste órgão, apresenta risco de traumatismo local e sangramento. Este artigo relato um caso de hemangioma de úvula num adulto jovem com disfagia orofaríngea progressiva e sensação de corpo estranho na garganta, tratado com sucesso por ressecção cirúrgica do tumor com bisturi de radiofreqüência.

Audiologia em comunidade: relato de experiência

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Barros,Paulo Marcelo Freitas de; Cavalcante,Tícia Cassiany Ferro; Andrade,Aline Fernanda de

2010-01-01

OBJETIVO: caracterizar queixas, sintomas e fatores de risco relacionados à perda auditiva em crianças com suspeita de perda auditiva no Distrito Sanitário I do Recife/Brasil. MÉTODOS: relato de experiência de atuação fonoaudiológica em parceria com Agentes Comunitários de Saúde - ACS que foram capacitados sobre saúde auditiva para identificar crianças com queixas de audição. Foram realizadas visitas aos domicílios indicados e ao posto de saúde e entrevistadas 80 mães obtendo-se informações a ...

Síndrome da disfunção apical reversível (Takotsubo Transient ventricular dysfunction (Takotsubo cardiomyopathy

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José Marconi Almeida de Sousa

2005-04-01

Full Text Available Homem portador de miastenia gravis, internado por descompensação da doença de base, em insuficiência respiratória aguda. Na evolução, apresentou quadro sugestivo de infarto agudo do miocárdio, com alterações eletrocardiográficas e enzimáticas compatíveis com o diagnóstico. Submetido a coronariografia de urgência, não evidenciou obstrução coronariana grave, entretanto o ventrículo esquerdo apresentava disfunção sistólica importante, com alteração característica pela ventriculografia da síndrome descrita como disfunção ventricular transitória ou síndrome de Takotsubo. Na evolução, houve completa recuperação das alterações eletrocardiográficas e da função ventricular sistólica avaliada pelo ecocardiograma, confirmando a síndrome.The patient was a male with myasthenia gravis, hospitalized with acute respiratory failure due to decompensation of the underlying disease. He evolved with findings suggestive of acute myocardial infarction, with electrocardiographic and enzymatic alterations compatible with that diagnosis. The patient underwent emergency coronary angiography, which showed no severe coronary obstruction, although his left ventricle had significant systolic dysfunction with characteristic alterations, on ventriculography, of the syndrome described as transient ventricular dysfunction or Takotsubo syndrome. On evolution, complete recovery of the electrocardiographic alterations and systolic ventricular function assessed on echocardiography occurred, confirming the syndrome.

Dermatomyositis and myastenia gravis: An uncommon association with therapeutic implications.

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Sangüesa Gómez, Clara; Flores Robles, Bryan Josué; Méndez Perles, Clara; Barbadillo, Carmen; Godoy, Hildegarda; Andréu, José Luis

2015-01-01

The association of dermatomyositis with myasthenia gravis (MG) is uncommon, having been reported so far in only 26 cases. We report the case of a 69 year-old man diagnosed with MG two years ago and currently treated with piridostigmyne. The patient developed acute proximal weakness, shoulder pain and elevated creatine-kinase (CK). He also developed generalized facial erythema and Gottron's papules. Laboratory tests showed positive antinuclear and anti-Mi2 antibodies. Further analysis confirmed CK levels above 1000 U/l. The clinical management of the patient and the therapeutic implications derived from the coexistence of both entities are discusssed. Copyright © 2014 Elsevier España, S.L.U. All rights reserved.

Regulatory T cells in multiple sclerosis and myasthenia gravis.

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Danikowski, K M; Jayaraman, S; Prabhakar, B S

2017-06-09

Multiple sclerosis (MS) is a chronic debilitating disease of the central nervous system primarily mediated by T lymphocytes with specificity to neuronal antigens in genetically susceptible individuals. On the other hand, myasthenia gravis (MG) primarily involves destruction of the neuromuscular junction by antibodies specific to the acetylcholine receptor. Both autoimmune diseases are thought to result from loss of self-tolerance, which allows for the development and function of autoreactive lymphocytes. Although the mechanisms underlying compromised self-tolerance in these and other autoimmune diseases have not been fully elucidated, one possibility is numerical, functional, and/or migratory deficits in T regulatory cells (Tregs). Tregs are thought to play a critical role in the maintenance of peripheral immune tolerance. It is believed that Tregs function by suppressing the effector CD4+ T cell subsets that mediate autoimmune responses. Dysregulation of suppressive and migratory markers on Tregs have been linked to the pathogenesis of both MS and MG. For example, genetic abnormalities have been found in Treg suppressive markers CTLA-4 and CD25, while others have shown a decreased expression of FoxP3 and IL-10. Furthermore, elevated levels of pro-inflammatory cytokines such as IL-6, IL-17, and IFN-γ secreted by T effectors have been noted in MS and MG patients. This review provides several strategies of treatment which have been shown to be effective or are proposed as potential therapies to restore the function of various Treg subsets including Tr1, iTr35, nTregs, and iTregs. Strategies focusing on enhancing the Treg function find importance in cytokines TGF-β, IDO, interleukins 10, 27, and 35, and ligands Jagged-1 and OX40L. Likewise, strategies which affect Treg migration involve chemokines CCL17 and CXCL11. In pre-clinical animal models of experimental autoimmune encephalomyelitis (EAE) and experimental autoimmune myasthenia gravis (EAMG), several strategies

Identidad y colonialidad en los relatos de Alberto Alabí

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Irene López

2011-06-01

Full Text Available En este artículo proponemos un análisis de algunos relatos del escritor argentino Alberto Alabí desde la noción de colonialidad en sus tres dimensiones —del poder, del saber y del ser— y sus implicaciones en las formaciones identitarias. Consideramos que los conflictos que estos relatos textualizan nos permiten comprender algunas de las problemáticas sociales candentes en el espacio norteño, como la racialización de la diferencia y, consecuentemente, el color de piel, tanto en la subalternización de sujetos y prácticas como en la construcción de representaciones identitarias.In this paper we propose an analysis of short stories of an argentinean writter, Alberto Alabí, from the notion of coloniality of power, of knowledge and being and its implications for identity formations. We believe that the conflicts textualized in these short stories permit us to understand many of social problems in the north of Argentina, such as the role played by the racialization of difference and, consequently, the color of skin in the subalternization of subjects and practices as well as in construction of identity representations.

[A comparative analysis of the informative value of anti-AChR-antibody radioimmunoassay and laser correlation spectroscopy in myasthenia gravis].

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Alchinova, I B; Yakovenko, E N; Sidnev, D V; Dedaev, S Yu; Sanadze, A G; Karganov, M Yu

An aim of the study was to compare informative value of traditional approach (anti-AChR antibody radioimmunoassay) and evaluation of metabolic shifts by laser correlation spectroscopy in myasthenia gravis. The search for the relationship between the disease severity in 77 patients, 12-80 years and the distribution pattern of subfraction serum components revealed three informative zones: 6-15, 27-67, and 127-223 nm. In patients without disturbances of vital functions, the contribution of the first zone particles into light scatter increases and that of the third zone particles decreases. Considerable differences attaining the level of statistical significance in zones 6 and 20 nm were revealed in the spectra of serum from patients with myasthenia gravis of the same severity with and without thymoma. This opens prospects for dynamic monitoring of the efficiency of therapy.

Indicadores de desempenho de bibliotecas no campo da saúde: relato de estudo piloto na Fiocruz

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Maria Cristina S. Guimarães

Full Text Available Relato de projeto de pesquisa, em curso, sobre a implantação de um programa de avaliação de desempenho de bibliotecas no campo da saúde. Uma equipe de pesquisadores e bibliotecários tomou como ponto de partida a Norma ISO 11620 para derivar um conjunto mínimo de indicadores adequados à avaliação de três diferentes bibliotecas especializadas da Fundação Oswaldo Cruz. O relato enfatiza a importância do processo de cooperação entre os profissionais, ingrediente essencial para sustentabilidade da iniciativa e os desafios futuros que se avizinham.

Appearance of Systemic Lupus Erythematosus in Patients with Myasthenia Gravis following Thymectomy: Two Case Reports

OpenAIRE

Park, Mi-Jeong; Kim, Yun-A; Lee, Shin-Seok; Kim, Byeong-Chae; Kim, Myeong-Kyu; Cho, Ki-Hyun

2004-01-01

We report two cases of systemic lupus erythematosus (SLE) in myasthenia gravis (MG) patients who had undergone thymectomy. SLE developed in the patients 3 months or 13 yr after thymectomy, and polyarthritis was the main clinical manifestation of SLE. Both patients fulfilled at least four of the revised criteria for the classification of SLE. In this report, we describe two postthymectomy lupus patients and perform a comparative review of previous cases.

A functional SNP in the regulatory region of the decay-accelerating factor gene associates with extraocular muscle pareses in myasthenia gravis

KAUST Repository

Heckmann, J M; Uwimpuhwe, H; Ballo, R; Kaur, M; Bajic, Vladimir B.; Prince, S

2009-01-01

Complement activation in myasthenia gravis (MG) may damage muscle endplate and complement regulatory proteins such as decay-accelerating factor (DAF) or CD55 may be protective. We hypothesize that the increased prevalence of severe extraocular

Does perioperative high-dose prednisolone have clinical benefits for generalized myasthenia gravis?

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Sekine, Yasuo; Kawaguchi, Naoki; Hamada, Chikuma; Sekiguchi, Hiromi; Yasufuku, Kazuhiro; Iyoda, Akira; Shibuya, Kiyoshi; Fujisawa, Takehiko

2006-06-01

The purpose of this study was to clarify the clinical benefits of perioperative administration of high-dose prednisolone (PSL) combined with extended thymectomy on the long-term outcomes of 116 consecutive patients with generalized myasthenia gravis (MG). A retrospective review was conducted on 116 patients diagnosed with generalized MG who received alternate-day oral administration of high-dose PSL (100 mg/alternate days) and had undergone transsternal extended thymectomy. Incidences of postoperative myasthenic crisis, adverse effects of steroid, long-term outcomes, such as complete stable remission (CSR), pharmacologic remission (PR) or improvement (Imp), and disease recurrence after CSR were evaluated. Six patients (5.2%) experienced post-thymectomy myasthenic crisis. Crude cumulative CSR and PR + CSR rates were 44.8 and 62.7%, respectively. Life table analysis showed that 41.8, 52.8 and 63.4% of the patients were in CSR at 3, 5 and 10 years, respectively. Multivariate analysis revealed that age and pretreatment classification according to the Myasthenia Gravis Foundation of America (MGFA) criteria tended to be independent predictors of CSR. There were 6.9% with compressive vertebral fracture, 13.8% with cataract, and 5.2% with steroid-induced diabetes. Life table analysis revealed that recurrence rates after CSR were 36.8 and 46.0% at 3 and 5 years, respectively. Patients with thymoma had a significantly higher rate of recurrence than those without thymoma (p = 0.001). Alternate-day administration of high-dose prednisolone reduced the risk of post-thymectomy myasthenic crisis. Presence of thymoma was a risk factor for MG recurrence after CSR.

Analysis of azaarenes in pan fried meat and its gravy by liquid chromatography with fluorescence detection.

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Błaszczyk, Urszula; Janoszka, Beata

2008-07-01

A method for analysis of six azaarenes (benzo[h]quinoline, benzo[a]acridine, benzo[c]acridine, dibenzo[a,c]acridine, dibenzo[a,j]acridine and dibenzo[a,h]acridine) in thermally treated high-protein food has been described. The clean-up procedure used based on alkaline hydrolysis, tandem solid phase extraction on columns filled with Extrelut - diatomaceous earth and cation exchanger (propyl sulfonic acid), enabled a selective isolation of carcinogenic compounds belonging to benzoacridines and dibenzoacridines from samples of cooked meat and its gravy. The isolated fractions of aza-PAHs were analysed by high-performance liquid chromatography with fluorescence detection. The detection limits for the azaarenes were between 0.0001ng and 0.005ng loaded on column. The recoveries for the four-ring and five-ring azaarenes were from 55% to 67%. Two types of dishes prepared from pork by pan-frying were investigated. Total contents of the benzoacridines and dibenzoacridines determined in cooked meat were 1.57 and 2.50ng/g in collar and chop samples, respectively; their gravies contained 0.34 and 0.59ng of these azaarenes per g of cooked meat. Copyright © 2007 Elsevier Ltd. All rights reserved.

Low antioxidant status of serum bilirubin, uric acid, albumin and creatinine in patients with myasthenia gravis.

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Yang, Dehao; Su, Zhongqian; Wu, Shengjie; Bi, Yong; Li, Xiang; Li, Jia; Lou, Kangliang; Zhang, Hongyu; Zhang, Xu

2016-12-01

Oxidative stress and low antioxidant status play a major role in the pathogenesis of inflammatory and autoimmune diseases. Myasthenia gravis (MG) is an autoimmune condition targeting the neuromuscular junction, and its antioxidant status is still controversial. Our study aimed to investigate the correlation between the clinical characteristics of MG and the serum antioxidant status of bilirubin (Tbil, Dbil and Ibil), uric acid, albumin and creatinine. We measured serum antioxidant molecule levels of bilirubin (Tbil, Dbil and Ibil), uric acid, albumin and creatinine in 380 individuals, including 166 MG and 214 healthy controls. We found that MG patients had significantly lower serum levels of bilirubin (Tbil, Dbil and Ibil), uric acid, albumin and creatinine than healthy controls, whether male or female. Moreover, it was also shown in our study that uric acid, albumin and creatinine levels in patients with MG were correlated with disease activity and classifications performed by the Myasthenia Gravis Foundation of America. Our findings demonstrated that serum levels of bilirubin (Tbil, Dbil and Ibil), uric acid, albumin and creatinine were reduced in patients with MG. This suggested an active oxidative process in MG patients who had low antioxidant status.

Myasthenic crisis: report of 24 cases Crise miastênica: relato de 24 casos

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Lineu Cesar Werneck

2002-09-01

Full Text Available Myasthenic crisis (MC is a life-threatening complication of myasthenia gravis (MG with a high mortality rate. The aim of our study was to review the different therapeutics approaches in the treatment of MC and their impact in the final outcome. We reviewed the medical files of patients diagnosed with MG admitted between February 1993 and October 1997, who developed MC. Sex, mean age, disease's duration, functional scale, symptoms preceding the crisis, crisis therapy in each set and mortality were then analysed. There were 24 patients who developed MC, 21 females and 3 males, with 1 neonatal, 1 congenital sporadic, 17 juvenile/adult, 3 over 50 years and 2 with thymoma. Dysphagia, dysphonia and dysartria were the most common symptoms preceding the crisis. A precipitating factor was elicited in 8 cases and the most common was infection (upper airway infection, urinary tract infection and pneumonia. 16 patients needed a nasogastric tube and 9 had a tracheostomy performed. 24 patients used anticholinesterase drugs, 21 prednisone, 7 immunosuppressive agents, 5 plasmapheresis, 3 human hyperimune gamma immunoglobulin and 12 had thymectomy. A good response was obtained in 13, satisfactory in 7 and there were 4 deaths. We concluded that in spite of all the therapeutics options, there were non statistically significant differences in the outcome of patients that underwent thymectomy and those who did not.A crise miastênica (CM é uma complicação preocupante da miastenia grave (MG que apresenta altos índices de mortalidade. Neste estudo revisamos as diferentes abordagens no tratamento da CM e seu impacto no resultado final. Levantamos os dados dos pacientes com MG que desenvolveram CM admitidos entre fevereiro de 1993 e outubro de 1997. Foram analisados as interrelações do sexo, idade media, duração da doença, escala funcional, sintomas e procedimentos precedendo as crises, terapêutica empregada e mortalidade. Foram encontrados 24 casos que

Hipomelanose de Ito - relato de um caso

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Adriana S. de Almeida

2001-02-01

Full Text Available Objetivo: Os autores têm como objetivo relatar um caso de hipomelanose de Ito (HI, uma síndrome neurocutânea rara, com alterações neurológicas e cromossômicas associadas ao comprometimento cutâneo e pneumonias de repetição. Relato do caso: Este relato é referente a um paciente masculino, 1 ano e 11 meses, internado no Hospital Universitário São Vicente de Paulo por broncopneumonia bilateral. Ao exame foram observadas máculas hipocrômicas na pele compatíveis com HI, além de atraso do desenvolvimento neuropsicomotor. O paciente foi submetido à biópsia incisional de pele das lesões do abdômen, eletroencefalograma, ressonância magnética e estudo citogenético. Resultados: Os exames histológico e imunoistoquímico evidenciaram ausência de melanina e diminuição de melanócitos em áreas focais da epiderme, respectivamente. O eletroencefalograma apresentou disfunção córtico-subcortical difusa. A ressonância magnética do encéfalo foi compatível com cisto aracnoídeo em região temporal. O cariótipo evidenciou mosaicismo cromossômico com uma linhagem normal (46,XY e uma linhagem celular que apresentava deleção intersticial nas bandas 22.2 - 24.2 do braço longo do cromossomo 10 (25%. Conclusões: Os autores, com o presente estudo, destacam a importância das lesões de pele na definição etiológica das desordens neuropediátricas.

Anti-musk positive myasthenia gravis and three semiological cardinal signs

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André P.C. Matta

2017-01-01

Full Text Available Myasthenia gravis (MG is a relatively uncommon disorder with an annual incidence of approximately 7 to 9 new cases per million. The prevalence is about 70 to 165 per million. The prevalence of the disease has been increasing over the past five decades. This is thought to be due to better recognition of the condition, aging of the population, and the longer life span of affected patients. MG causes weakness, predominantly in bulbar, facial, and extra-ocular muscles, often fluctuating over minutes to weeks, in the absence of wasting, sensory loss, or reflex changes. The picture of fluctuating, asymmetric external ophthalmoplegia with ptosis and weak eye closure is virtually diagnostic of myasthenia. We report an atypical MG case with three semiological cardinal signs.

Myasthenia gravis: 75Se-seleno-methionine scanning of thymus gland

International Nuclear Information System (INIS)

Szobor, A.; Fornet, B.

1986-01-01

The 75 Se-seleno-methionine isotope thymus scanning was examined in a series of patients with myasthenia gravis. The patients were given 4 μC/kg hence a total of 250-300 μC 75 Se-seleno-methionine, intravenously. The method proved useful and informative in the diagnostics of myasthenia. Prior to thymectomy, the thymic tumour or a large gland could be observed and some hints could be gained for the biological activity of the gland. After the operation, the success of thymectomy could be checked and later a possible recidive could be shown or excluded. In non-operative cases the change in thymic activity could be followed which was an important sign of a malignant or tumorous growth of the thymus. (author)

Exposição ao vivo no tratamento de agorafobia: relato de caso

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Gustavo J. Fonseca D'El Rey

Full Text Available Relato de caso, em que a Exposição ao Vivo foi utilizada no tratamento de paciente com diagnóstico de agorafobia. O tratamento foi realizado em doze sessões, apresentando êxito no uso dessa técnica comportamental.

Severe Preeclampsia in the Setting of Myasthenia Gravis

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Adam J. Lake

2017-01-01

Full Text Available Myasthenia gravis (MG is a rare autoimmune disease that leads to progressive muscle weakness and is common during female reproductive years. The myasthenic mother and her newborn must be observed carefully, as complications during all stages of pregnancy and the puerperium may arise suddenly. Preeclampsia is a common obstetrical condition for which magnesium sulfate is used for seizure prophylaxis. However, magnesium sulfate is strongly contraindicated in MG as it impairs already slowed nerve-muscle connections. Similarly, many first-line antihypertensive medications, including calcium channels blockers and β-blockers, may lead to MG exacerbation. This case describes the effective obstetrical management of a patient with MG who developed severe preeclampsia. The effective use of levetiracetam and various antihypertensive medications including intravenous labetalol is described. A review of the ten reported cases of MG complicated by preeclampsia is examined to aggregate observations of clinical care, with focus on delivery methods, anticonvulsants, and antihypertensive medications.

Three Types of Striational Antibodies in Myasthenia Gravis

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Shigeaki Suzuki

2011-01-01

Full Text Available Myasthenia gravis (MG is caused by antibodies that react mainly with the acetylcholine receptor on the postsynaptic site of the neuromuscular junction. A wide range of clinical presentations and associated features allow MG to be classified into subtypes based on autoantibody status. Striational antibodies, which react with epitopes on the muscle proteins titin, ryanodine receptor (RyR, and Kv1.4, are frequently found in MG patients with late-onset and thymoma. Antititin and anti-RyR antibodies are determined by enzyme-linked immunosorbent assay or immunoblot. More recently, a method for the detection of anti-Kv1.4 autoantibodies has become available, involving 12–15% of all MG patients. The presence of striational antibodies is associated with more severe disease in all MG subgroups. Anti-Kv1.4 antibody is a useful marker for the potential development of lethal autoimmune myocarditis and response to calcineurin inhibitors. Detection of striational antibodies provides more specific and useful clinical information in MG patients.

Animal models of myasthenia gravis: utility and limitations

Science.gov (United States)

Mantegazza, Renato; Cordiglieri, Chiara; Consonni, Alessandra; Baggi, Fulvio

2016-01-01

Myasthenia gravis (MG) is a chronic autoimmune disease caused by the immune attack of the neuromuscular junction. Antibodies directed against the acetylcholine receptor (AChR) induce receptor degradation, complement cascade activation, and postsynaptic membrane destruction, resulting in functional reduction in AChR availability. Besides anti-AChR antibodies, other autoantibodies are known to play pathogenic roles in MG. The experimental autoimmune MG (EAMG) models have been of great help over the years in understanding the pathophysiological role of specific autoantibodies and T helper lymphocytes and in suggesting new therapies for prevention and modulation of the ongoing disease. EAMG can be induced in mice and rats of susceptible strains that show clinical symptoms mimicking the human disease. EAMG models are helpful for studying both the muscle and the immune compartments to evaluate new treatment perspectives. In this review, we concentrate on recent findings on EAMG models, focusing on their utility and limitations. PMID:27019601

A case of radiation-related pneumonia and bilateral tension pneumothorax after extended thymectomy and adjuvant radiation for thymoma with myasthenia gravis

International Nuclear Information System (INIS)

Nakasone, Etsuko; Nakayama, Masayuki; Bando, Masashi; Endo, Shunsuke; Hironaka, Mitsugu; Sugiyama, Yukihiko

2010-01-01

A 62-year-old man was admitted to our hospital with a 2-month history of progressive cough and dyspnea. He had undergone thymectomy for thymoma with myasthenia gravis. Adjuvant radiation of 50 Gy had been performed until 6 months before the symptoms developed. Chest computed tomography showed infiltrative findings even outside the irradiated area. We diagnosed radiation-related pneumonia, and 30 mg per day prednisolone was initiated. On the final day, he developed bilateral tension pneumothorax. After chest tube drainage, the right S 5 bulla was resected with video-assisted thoracoscopic surgery (VATS). The right pneumothorax caused the bilateral tension pneumothorax, because the right and left thoracic cavity communicated in the anterior mediastinum after thymectomy. We should be aware of the risk of bilateral tension pneumothorax following radiation-related pneumonia after extended thymectomy and adjuvant radiation in patients with myasthenia gravis. (author)

Doença de Moyamoya e anestesia com sevoflurano fora do centro cirúrgico: relato de caso Enfermedad de Moyamoya y anestesia con sevoflurano fuera del centro quirúrgico: relato de caso Moyamoya disease and sevoflurane anesthesia outside the surgery center: case report

OpenAIRE

Sheila Braga Machado; Florentino Fernandes Mendes; Adriana de Campos Angelini

2002-01-01

JUSTIFICATIVA E OBJETIVOS: A doença de Moyamoya é uma desordem cerebrovascular progressiva que representa um desafio anestésico em virtude da precária circulação cerebral destes pacientes, constituindo-se numa importante causa de acidente vascular cerebral em indivíduos jovens. O objetivo deste relato é apresentar o caso de um paciente com doença de Moyamoya que foi submetido à anestesia geral com sevoflurano para procedimento diagnóstico fora do centro cirúrgico. RELATO DO CASO: Criança com ...

Raquianestesia total após bloqueio do plexo lombar por via posterior: relato de caso Raquianestesia total después del bloqueo del plexo lumbar por vía posterior: relato de caso Total spinal block after posterior lumbar plexus blockade: case report

OpenAIRE

Leonardo Teixeira Domingues Duarte; Renato Ângelo Saraiva

2006-01-01

JUSTIFICATIVA E OBJETIVOS: O bloqueio do plexo lombar pode ser bastante útil em procedimentos cirúrgicos do quadril, da coxa e do joelho, mas exige prática do anestesiologista tendo em vista suas potenciais complicações. O presente relato tem por objetivo apresentar um caso de raquianestesia total após bloqueio do plexo lombar pela via posterior e discutir o provável mecanismo fisiopatológico. RELATO DO CASO: Paciente do sexo masculino, 34 anos, 97 kg, estado físico ASA I, programado para art...

Síndrome de Asperger: relato de um caso

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Letícia Viana Pereira

2012-04-01

Full Text Available Objetivo: O presente estudo tem como real objetivo o relato de um caso de Síndrome de Asperger comprado às características descritas na literatura. Metodologia: A partir de um relato de um caso atendido na Clínica Escola de Fonoaudiologia do Centro Universitário Metodista Izabela Hendrix, determinadas características foram comparadas com o descrito na literatura pertinente. Os trabalhos foram lidos, fichados e agrupados, segundos algumas características de abordagem, para posterior análise e comparação com o caso clínico relatado. Discussão e resultados: As crianças com Síndrome de Asperger são, em geral, diferentes entre si. Freqüentemente procedimentos realizados que podem ser indicados para uma criança seriam inadequados para outra. Assim, sugestões apresentadas pela literatura devem ser consideradas para melhorar a assistência à criança, como a compreensão mais abrangente e a reflexão aprofundada em relação a cada caso em específico. Conclusão: O acompanhamento e tratamento fonoaudiológico nesses casos, é de suma importância para o desenvolvimento o mais próximo possível do considerado formal na linguagem e na comunicação destes indivíduos. Vivências clínicas mostram as estratégias que podem ser sugeridas para estas crianças, lembrando que casa criança possui suas particularidades, que devem sempre ser levadas em consideração.

Using Magnetic Forces to Probe the Gravi-response of Swimming Paramecium

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Guevorkian, Karine; Valles, James M., Jr.

2004-03-01

Paramecium Caudatum, a single celled ciliate, alters its swimming behavior when subjected to different gravity environments (e.g. centrifugation and micro-gravity). To dissect the mechanisms behind this gravi-response and that of other biological systems, we are developing the use of magnetic body forces as a means of creating a rapidly tunable, simulated variable gravity environment. Since biological materials are weakly diamagnetic, we must subject them to intense inhomogeneous magnetic fields with characteristic field-field gradient products on the order of 16 T^2/cm. We will describe experiments on Paramecium Caudatum in which we adjust their net buoyancy with magnetic forces and measure the resulting changes in their swimming behavior.

Los Relatos Zapatistas y su Vínculo con la Oralidad Tradicional

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Ezequiel Maldonado

2001-01-01

Full Text Available Se examina, en la primera parte, la presencia inherente de una tradición oral en las literaturas indias. Una oralidad que permite en el 2001 no sólo la continuidad cul tural en el seno comunitario sino que, en su forma escrita, trasmite relatos e historias que atrapan a escuchas y lectores sin demeritar planos estéticos. En la segunda parte se ejemplifica esta persistente oralidad en los relatos de El Viejo An tonio a través de variados recursos estilísticos: uso de frases recurrentes, trastocamiento de tiempo y espacio, improvisación, sentido de hu mor, ausencia de un orden cronológico etcétera. En sentido estricto, se habla de literatura in dia aquella relatada y escrita por los propios indios; sin em bargo, en el presente caso, Marcos será la voz, el comunicador o “traductor” de An tonio. El ¡Ya basta! indio de 1994 abrió sendas inéditas e impulsó la voz de los sin rostro en el pan orama de la literatura nacional.

Derechos colectivos en el empleo público. Construcción de relatos en la justicia constitucional costarricense. 1990-2015

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Castro Méndez, Mauricio

2016-01-01

Resumen Este artículo propone una lectura crítica de la jurisprudencia de la Sala Constitucional sobre derechos colectivos del empleo público costarricense a partir de un análisis argumentativo y desde la perspectiva crítica del derecho. Más concretamente, el estudio se enfoca en el relato histórico mediante el cual la Sala Constitucional justifica su jurisprudencia y lo problematiza a partir de los estudios académicos disponibles. El análisis muestra que su relato se basa en opciones ideológ...

Mapuche y ranqueles en la Argentina: relatos sobre su pasado y presente

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Graciana Pérez Zavala

2008-07-01

Full Text Available En este artículo se planteo la reconstrucción de los relatos del pasado por parte de indígenas que viven en el territorio argentino, particularmente los mapuche y ronqueles. Para explicar estos variaciones en la interpretación del pasado por parte de las diferentes organizaciones indigenas, se examina la "Conquista del Desierto" (1878-1885, en tanto acontecimiento nodal en la estructuración de las relaciones interétnicas actuales en Argentina. En primer lugar, se sintetizan varios elementos de la historia de los indígenas de Pampa y Patagonia en los años previos a su sometimiento. Posteriormente, se describen los acontecimientos ligados a la "Conquista del Desierto", teniendo en cuenta al Estado argentino y a las diferentes "tribus". Luego, se caracteriza la situación de los indigenas después de 1885, y para finalizar, se analizan los relatos de los mapuche y ranqueles contemporaneos en el marco de su resurgimiento como pueblos preexistentes al Estado Nacional argentino.

Oral and parenteral pyridostigmine in preparing Myasthenia Gravis patients for thymectomy;a randomized Clinical trial

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Tadjeddein A

2007-06-01

Full Text Available Background: Respiratory failure and crisis is one of major complications of thymectomy in myasthenia gravis patients. There are different medication regimes for preparing these patients for surgery and reducing post-operative side effects. The goal of this study is to compare respiratory complications of oral vs. Parenteral preoperative administration of anticholinesterase agents for thymectomy in myasthenia gravis patients. Methods: This randomized controlled trial included 101 patients in class IIA or IIB of myasthenia gravis according to the Osserman classification system. The control group fasted for eight hours before surgery and oral anticholines-terase agents were replaced with parenteral ones. The case group also fasted for 8 hours before surgery, but pyridostigmine was continued at its usual dose until the time of operation and the last dose was given to patients with a small amount of water in the operating room on the operating bed. Results: There was no statistically meaningful difference between the two groups in terms of age, sex and pathologic findings. In comparison, the mean hospital stay for the case group was 3.98 days and 6.34 for the control group (p value = 0.003. There were eight cases of respiratory crisis or failure (16% in the control group but only 1 case (2% was observed in case group (p value = 0.014. Only one patient in the case group required re-intubation after the surgery; however, six patients in control group were re-intubated (p value = 0.053. Plasmapheresis was required for five patients in the control group and one patient in the case group (p value = 0.098. Tracheostomy was performed on two patients in the control group to accommodate prolonged intubation, but none of the case group required this procedure. Conclusion: This study shows that continuing oral anticholinesterase agents up to the time of operation, with the last dose at the operative theater, lowers the incidence of post-operative myasthenia

La ira de Dios: sobre ‘Relatos salvajes’ de Damián Szifrón

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Gonzalo Aguilar

2017-12-01

Full Text Available “La ira de Dios (sobre ‘Relatos salvajes’ de Damián Szifrón” propone un debate sobre una de las películas argentinas más exitosas de todos los tiempos. En polémica con las lecturas que señalaban y cuestionaban el carácter prepolítico del film y su uso de las pasiones más bajas del público, este ensayo busca otro camino: por un lado, analizar la ira como uno de los capitales políticos más importantes y decisivos de la sociedad contemporánea. Por otro, investigar los vínculos entre la película Relatos salvajes y la pospolítica en la sociedad del espectáculo.

Osteocondromatose em gato: relato de caso

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S. Winter

Full Text Available RESUMO A osteocondromatose é caracterizada por nódulos únicos ou múltiplos decorrentes de um crescimento ósseo excessivo benigno. É encontrada em cães, gatos, equinos e humanos. Em felinos, tem maior incidência dos dois aos quatro anos de idade. A etiologia em gatos está relacionada ao vírus da leucemia felina, e também já foi encontrada relação com o fibrossarcoma. A manifestação clínica depende do local acometido e do tamanho da lesão. O diagnóstico definitivo é por meio de histopatologia e o prognóstico é desfavorável, pois ocorrem muitas recidivas. Este relato de caso objetiva descrever a apresentação dessa enfermidade em um felino jovem.

Mobilidade internacional na graduação em medicina: relato de experiência

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Iago Gonçalves Ferreira

2017-08-01

Full Text Available Introdução: A mobilidade acadêmica nas universidades apresenta-se como uma ferramenta para a internacionalização do ensino superior brasileiro, promovendo a inserção dos estudantes universitários em diferentes contextos culturais e educacionais, agregando tanto novos conhecimentos teóricos como habilidades sociais e linguísticas. Relato de experiência: Este artigo trata‑se de um relato de experiência de um estudante de medicina viabilizado pelo programa de mobilidade internacional Ciência Sem Fronteiras, realizado no curso de Promoção da Saúde do Waterford Institute of Technology, na cidade de Waterford, Irlanda, no período de setembro de 2014 a julho de 2015. O relato apresenta as atividades acadêmicas desenvolvidas na instituição, bem como analisa a contribuição dos programas de intercâmbio para a graduação médica. Conclusão: A mobilidade internacional pode representar um importante instrumento para a formação médica, pois estimula o amadurecimento profissional e pessoal dos estudantes, ao expô‑los a um cenário diferenciado, no qual são incitados a refletir sobre suas práticas e seu conhecimento, ao se deparar com o novo e o diferente. Tais contribuições podem representar o aprimoramento das habilidades médicas e humanísticas dos graduandos, configurando-se como um avanço para o ensino médico no Brasi.

PRESENCE OF THYMIC TISSUE IN THE ANTERIOR MEDIASTINAL FATTY TISSUE AND ITS SIGNIFICANCE IN THYMECTOMY FOR MYASTHENIA GRAVIS PATIENT: A CASE REPORT

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Athouba

2016-03-01

Full Text Available In 1970s, presence of thymic tissue in anterior mediastinal adipose tissue around the thymus was found. Here we report a case of ectopic thymic tissue in the mediastinum and the possible relevance of this distribution of thymic tissue outside thymus to the therapeutic yield of thymectomies in myasthenia gravis. A 30-year lady with myasthenia gravis (nonthymomatous presented with difficulty in swallowing and breathing for the last 1 years. She was under medical treatment but with little improvement. She underwent extended thymectomy, after which an en bloc resection of the anterior mediastinal fat tissues from pericardium and pleura, including the thymus, was performed. Grossly the soft tissue specimen taken from near left lateral area of heart was fibrofatty tissue. Microscopically isolated thymic tissues were seen interspersed among the fatty tissues composed of mature lymphocytes, epithelial cells and few Hassall's corpuscles were observed. Thymus specimen was within normal histological limits. To ensure complete removal, the adipose tissue at the anterior mediastinum as well as the gross thymus should be removed. Thymic tissue incidence in individual locations was as follows: Retrothyroid, 3(6%; peritracheal, 5 (10%; retrotracheal, 1 (2%; right phrenic nerve, 2 (4%; left phrenic nerve, 14 (28%; right recurrent laryngeal nerve, 2 (4%; left recurrent laryngeal nerve, 2 (4% and periaortic, 0. Trans-sternal thymectomy was found to be beneficial to all patients of mild-to-moderate myasthenia gravis with 70.2% patients showing improvement postoperatively.

Anestesia venosa total (AVT) em lactente com doença de Werdnig-Hoffmann: relato de caso Anestesia general intravenosa (AVT) en lactante con enfermedad de Werdnig-Hoffmann: relato de caso Total intravenous anesthesia (TIVA) in an infant with Werdnig-Hoffmann disease: case report

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Marco Antonio Cardoso de Resende; Elizabeth Vaz da Silva; Osvaldo José Moreira Nascimento; Alberto Esteves Gemal; Giseli Quintanilha; Eliana Maria Vasconcelos

2010-01-01

JUSTIFICATIVA E OBJETIVOS: A doença de Werdnig-Hoffmann é a causa mais comum de hipotonia no lactente e quando presente logo após o nascimento tem pior prognóstico. Fraqueza muscular simétrica, arreflexia e fasciculações da língua são característicos. A maioria dos lactentes morre antes dos dois anos por insuficiência respiratória. O presente relato apresenta um caso com técnica venosa total durante anestesia. RELATO DO CASO: Paciente feminina, branca, um ano, 10 kg, estado físico ASA III, co...

Validation of the italian version of the 15-item Myasthenia Gravis Quality-of-Life questionnaire.

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Raggi, Alberto; Leonardi, Matilde; Ayadi, Roberta; Antozzi, Carlo; Maggi, Lorenzo; Baggi, Fulvio; Mantegazza, Renato

2017-10-01

In this study we assess the Italian version of the 15-item Myasthenia Gravis Quality-of-Life questionnaire (MG-QOL15). The validation protocol included the MG-QOL15, the 36-item Short Form (SF-36), the Besta Neurological Institute Rating Scale for Myasthenia Gravis, and the MG-Composite. We used the Cronbach α to test reliability, the Spearman correlation to test short-term test-retest, the Kruskal-Wallis test to assess differences in MG-QOL15 between patients with different disease severity, and the Wilcoxon signed-rank test to assess sensitivity to change. Seventy-two patients were enrolled in the study. The mean MG-QOL15 score was 15.2 ± 12.2, with α = 0.93 and test-retest correlation = 0.93. Compared with the SF-36, the MG-QOL15 was superior in differentiating patients with different MG types (P = 0.041) and severity (P = 0.004), showed higher sensitivity to change (P = 0.003 for improved and P = 0.024 for worsened patients), and had higher correlations with the MG-Composite (rho = 0.367 vs. -0.213 and -0.154). The Italian version of the MG-QOL15 is valid, reliable, stable, and sensitive to changes. Muscle Nerve 56: 716-720, 2017. © 2016 Wiley Periodicals, Inc.

Bloqueio bilateral do nervo pudendo para hemorroidectomia em paciente acondroplásico: relato de caso Bloqueo bilateral del nervio pudendo para hemorroidectomía en paciente acondroplásico: relato de caso Bilateral blockade of the pudend nerve to hemorrhoidectomy in achondroplasic patient: case report

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Bruno Salomé de Morais; Marcos Guilherme Cunha Cruvinel; Yerkes Pereira Silva; Dener Augusto Diniz; Carlos Henrique Viana de Castro

2006-01-01

JUSTIFICATIVA E OBJETIVOS: O nanismo acondroplásico ou acondroplasia é a forma mais comum de nanismo e ocorre na maioria dos casos por alteração genética espontânea. A anestesia desses pacientes apresenta várias particularidades. O objetivo do presente relato foi descrever um caso de paciente acondroplásico, com história prévia de intervenção cirúrgica da coluna para descompressão medular, submetido a hemorroidectomia através de bloqueio bilateral dos nervos pudendos. RELATO DO CASO: Paciente...

Bacteriological survey of frozen meat and gravy produced at establishments under federal inspection.

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Surkiewicz, B F; Harris, M E; Johnston, R W

1973-10-01

During visits to 34 federally inspected establishments producing frozen meat and gravy, 541 production line samples and 535 finished product units were collected for bacteriological analyses. It was found that more than 70% of the sets of finished product (10 units/set) produced under good manufacturing practices had: (i) four or fewer coliform-positive units, (ii) two or fewer Escherichia coli-positive units, (iii) three or fewer Staphylococcus aureus-positive units, and (iv) an aerobic plate count of fewer than 50,000/g (geometric mean of 10 units). All finished product units were negative for salmonellae.

Relatos reconfiguradores de la violencia familiar en Antioquia (Colombia)*

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Builes Correa, María Victoria; López Serna, Lina María

2009-01-01

Introducción: La autoridad impuesta con sangre y lágrimas ha empezado a cuestionarse, debido a que se percibe como inefciente y plantea la necesidad de introducir el diálogo y la negociación en las prácticas de crianza. Objetivo: Reconocer los relatos de buen trato en la experiencia de las familias donde hay violencia. Método: En este estudio comprensivo, de tipo fenomenológico-hermenéutico, se privilegia la salida ética frente a la experiencia de violencia intrafamiliar y se preocupa por dev...

Myasthenia Gravis: Unusual Presentations and Diagnostic Pitfalls.

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Rodolico, Carmelo; Parisi, Daniela; Portaro, Simona; Biasini, Fiammetta; Sinicropi, Stefano; Ciranni, Annamaria; Toscano, Antonio; Messina, Sonia; Musumeci, Olimpia; Vita, Giuseppe; Girlanda, Paolo

2016-08-30

Myasthenia gravis (MG) is an autoimmune disorder presenting with fluctuating, fatigable muscle weakness. Initial symptoms classically involve ocular and proximal limb muscles. Rarely, MG may onset with unusual features, so it can be misdiagnosed with other neuromuscular diseases. To describe unusual and atypical presentations of MG in a large cohort of patients, considering and discussing diagnostic difficulties and pitfalls. We report on 21 out of 508 MG patients, coming to our department in the last 27 years and presenting with atypical or unusual features. The diagnosis was achieved performing a careful clinical examination, a proper neurophysiological assessment, the neostigmine test, the AChR and MuSK antibodies assay and chest CT-scan. Patients with atypical/unusual MG onset were the 4.4% of all MG patients population. We describe seven different clinical categories: asymmetric distal upper limbs weakness, foot drop, isolated triceps brachii weakness and foot drop, post exertional axial weakness with dropped head, acute facial dyplegia, limb-girdle MG and MG with sudden lower limbs weakness and recurrent falls. Atypical and unusual presentations may increase the risk to misdiagnose or delay MG diagnosis. Isolated limb-girdle presentation is the most frequent atypical form in our series.

Algunos relatos sobre santería, magia y etnomedicina en Cuba

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Carrascosa Palomera, Cruz

2008-01-01

Este artículo presenta una colección de relatos sobre santería, magia y etnomedicina en Cuba. Incluye información sobre ritos y prácticas de iniciación, de purificación, de curación. This paper offers a selection of stories about santería, magic and folk medicine in Cuba. Includes reports on iniciation, purification and curation rites and practices

Radiographical diagnosis of the thymus in myasthenia gravis

International Nuclear Information System (INIS)

Tanimura, Shigeo; Banba, Jiro; Masaki, Mikio; Irimoto, Masahiro; Matsushita, Akira

1987-01-01

Comparison of radiographical findings of the thymus between pneumomediastinography and computed tomography were studied in 35 patients with myasthenia gravis. The patients consisted of 10 patients with thymoma and 25 without thymoma confirmed at the operations. Pneumomediastinography was very useful to discern whether the thymoma was invasive or noninvasive, but not contributory to know whether the thymus was composed of folicular lymphoid hyperplasia or normal thyimic tissues. Computed tomography was also useful to dertermine the localization and the invasiveness of the thymoma, but not helpful to know whether the thymus was of follicular lymphoid hyperplasia or normal tissues. However, the finding of ''reticular pattern''-many small nodules scattered resicularly in the thymus-in computed tomography could be regarded as a sign suggesting follicular lymphoid hyperplasia of the thymus. Therfore, both pneumomediastinography and computed tomography were very useful in the diagnosis of the localization and the invasiveness of the thymoma but not for the diagnosis of follicular lymphoid hyperplasia of the thymus. Neverethless the finding of ''reticular pattern'' computred tomography was helpful in the diagnosis of follicular lymphoid hyperplasia. (author)

Computed tomography of the thymus in myasthenia gravis

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Tanimura, Shigeo; Sakaguchi, Kozo; Tomoyasu, Hiroshi; Banba, Jiro; Masaki, Mikio; Kurosaki, Atsuko; Matsushita, Hisashi [Toranomon Hospital, Tokyo (Japan)

1995-01-01

Comparison of preoperative CT diagnosis and histopathological diagnosis for the thymus were studied in 39 patients with myasthenia gravis. The patients consisted of 10 patients with thymoma and 29 without thymoma confirmed at the operation. CT diagnosis was 11 thymomas, 18 thymic hyperplasias and 10 normal thymuses. Eleven thymomas revealed histopathologically 9 thymomas, one follicular lymphoid hyperplasia (FLH) and one involved thymus. Out of 18 thymic hyperplasias 15 cases were FLH and 3 involved thymus. There were 5 involved thymuses, 4 FLHs and one thymoma in the 10 normal thymuses on CT. The finding of `reticular pattern`, many small nodules scattered reticularly in the thymus, in computed tomography could be regarded as a sign suggesting FLH of the thymus. The accuracy of this finding of CT was 83% for FLH. The finding of thymoma on CT revealed 82% of the accuracy. Therefore, CT was very useful in the diagnosis of the localization of the thymoma but not for the diagnosis of FLH of the thymus. Nevertheless the finding of `reticular pattern` on CT was helpful in the diagnosis of FLH. (author).

Computed tomography of the thymus in myasthenia gravis

International Nuclear Information System (INIS)

Tanimura, Shigeo; Sakaguchi, Kozo; Tomoyasu, Hiroshi; Banba, Jiro; Masaki, Mikio; Kurosaki, Atsuko; Matsushita, Hisashi

1995-01-01

Comparison of preoperative CT diagnosis and histopathological diagnosis for the thymus were studied in 39 patients with myasthenia gravis. The patients consisted of 10 patients with thymoma and 29 without thymoma confirmed at the operation. CT diagnosis was 11 thymomas, 18 thymic hyperplasias and 10 normal thymuses. Eleven thymomas revealed histopathologically 9 thymomas, one follicular lymphoid hyperplasia (FLH) and one involved thymus. Out of 18 thymic hyperplasias 15 cases were FLH and 3 involved thymus. There were 5 involved thymuses, 4 FLHs and one thymoma in the 10 normal thymuses on CT. The finding of 'reticular pattern', many small nodules scattered reticularly in the thymus, in computed tomography could be regarded as a sign suggesting FLH of the thymus. The accuracy of this finding of CT was 83% for FLH. The finding of thymoma on CT revealed 82% of the accuracy. Therefore, CT was very useful in the diagnosis of the localization of the thymoma but not for the diagnosis of FLH of the thymus. Nevertheless the finding of 'reticular pattern' on CT was helpful in the diagnosis of FLH. (author)

Dor e Gozo: relatos de mulheres jovens sobre automutilações

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José Juliano Cedaro

2013-08-01

Full Text Available Este artigo discute a prática da automutilação, destacando os relatos de mulheres jovens (entre 15 e 21 anos, atendidas em um Centro de Atenção Psicossocial (CAPS. Tais atitudes consistem em provocar, de forma consciente, feridas no próprio corpo, como cortes, arranhões, queimaduras ou perfurações, não havendo nessas ações o desejo manifesto de suicídio, embora seja comum aparecerem ideações nesse sentido nas falas das pacientes. Os relatos acerca de tal comportamento são apresentados por meio de fragmentos de falas, registrados após atendimentos psicológicos, que esboçam a descrição dos sentimentos expostos dentro do setting terapêutico. As questões suscitadas são discutidas a partir das concepções psicanalíticas a respeito do gozo e do masoquismo, com ênfase nas proposições de Freud e Lacan, focalizando a seguinte indagação: qual é a função da dor autoprovocada - e das marcas corporais autoinflingidas - na dinâmica psíquica das pacientes com histórico de automutilações?

DIAGNOSIS OF MYASTHENIA GRAVIS USING FUZZY GAZE TRACKING SOFTWARE

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Javad Rasti

2015-04-01

Full Text Available Myasthenia Gravis (MG is an autoimmune disorder, which may lead to paralysis and even death if not treated on time. One of its primary symptoms is severe muscular weakness, initially arising in the eye muscles. Testing the mobility of the eyeball can help in early detection of MG. In this study, software was designed to analyze the ability of the eye muscles to focus in various directions, thus estimating the MG risk. Progressive weakness in gazing at the directions prompted by the software can reveal abnormal fatigue of the eye muscles, which is an alert sign for MG. To assess the user’s ability to keep gazing at a specified direction, a fuzzy algorithm was applied to images of the user’s eyes to determine the position of the iris in relation to the sclera. The results of the tests performed on 18 healthy volunteers and 18 volunteers in early stages of MG confirmed the validity of the suggested software.

Myasthenia gravis in pregnancy: Experience of a portuguese center.

Science.gov (United States)

Braga, António Costa; Pinto, Clara; Santos, Ernestina; Braga, Jorge

2016-10-01

We evaluated the clinical course during pregnancy and neonatal outcomes of a cohort of Portuguese women with myasthenia gravis (MG). Retrospective study. Twenty-five patients with 30 pregnancies were included. Mean maternal age was 32.4 ± 4.1 years. Miscarriage rate was 6.7%, with delivery of 28 newborns. Deterioration in MG during pregnancy occurred in 43.3%, and 46.4% occurred at postpartum. Eighty percent were medicated with pyridostigmine, 43.3% with corticosteroids, and 40% with intravenous immunoglobulin. There were no maternal or neonatal deaths. Mean gestational time at delivery was 38.2 weeks. No cases of fetal growth restriction, preeclampsia, preterm delivery, or fetal demise were observed. Global cesarean rate was 64.3%. Two newborns developed transient neonatal myasthenia. A high rate of clinical worsening of MG in the mother was observed in this retrospective study, which highlights the importance of a multidisciplinary approach for avoiding maternal adverse outcomes. Muscle Nerve 54: 715-720, 2016. © 2016 Wiley Periodicals, Inc.

Outcome Measures in Myasthenia Gravis: Incorporation Into Clinical Practice.

Science.gov (United States)

Muppidi, Srikanth

2017-03-01

The development of validated assessment tools for evaluating disease status and response to interventions in patients with myasthenia gravis (MG) has been driven by clinical studies of emerging MG therapies. However, only a small proportion of MG-focused neurology practices have adopted these assessment tools for routine clinical use. This article reviews the suitability of 5 assessment instruments for incorporation into clinical practice, which should be driven by their ability to contribute to improved patient outcomes, and to be implemented within practice personnel and resource constraints. It is recommended that assessments based on both physician-evaluated and patient-reported outcomes be selected, to adequately evaluate both point-in-time symptom load and functional impact of MG symptoms over time. Provider resource allocation and reimbursement issues may be the most significant roadblocks to successful ongoing use of these tools; to that end, the addition of regular assessments to MG standards of care is recommended.

The Myotonic Plot Thickens: Electrical Myotonia in Antimuscle-Specific Kinase Myasthenia Gravis

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Marcus Magnussen

2015-01-01

Full Text Available Electrical myotonia is known to occur in a number of inherited and acquired disorders including myotonic dystrophies, channelopathies, and metabolic, toxic, and inflammatory myopathies. Yet, electrical myotonia in myasthenia gravis associated with antibodies against muscle-specific tyrosine kinase (MuSK has not been previously reported. We describe two such patients, both of whom had a typical presentation of proximal muscle weakness with respiratory failure in the context of a significant electrodecrement in repetitive nerve stimulation. In both cases, concentric needle examination revealed electrical myotonia combined with myopathic motor unit morphology and early recruitment. These findings suggest that MuSK myasthenia should be included within the differential diagnosis of disorders with electrical myotonia.

RELATO EVANGÉLICO E SENTIDO DA FAMÍLIA

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Ignacio Antonio Madera Vargas, SDS

2015-01-01

Full Text Available Alguns dos relatos sobre a relação de Jesus com a sua família nos possibilitam considerar o sentido que podemos encontrar nos mesmos para uma compreensão teológica que aponte para o sentido da experiência das relações no interior da mesma. Tanto o casal como a família constituem uma realidade que, interpretada à luz da experiência de fé que os evangelistas nos oferecem como a Boa Nova do Reino, orientam-se em direção a uma relação a se viver à imagem da comunhão trinitária. A liberdade e a capacidade de crescer em adultícia como sabedoria e graça caracterizam os relatos os quais nos apresentam Jesus e sua família ou familiares. Uma visão de Jesus de Nazaré que o apresenta não afetado pelos traumas e as limitações, que provoca a relação no interior das famílias, em qualquer um de nós, eco da visão que os relatos nos oferecem dEle à luz da ressurreição. Este artigo é resultado das investigações do Projeto Significado da linguagem teológica do Grupo de Investigação Teologia e Mundo Contemporâneo da Faculdade de Teologia da Pontifícia Universidade Javeriana.    ABSTRACT: Some of the tales about the relationship of Jesus to his family allow us enter to consider the sense that we can Knd in them for a theological under standing that in dicate the meaning of the experience of relations to the interior of the family. Both the couple and the family constitute a reality which, interpreted in the light of the experience of faith that the evangelists oVer us as the good news of the Kingdom, directed towards a relationship to living in the image of the Trinitarian communion. Freedom and the ability to grow in adulthood as wisdom and grace characterize the stories in which presents itself to Jesus and his family or relatives. A view of Jesus from Nazareth which presents it not aVected by trauma and limitations that causes the relationship to the interior of the families at any one of us, echo of the vision that the

An association of myasthenia gravis with Hashimoto’s thyroiditis in a patient with a multinodular goitre

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Nik Siti Fatimah Mohamed

2017-08-01

Full Text Available Introduction: The association of myasthenia gravis (MG with other autoimmune diseases including autoimmune thyroid disease (ATD is well recognised, although rare. The occurrence of both diseases can occur in two ways: either disease preceding the other, or concurrently. The presentation of MG in association with ATD can range from ocular to generalised disease. Case Summary: A 26-year-old Malay female with persistent hyperthyroidism secondary to Hashimoto’s thyroiditis in multinodular goitre was diagnosed with generalised MG after 2 years. She presented with right eye ptosis (ocular and difficulty in swallowing and chewing (bulbar. The diagnosis of MG was confirmed by fatigability testing, electromyography and the presence of AChR antibodies. Her symptoms showed improvement with pyridostigmine (Mestinon 60 mg 6-hourly. Her antithyroid drug was tapered down according to her thyroid function test. Throughout a year of follow-ups, her hyperthyroidism and fatigability symptoms improved with treatment. She was later counselled for total thyroidectomy and thymectomy. Conclusion: Myasthenia gravis and hyperthyroidism may present with similar symptoms such as dysphagia due to neuromuscular weakness or fatigue. When the diseases occur together, one of the diagnoses may be missed. Therefore, the occurrence of new symptoms in a patient with underlying ATD should should trigger the early identification of other autoimmune diseases by primary care doctors.

Hinge-deleted IgG4 blocker therapy for acetylcholine receptor myasthenia gravis in rhesus monkeys.

Science.gov (United States)

Losen, Mario; Labrijn, Aran F; van Kranen-Mastenbroek, Vivianne H; Janmaat, Maarten L; Haanstra, Krista G; Beurskens, Frank J; Vink, Tom; Jonker, Margreet; 't Hart, Bert A; Mané-Damas, Marina; Molenaar, Peter C; Martinez-Martinez, Pilar; van der Esch, Eline; Schuurman, Janine; de Baets, Marc H; Parren, Paul W H I

2017-04-20

Autoantibodies against ion channels are the cause of numerous neurologic autoimmune disorders. Frequently, such pathogenic autoantibodies have a restricted epitope-specificity. In such cases, competing antibody formats devoid of pathogenic effector functions (blocker antibodies) have the potential to treat disease by displacing autoantibodies from their target. Here, we have used a model of the neuromuscular autoimmune disease myasthenia gravis in rhesus monkeys (Macaca mulatta) to test the therapeutic potential of a new blocker antibody: MG was induced by passive transfer of pathogenic acetylcholine receptor-specific monoclonal antibody IgG1-637. The effect of the blocker antibody (IgG4Δhinge-637, the hinge-deleted IgG4 version of IgG1-637) was assessed using decrement measurements and single-fiber electromyography. Three daily doses of 1.7 mg/kg IgG1-637 (cumulative dose 5 mg/kg) induced impairment of neuromuscular transmission, as demonstrated by significantly increased jitter, synaptic transmission failures (blockings) and a decrease in the amplitude of the compound muscle action potentials during repeated stimulations (decrement), without showing overt symptoms of muscle weakness. Treatment with three daily doses of 10 mg/kg IgG4Δhinge-637 significantly reduced the IgG1-637-induced increase in jitter, blockings and decrement. Together, these results represent proof-of principle data for therapy of acetylcholine receptor-myasthenia gravis with a monovalent antibody format that blocks binding of pathogenic autoantibodies.

Novel immunotoxin: a fusion protein consisting of gelonin and an acetylcholine receptor fragment as a potential immunotherapeutic agent for the treatment of Myasthenia gravis.

Science.gov (United States)

Hossann, Martin; Li, Zhuoyu; Shi, Yawei; Kreilinger, Ulrike; Büttner, Jörn; Vogel, Pia D; Yuan, Jingming; Wise, John G; Trommer, Wolfgang E

2006-03-01

In continuation of our attempts for antigen-specific suppression of the immune system [I.L. Urbatsch, R.K.M. Sterz, K. Peper, W.E. Trommer, Eur. J. Immunol. 23(1993) 776-779] a novel fusion protein composed of amino acids 4-181 of the extracellular domain of the alpha-subunit of the human muscle acetylcholine receptor and the plant toxin gelonin was expressed in Escherichia coli. The fusion protein formed inclusion bodies but could be solubilized in the presence of guanidinium hydrochloride. After a simple two step purification and refolding procedure, it exhibited a native structure at least in the main immunogenic region as shown by antibodies recognizing a conformational epitope. Half maximal inhibition of translation was achieved at 46 ng/ml as compared to 4.6 ng/ml for native and 2.4 for recombinant gelonin. Its use as therapeutic agent for the treatment of Myasthenia gravis was investigated in an animal model. Female Lewis rats were immunized with complete acetylcholine receptor from the electric ray Torpedo californica and developed thereafter experimental autoimmune M. gravis. Quantitative assessment of the disease was achieved by repetitive stimulation of the Nervus tibialis. Rats showed no symptoms of M. gravis, neither visually nor electrophysiologically after treatment with the fusion protein as determined one and seven weeks after the second application. This approach may also be useful for the therapy of further autoimmune diseases by substituting other autoantigens for the AchR fragment in the fusion protein.

Immunopathogenesis in Myasthenia Gravis and Neuromyelitis Optica

Science.gov (United States)

Wang, Zhen; Yan, Yaping

2017-01-01

Myasthenia gravis (MG) and neuromyelitis optica (NMO) are autoimmune channelopathies of the peripheral neuromuscular junction (NMJ) and central nervous system (CNS) that are mainly mediated by humoral immunity against the acetylcholine receptor (AChR) and aquaporin-4 (AQP4), respectively. The diseases share some common features, including genetic predispositions, environmental factors, the breakdown of tolerance, the collaboration of T cells and B cells, imbalances in T helper 1 (Th1)/Th2/Th17/regulatory T cells, aberrant cytokine and antibody secretion, and complement system activation. However, some aspects of the immune mechanisms are unique. Both targets (AChR and AQP4) are expressed in the periphery and CNS, but MG mainly affects the NMJ in the periphery outside of CNS, whereas NMO preferentially involves the CNS. Inflammatory cells, including B cells and macrophages, often infiltrate the thymus but not the target—muscle in MG, whereas the infiltration of inflammatory cells, mainly polymorphonuclear leukocytes and macrophages, in NMO, is always observed in the target organ—the spinal cord. A review of the common and discrepant characteristics of these two autoimmune channelopathies may expand our understanding of the pathogenic mechanism of both disorders and assist in the development of proper treatments in the future. PMID:29312313

Uma análise comportamentalista de relatos verbais e práticas educativas parentais: alcance e limites

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Vanessa Barbosa Romera Leme

2009-08-01

Full Text Available O presente artigo discute a relevância de algumas estratégias metodológicas da Análise Aplicada do Comportamento para examinar práticas educativas parentais. Um instrumento e um conceito relacionados à coleta e análise de dados são discutidos sob a ótica teórica e aplicada: relatos verbais e análise funcional comportamental descritiva. Conclui-se que, consideradas as limitações dos relatos, as informações obtidas com essa estratégia e com a análise funcional comportamental descritiva permitem inferir algumas hipóteses sobre os comportamentos de pais e de filhos que podem ser utilizadas em ações que procuram investigar e intervir sobre os comportamentos problema e as habilidades sociais.

FALANDO SOBRE SEXUALIDADE NA ADOLESCÊNCIA: RELATO DE EXPERIÊNCIA

OpenAIRE

Rabin, Eliane Goldberg; UFCSPA; Waterkemper, Roberta; UFCSPA; Caregnato, Rita C. A.; UFCSPA; Souza, Emiliane Nogueira de; UFCSPA

2014-01-01

Trata-se de um relato de experiência sobre atividade de extensão realizada por docentes e acadêmicos do Curso de Graduação em Enfermagem sobre sexualidade na adolescência. Atividade realizada com jovens de 10 a 19 anos, estudantes do ensino fundamental e médio de escolas públicas de Porto Alegre. Foram utilizadas diferentes estratégias e dinâmicas para o desenvolvimento das atividades, com vistas à maior participação dos jovens escolares. Os temas abordados foram as mudanças corporais, por me...

Experimental autoimmune myasthenia gravis may occur in the context of a polarized Th1- or Th2-type immune response in rats

DEFF Research Database (Denmark)

Saoudi, A; Bernard, I; Hoedemaekers, A

1999-01-01

Experimental autoimmune myasthenia gravis (EAMG) is a T cell-dependent, Ab-mediated autoimmune disease induced in rats by a single immunization with acetylcholine receptor (AChR). Although polarized Th1 responses have been shown to be crucial for the development of mouse EAMG, the role of Th cell...

Anesthetic management of patient with myasthenia gravis and uncontrolled hyperthyroidism for thymectomy

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Datt Vishnu

2010-01-01

Full Text Available The relationship between myasthenia gravis (MG and other autoimmune disorders like hyperthyroidism is well known. It may manifest earlier, concurrently orafter the appearance of MG. The effect of treatment of hyperthyroidism on the control of MG is variable. There may be resolution or conversely, deterioration of the symptoms also. We present a patient who was diagnosed to be hyperthyroid two and half years before the appearance of myasthenic symptoms. Pharmacotherapy for three months neither improved the myasthenic symptoms nor the thyroid function tests. Thymectomy resulted in control of MG as well as hyperthyroidism. In conclusion, effective control of hyperthyroidism in the presence of MG may be difficult. The authors opine that careful peri-operative management of thymectomy is possible in a hyperthyroid state.

Anesthetic management of patient with myasthenia gravis and uncontrolled hyperthyroidism for thymectomy.

Science.gov (United States)

Datt, Vishnu; Tempe, Deepak K; Singh, Baljit; Tomar, Akhlesh S; Banerjee, Amit; Dutta, Devesh; Bhandari, Hricha

2010-01-01

The relationship between myasthenia gravis (MG) and other autoimmune disorders like hyperthyroidism is well known. It may manifest earlier, concurrently or after the appearance of MG. The effect of treatment of hyperthyroidism on the control of MG is variable. There may be resolution or conversely, deterioration of the symptoms also. We present a patient who was diagnosed to be hyperthyroid two and half years before the appearance of myasthenic symptoms. Pharmacotherapy for three months neither improved the myasthenic symptoms nor the thyroid function tests. Thymectomy resulted in control of MG as well as hyperthyroidism. In conclusion, effective control of hyperthyroidism in the presence of MG may be difficult. The authors opine that careful peri-operative management of thymectomy is possible in a hyperthyroid state.

Myasthenia Gravis After Nivolumab Therapy for Squamous Cell Carcinoma of the Bladder.

Science.gov (United States)

Chang, Elaine; Sabichi, Anita L; Sada, Yvonne H

2017-04-01

Checkpoint inhibitors have become standard therapy for multiple cancers, and their use will increase in the next year as regulatory approvals for additional indications are expected. It is essential for clinicians to be aware of the potential for rare immune-related adverse effects. Here, we report the case of a new diagnosis of myasthenia gravis (MG) after the use of nivolumab for squamous cell carcinoma of the bladder. A review the literature identified 10 cases of MG diagnosed after programmed cell death protein 1 inhibitor therapy. This is the first case, to our knowledge, reported in association with bladder cancer. The precise diagnosis of MG has important implications on management, as treatment with steroids can transiently worsen myasthenia in nearly 50% of cases.

Familias homoparentales: relatos desde la perspectiva de los “conocimientos situados”.

OpenAIRE

Buzaglo, Analía; Culla, Mónica; Morandi, Mariela

2015-01-01

En el presente trabajo nos proponemos indagar los aportes de la epistemología feminista en las investigaciones críticas en Ciencias Sociales. Partimos de una experiencia de investigación singular y situada: “Familias homoparentales: narrativas contrahegemónicas del parentesco” basada en la metodología de las producciones narrativas que retoma la propuesta de “conocimientos situados” de Donna Haraway (1995). Estos relatos, cuestionan teorías y prácticas hegemónicas con efectos concretos...

Myasthenia gravis in children: analysis of 18 patients

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Morita Maria da Penha A.

2001-01-01

Full Text Available Myasthenia gravis (MG in childhood is rare comprising 10 to 20 % of all myasthenic patients. We studied 18 patients with MG whose first symptoms started from 1 to 12 years of age, followed at the Department of Neurology of the UNIFESP-EPM, from January 1983 to August 1997. There were 10 girls and 8 boys (1.2:1. Eleven patients (61% presented moderate or severe generalized disease and 4 (22% had at least one myasthenic crisis. EMG with supramaximal repetitive nerve stimulation was diagnostic in 8 (47% out of 17 patients, and chest CT was normal in 14 patients. Seropositivity to acetylcholine receptor antibodies was found in 81.6% (9 out of 11 tested and the levels had no relation to clinical severity. Nine out of 16 patients (56% worsened with pyridostigmine alone and were treated with prednisone. Four out of those nine continued worsening despite steroids and were subjected to thymectomy (all showed thymic lymphoid follicular hyperplasia. Three patients (75% improved markedly after thymectomy and one (25% worsened, eventually getting better with intravenous immunoglobulin and oral azathioprine. MG treatment, using all resources available, has to be individualized for each child.

Embolia pulmonar na sala de cirurgia: relato de caso Embolia pulmonar en sala operatoria: relato de caso Pulmonary embolism in the operating room: case report

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Karina Bernardi Pimenta

2002-04-01

Full Text Available JUSTIFICATIVA E OBJETIVOS: Embolia pulmonar é uma complicação freqüente no período pós-operatório. O objetivo deste relato é apresentar um caso de embolia pulmonar ocorrida na sala de operação e chamar a atenção para a importância da profilaxia de trombose venosa em pacientes cirúrgicos. RELATO DO CASO: Trata-se de um paciente do sexo masculino, 55 anos e 83 kg com diagnóstico de câncer de próstata, submetido a prostatectomia supra-púbica sob anestesia geral. Ao final da cirurgia, o paciente já extubado e logo após sua passagem para a maca de transporte apresentou instabilidade hemodinâmica e diminuição da SpO2 para 80%. Foi reintubado e encaminhado para a UTI. A tomografia computadorizada mostrou imagens com aspecto de embolia pulmonar. O paciente evoluiu para óbito no 5º dia de pós-operatório. CONCLUSÕES: O elevado índice de suspeita não é suficiente para firmar o diagnóstico pois a embolia pulmonar é uma doença silenciosa e a rotina de investigação não possui elevada sensibilidade. A profilaxia precoce e adequada é a melhor estratégia.JUSTIFICATIVA Y OBJETIVOS: Embolia pulmonar es una complicación frecuente en el período pós-operatorio. El objetivo de este relato es presentar un caso de embolia pulmonar ocurrida en la sala de operación y llamar la atención para la importancia de la profilaxis de trombosis venosa en pacientes cirúrgicos. RELATO DE CASO: Se trata de un paciente del sexo masculino, 55 años y 83 kg con diagnóstico de cáncer de próstata, sometido a prostatectomia supra-púbica bajo anestesia general. Al final de la cirugía, el paciente ya entubado y luego después de pasar para la camilla de transporte presentó inestabilidad hemodinámica y diminución de la SpO2 para 80%. Fue reintubado y encaminado para la UTI. La tomografía computadorizada mostró imágenes con aspecto de embolia pulmonar. El paciente evolucionó para óbito en el 5º día de pós-operatorio. CONCLUSIONES: El

Guidelines for standard preclinical experiments in the mouse model of myasthenia gravis induced by acetylcholine receptor immunization.

Science.gov (United States)

Tuzun, Erdem; Berrih-Aknin, Sonia; Brenner, Talma; Kusner, Linda L; Le Panse, Rozen; Yang, Huan; Tzartos, Socrates; Christadoss, Premkumar

2015-08-01

Myasthenia gravis (MG) is an autoimmune disorder characterized by generalized muscle weakness due to neuromuscular junction (NMJ) dysfunction brought by acetylcholine receptor (AChR) antibodies in most cases. Although steroids and other immunosuppressants are effectively used for treatment of MG, these medications often cause severe side effects and a complete remission cannot be obtained in many cases. For pre-clinical evaluation of more effective and less toxic treatment methods for MG, the experimental autoimmune myasthenia gravis (EAMG) induced by Torpedo AChR immunization has become one of the standard animal models. Although numerous compounds have been recently proposed for MG mostly by using the active immunization EAMG model, only a few have been proven to be effective in MG patients. The variability in the experimental design, immunization methods and outcome measurements of pre-clinical EAMG studies make it difficult to interpret the published reports and assess the potential for application to MG patients. In an effort to standardize the active immunization EAMG model, we propose standard procedures for animal care conditions, sampling and randomization of mice, experimental design and outcome measures. Utilization of these standard procedures might improve the power of pre-clinical EAMG experiments and increase the chances for identifying promising novel treatment methods that can be effectively translated into clinical trials for MG. Copyright © 2015 Elsevier Inc. All rights reserved.

Myasthaenia Gravis : Clinical management issues before, during and after pregnancy

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Ali Hassan

2017-08-01

Full Text Available Myasthaenia gravis (MG is an autoimmune neuromuscular disorder which is twice as common among women, often presenting in the second and third decades of life. Typically, the first trimester of pregnancy and first month postpartum are considered high-risk periods for MG exacerbations. During pregnancy, treatment for MG is usually individualised, thus improving its management. Plasma exchange and immunoglobulin therapies can be safely used to treat severe manifestations of the disease or myasthaenic crises. However, thymectomies are not recommended because of the delayed beneficial effects and possible risks associated with the surgery. Assisted vaginal delivery—either vacuum-assisted or with forceps—may be required during labour, although a Caesarean section under epidural anaesthesia should be reserved only for standard obstetric indications. Myasthaenic women should not be discouraged from attempting to conceive, provided that they seek comprehensive counselling and ensure that the disease is under good control before the start of the pregnancy.

Beneficial effect of tocilizumab in myasthenia gravis refractory to rituximab.

Science.gov (United States)

Jonsson, Dagur Ingi; Pirskanen, Ritva; Piehl, Fredrik

2017-06-01

Muscle fatigue associated with myasthenia gravis is caused by autoantibodies interfering with neuromuscular transmission. Immunomodulating treatment is widely used in moderate to severe myasthenia, although the use of newer biological drugs except rituximab is rare. We describe the effect of tocilizumab, a blocker of interleukin-6 signalling, in two female myasthenia patients with high titres of serum acetylcholine receptor antibodies and insufficient response to rituximab. The first patient had been treated with high dose immunoglobulins regularly for several years and the second patient had been treated both with different oral immune suppressants and immunoglobulins before testing a low dose of rituximab without significant clinical effect. Subsequent treatment with tocilizumab resulted in clinical improvement within a few months. The first patient was switched back to rituximab, which resulted in worsening until tocilizumab was restarted. Tocilizumab can be a therapeutic option in cases not responding to rituximab. Copyright © 2017 Elsevier B.V. All rights reserved.

Myasthenia gravis in Ceará, Brazil: clinical and epidemiological aspects

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Aline de Almeida Xavier Aguiar

2010-12-01

Full Text Available A retrospective chart review was performed on patients diagnosed as having myasthenia gravis in Ceará State, Brazil and who were followed from October 1981 to June 2009. 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 first main symptoms and signs were ptosis, diplopia and limb 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. The data presented are similar to those of studies performed in other countries.

Anaesthetic Management of a patient with Myasthenia Gravis and Small Bowel Intussusception for Jejuno-Ileal Anastomoses

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Pawan Kumar Jain

2016-01-01

Full Text Available Myasthenia gravis is a chronic autoimmune disease affecting voluntary skeletal muscles. The altered sensitivity of acetylcholine receptors to muscle relaxants and concomitant treatment with anticholinesterase in these patients affect their anaesthetic management. Patients who have undergone bowel anastomosis and are on regular anticholinesterase treatment are susceptible to anastomotic leaks. We report successful anaesthetic management of class I myasthenic patient with coexisting small bowel intussusception operated for jejuno-ileal anastomoses using regional, inhalational and intravenous (i.v anaesthesia based on train of four responses, and avoiding the use of reversal (anticholinesterase.

Familias homoparentales: relatos desde la perspectiva de los “conocimientos situados”

OpenAIRE

Buzaglo, Analía; Culla, Mónica; Morandi, Mariela

2015-01-01

En el presente trabajo nos proponemos indagar los aportes de la epistemología feminista en las investigaciones críticas en Ciencias Sociales. Partimos de una experiencia de investigación singular y situada: “Familias homoparentales: narrativas contrahegemónicas del parentesco” basada en la metodología de las producciones narrativas que retoma la propuesta de “conocimientos situados” de Donna Haraway (1995). Estos relatos, cuestionan teorías y prácticas hegemónicas con efectos concretos en la ...

Specific removal of autoantibodies by extracorporeal immunoadsorption ameliorates experimental autoimmune myasthenia gravis.

Science.gov (United States)

Lazaridis, Konstantinos; Dalianoudis, Ioannis; Baltatzidi, Vasiliki; Tzartos, Socrates J

2017-11-15

Myasthenia gravis (MG) is caused by autoantibodies, the majority of which target the muscle acetylcholine receptor (AChR). Plasmapheresis and IgG-immunoadsorption are useful therapy options, but are highly non-specific. Antigen-specific immunoadsorption would remove only the pathogenic autoantibodies, reducing the possibility of side effects while maximizing the benefit. We have extensively characterized such adsorbents, but in vivo studies are missing. We used rats with experimental autoimmune MG to perform antigen-specific immunoadsorptions over three weeks, regularly monitoring symptoms and autoantibody titers. Immunoadsorption was effective, resulting in a marked autoantibody titer decrease while the immunoadsorbed, but not the mock-treated, animals showed a dramatic symptom improvement. Overall, the procedure was found to be efficient, suggesting the subsequent initiation of clinical trials. Copyright © 2017 Elsevier B.V. All rights reserved.

[Onset of myasthenia gravis in primary care. Presentation of a case].

Science.gov (United States)

Álvarez-Cordovés, M M; Mirpuri-Mirpuri, P G; Pérez-Monje, A

2013-10-01

Myasthenia gravis is an autoimmune disorder of neuromuscular transmission involving the production of autoantibodies directed against skeletal muscle receptors, in most cases of acetylcholine. Clinically it is characterized by the appearance of muscle weakness after prolonged activity, which tends to recover after a period of rest, or administration of acetylcholinesterase inhibitors. It is a relatively rare disease, although the prevalence has increased by improved diagnosis and increased longevity of the population. The diagnosis can be based on evidence after it is suspected using pharmacological, immunological or electrophysiology tests. Treatment can be divided into: symptomatic, short term and long term. We report the case of a patient who complained of diplopia, this muscle weakness being the most common initial symptom of the disease. Copyright © 2012 Sociedad Española de Médicos de Atención Primaria (SEMERGEN). Publicado por Elsevier España. All rights reserved.

Clinical Characteristics of MuSK Antibody-positive Myasthenia Gravis in Taiwan

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Yung-Chuan Huang

2008-07-01

Full Text Available Circulating antibodies of the acetylcholine receptor (AchRAb are detectable in most patients with generalized myasthenia gravis (MG. A newly discovered antibody against muscle-specific kinase (MuSKAb has been detected in 40–70% of AchRAb-negative MG patients. We report a series of Taiwanese MuSKAb-positive patients, and compare their clinical features with MuSKAb-negative patients and also with MuSKAb-positive Caucasians. Five out of 44 seronegative generalized MG patients (11.4% were positive for MuSKAb. Patients with MuSKAb tended to have severe disability and bulbar involvement, and more often experienced crisis or impending crisis. Although all of these patients showed an initial response to immunosuppressant therapy, they had greater disability at follow-up. The clinical features of Taiwanese MuSKAb-positive patients were not different from those of Caucasians, except for a lower prevalence.

Aprendizagem significativa: relato de experiência no ensino superior

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Caren E. Studer

1999-12-01

Full Text Available O presente artigo tem como finalidade, o relato e a análise da prática de elaboração de relatórios semanais, a partir de textos pertinentes ao conteúdo de uma disciplina em cursos de nível superior em uma faculdade particular no interior do Estado de S. Paulo.Tendo como referencial teórico a Teoria Crítica da Educação, procura analisar a abrangência dessa experiência situada em um ambiente preponderantemente tradicional de ensino, utilizando-se como ferramentas de análise as categorias de aprendizagem significativa de Masetto (1998 e alienação desenvolvida por Castanho (1989.

Safety and efficacy of eculizumab in anti-acetylcholine receptor antibody-positive refractory generalised myasthenia gravis (REGAIN): a phase 3, randomised, double-blind, placebo-controlled, multicentre study

NARCIS (Netherlands)

Howard, James F.; Utsugisawa, Kimiaki; Benatar, Michael; Murai, Hiroyuki; Barohn, Richard J.; Illa, Isabel; Jacob, Saiju; Vissing, John; Burns, Ted M.; Kissel, John T.; Muppidi, Srikanth; Nowak, Richard J.; O'Brien, Fanny; Wang, Jing-Jing; Mantegazza, Renato; Mazia, Claudio Gabriel; Wilken, Miguel; Ortea, Carolina; Saba, Juliet; Rugiero, Marcelo; Bettini, Mariela; Vidal, Gonzalo; Garcia, Alejandra Dalila; Lamont, Phillipa; Leong, Wai-Kuen; Boterhoven, Heidi; Fyfe, Beverly; Roberts, Leslie; Jasinarachchi, Mahi; Willlems, Natasha; Wanschitz, Julia; Löscher, Wolfgang; de Bleecker, Jan; van den Abeele, Guy; de Koning, Kathy; de Mey, Katrien; Mercelis, Rudy; Wagemaekers, Linda; Mahieu, Delphine; van Damme, Philip; Smetcoren, Charlotte; Stevens, Olivier; Verjans, Sarah; D'Hondt, Ann; Tilkin, Petra; Alves de Siqueira Carvalho, Alzira; Hasan, Rosa; Dias Brockhausen, Igor; Feder, David; van der Kooi, Anneke

2017-01-01

Complement is likely to have a role in refractory generalised myasthenia gravis, but no approved therapies specifically target this system. Results from a phase 2 study suggested that eculizumab, a terminal complement inhibitor, produced clinically meaningful improvements in patients with

Effects of nisin and temperature on survival, growth, and enterotoxin production characteristics of psychrotrophic Bacillus cereus in beef gravy.

OpenAIRE

Beuchat, L R; Clavero, M R; Jaquette, C B

1997-01-01

The presence of psychrotrophic enterotoxigenic Bacillus cereus in ready-to-serve meats and meat products that have not been subjected to sterilization treatment is a public health concern. A study was undertaken to determine the survival, growth, and diarrheal enterotoxin production characteristics of four strains of psychrotrophic B. cereus in brain heart infusion (BHI) broth and beef gravy as affected by temperature and supplementation with nisin. A portion of unheated vegetative cells from...

Hannah Arendt: importancia del relato histórico y ficticio como vía para la reflexión ética y política

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Fonnegra Osorio, Claudia Patricia

2017-07-01

Full Text Available En este artículo se presenta la importancia que le da Hannah Arendt al relato histórico y ficticio como vía para la reflexión ética y política. Se estudia, en primer lugar, cómo los relatos posibilitan la configuración de la identidad, tanto de un hombre como de una comunidad, ya que la narración permite dar sentido a la fragilidad y contingencia propia de las acciones humanas. En segundo lugar se analiza de qué manera la narración permite conservar en la memoria acciones para que no sean olvidadas. Finalmente se estudia cómo el seguimiento de relatos contribuye a la comprensión del mundo humano y a potenciar la facultad de juzgar para superar pensamientos solipsistas, elemento necesario para el ejercicio de una concepción ética y política basada en el reconocimiento de la pluralidad humana.

Paracoccidioidomicose e síndrome de imunodeficiência adquirida: relato de necropsia

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Godoy Pérsio

2006-01-01

Full Text Available Descrição de um caso de paracoccidioidomicose generalizada em homem de 59 anos com síndrome de imunodeficiência adquirida, sem diagnóstico clínico prévio, observado à necropsia. Houve disseminação do fungo para vários órgãos, caracterizando-se, especialmente nos linfonodos, reativação aguda de processo antigo. O relato acrescenta-se ao número relativamente reduzido dos anotados na literatura médica.

Perioperative and long-term outcome of thymectomy for myasthenia gravis: comparison of surgical approaches and prognostic analysis.

Science.gov (United States)

Liu, Cheng-wu; Luo, Meng; Mei, Jian-dong; Zhu, Yun-ke; Pu, Qiang; Ma, Lin; Che, Guo-wei; Lin, Yi-dan; Wu, Zhu; Wang, Yun; Kou, Ying-li; Liu, Lun-xu

2013-01-01

Thymectomy is an established treatment for myasthenia gravis (MG), and video-assisted thoracoscopic surgery (VATS) thymectomy has become an acceptable surgical procedure. This study aimed to compare the results of VATS thymectomy and open thymectomy and to identify the prognostic factors after thymectomy. The clinical data of 187 consecutive thymectomies performed between July 2000 and December 2009 were retrospectively reviewed; 75 open thymectomies and 112 VATS thymectomies. Clinical efficacy and variables influencing outcome were assessed by Kaplan-Meier survival curves and Cox proportional hazards regression analysis. The operative blood loss in the VATS group was significantly less than that in the open group ((62.14 ± 55.43) ml vs. (137.87 ± 165.25) ml, P CSR) was the end point for evaluation of the treatment results. The overall five-year CSR rate was 57.5%. Two good prognostic factors were identified; preoperative prescription of anticholinesterase alone (P = 0.035) and non-thymomatous MG (P = 0.003). The five-year CSR rate of the ocular type of MG reached a high level of 67.4%. Thymectomy can achieve good long-term CSR in MG, and VATS is an ideal alternative method. High-dose prescription of anticholinesterase and the advanced stage by Myasthenia Gravis Foundation of America (MGFA) classification have higher risks of postoperative crisis. Preoperative prescription of anticholinesterase alone and non-thymomatous MG are good prognostic factors. Thymectomy should also be considered for the ocular type of MG.

Comparative study on the value of accessory examinations in the diagnosis of ocular myasthenia gravis

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Fu-ru LIANG

2016-10-01

Full Text Available Objective To compare the sensitivity and specificity of ice test, neostigmine test and single fiber electromyography (SFEMG in the diagnosis of ocular myasthenia gravis (OMG, so as to establish an appropriate process for the diagnosis of OMG. Methods A total of 116 patients with newly onset ptosis and/or diplopia were detected by ice test, neostigmine test and SFEMG. Patients were followed up for 6 months to observe the disease changes and experimental treatment effects. Results Apart from patients whose diagnosis was in doubt or lost to follow - up, and who were diagnosed as generalized myasthenia gravis (GMG, the definite diagnosis was finally made in 81 patients, including 21 OMG patients and 60 patients with ptosis and/or diplopia caused by other diseases. The sensitivity of ice test for diagnosis of OMG was 95.24% (20/21, and specificity was 98.33%(59/60. The sensitivity of neostigmine test for diagnosis of OMG was 90.48% (19/21, and specificity was 85% (51/60. The sensitivity of SFEMG for diagnosis of OMG was 95.24% (20/21, and specificity was 80% (48/60. Among 3 tests, only specificity was found significant difference (χ2 = 5.232, P = 0.022. The specificity of ice test was better than that of neostigmine test (χ2 = 5.707, P = 0.017 and SFEMG (χ2 = 6.023, P = 0.014. Conclusions The ice test achieved high sensitivity and specificity in the diagnosis of OMG. The combination of ice test, neostigmine test and SFEMG may have important clinical value for the early diagnosis of OMG. DOI: 10.3969/j.issn.1672-6731.2016.10.008

An update on laboratory diagnosis in myasthenia gravis.

Science.gov (United States)

Oger, Joel; Frykman, Hans

2015-09-20

This review describes the state of the art for the use of laboratory testing in myasthenia gravis. The review brings a detailed description of the different clinical forms of auto-immune myasthenia and of the Lambert Eaton Myasthenic Syndrome (LEMS). They stress the differences between the different forms of acquired (auto-immune) myasthenia. Then they present a summary of the different antibodies found in the disease. They insist on the advantage of the RIPA assay to measure antibodies to the acetylcholine receptor. They stress the different types of contribution of each of these antibodies to the clinical diagnosis. They also describe the methods to measure each of the specific antibodies that have recently permitted to split the diagnosis: Abs to omega-conotoxin receptor in Lambert Eaton Myasthenic Syndrome (LEMS), abs to the acetylcholine receptor (AchR) in MG, Abs to muscle specific tyrosine kinase (MuSK) in Ab negative MG, and Abs to low molecular weight receptor related low-density lipo protein-4 (LRP-4). They also broach over the striated antibodies, less frequent and clinically less useful such as anti-titin, -ryanodine, -agrin and -rapsyn. This represent a 360° view of the field as presented in Toronto in October 2014. Copyright © 2015. Published by Elsevier B.V.

Staphylococcus cohnii spp urealyticus: relato de caso de um patógeno incomum

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d'Azevedo,Pedro Alves; Antunes,Ana Lúcia Sousa; Martino,Marinês Dalla Valle; Pignatari,Antonio Carlos Campos

2008-01-01

Staphylococcus coagulase negativos tem surgido como importantes agentes em infecções de pacientes hospitalizados. Neste estudo, relatamos o caso de bacteremia associada a cateter venoso central devido a Staphylococcus cohnii spp urealyticus isolado em hemocultura de um paciente do sexo masculino, 53 anos, internado em hospital geral da cidade de São Paulo. Discutimos nesse relato a dificuldade em identificar rotineiramente esse microrganismo no Laboratório de Microbiologia Clínica. Staphyloco...

Agonia de um mito pós-moderno: narrativas míticas arcaicas e o relato jornalístico do 11 de setembro

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Mario Geraldo Rocha da Fonseca

2006-01-01

Este é um trabalho de comparação entre o relato jornalístico e algumas narrativas antigas. O objetivo é encontrar afinidades estruturais entre as narrativas contemporâneas e as narrativas arcaicas. Para isso, tomou-se como objeto de estudo o relato dos atentados terroristas aos Estados Unidos da América (EUA) que ficaram conhecidos pelo dia e pelo mês - 11 de Setembro - em que ocorreram, em 2001. Para a análise, escolheu-se 15 primeiras - páginas de jornais de várias partes do mundo, a maiori...

7-Ketocholesterol and 7-hydroxycholesterol in pork meat and its gravy thermally treated without additives and in the presence of onion and garlic.

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Janoszka, Beata

2010-12-01

The effect of onion and garlic on the formation of two cholesterol oxidation products (COPs): 7-ketocholesterol and 7-hydroxycholesterol was evaluated by comparing their concentrations in meat and gravy samples obtained from three pork dishes prepared in the presence and absence of these flavourings. The concentration of these compounds in meat samples was between 82.4 and 1331.6 ng/g of cooked meat. Gravies contained lower amounts: from 18.3 to 45.6 ng/g of cooked meat. The addition of onion (30 g/100g of meat) caused a decrease in 7-ketocholesterol and 7-hydroxycholesterol concentrations in all of the investigated pork dishes by 9.5-79%, whilst the addition of 15 g of garlic to 100g of meat lowered the concentration by 17 to 88%. The greatest decrease was found in grilled minced chops. The quantitative assessment of 7-ketocholesterol and 7-hydroxycholesterol was carried out by thin-layer chromatography with densitometric detection. Copyright © 2010 The American Meat Science Association. Published by Elsevier Ltd.. All rights reserved.

Relato autobiográfico e interpretación: una concepción narrativa de la identidad personal

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Dante G. Duero

2006-05-01

Full Text Available En este trabajo analizo el lugar de la explicación y la interpretación dentro de las ciencias en general y de las ciencias sociales e históricas en particular. Expongo los argumentos de algunos autores en favor de la especificidad y la legitimidad del modelo narrativo dentro de las ciencias sociales e históricas. A continuación analizo la tesis que propone pensar a la identidad personal como parte de un relato que presenta grados diferentes de coherencia e integración. A partir de propuestas teóricas de diferentes autores, hago una caracterización de esta clase de narraciones y los elementos que la componen. Finalmente, reviso la tesis que afirma que la psicoterapia es un espacio para la reconstrucción y reelaboración de los relatos de los pacientes, con vistas a lograr narraciones más coherentes.

Muscle satellite cells are functionally impaired in myasthenia gravis: consequences on muscle regeneration.

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Attia, Mohamed; Maurer, Marie; Robinet, Marieke; Le Grand, Fabien; Fadel, Elie; Le Panse, Rozen; Butler-Browne, Gillian; Berrih-Aknin, Sonia

2017-12-01

Myasthenia gravis (MG) is a neuromuscular disease caused in most cases by anti-acetyl-choline receptor (AChR) autoantibodies that impair neuromuscular signal transmission and affect skeletal muscle homeostasis. Myogenesis is carried out by muscle stem cells called satellite cells (SCs). However, myogenesis in MG had never been explored. The aim of this study was to characterise the functional properties of myasthenic SCs as well as their abilities in muscle regeneration. SCs were isolated from muscle biopsies of MG patients and age-matched controls. We first showed that the number of Pax7+ SCs was increased in muscle sections from MG and its experimental autoimmune myasthenia gravis (EAMG) mouse model. Myoblasts isolated from MG muscles proliferate and differentiate more actively than myoblasts from control muscles. MyoD and MyoG were expressed at a higher level in MG myoblasts as well as in MG muscle biopsies compared to controls. We found that treatment of control myoblasts with MG sera or monoclonal anti-AChR antibodies increased the differentiation and MyoG mRNA expression compared to control sera. To investigate the functional ability of SCs from MG muscle to regenerate, we induced muscle regeneration using acute cardiotoxin injury in the EAMG mouse model. We observed a delay in maturation evidenced by a decrease in fibre size and MyoG mRNA expression as well as an increase in fibre number and embryonic myosin heavy-chain mRNA expression. These findings demonstrate for the first time the altered function of SCs from MG compared to control muscles. These alterations could be due to the anti-AChR antibodies via the modulation of myogenic markers resulting in muscle regeneration impairment. In conclusion, the autoimmune attack in MG appears to have unsuspected pathogenic effects on SCs and muscle regeneration, with potential consequences on myogenic signalling pathways, and subsequently on clinical outcome, especially in the case of muscle stress.

Skeletal-muscle CT, with special reference to polymyositis and myasthenia gravis

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Higashi, Yasuto; Ono, Shimato; Yasuda, Takeshi; Morimoto, Kenji; Terao, Akira; Shirabe, Teruo; Yokobayashi, Tsuneo (Kawasaki Medical School, Kurashiki, Okayama (Japan))

1984-10-01

We here report on skeletal-muscle CT at the thigh level as studied using a whole-body CT scanner, with special reference to polymyositis (PM) and myasthenia gravis (MG). Early diseased muscles appeared homogenous and were likely to be almost normal. The first sign of muscular atrophy was the appearance of small, patchy or linear, low-density tissues in several muscles. These low-density tissues gradually increased in number until finally the diseased muscles were totally replaced by low-density tissue. These pathological findings were more severe in PM than in MG. There was a maldistribution of low-density tissue in several cases of PM. According to these findings, skeletal-muscle CT was thought to be of great help for the recognition of the general condition of muscles and for the follow-up on the patients. We think skeletal-muscle CT has a very practical application for the better selection of suitable muscular biopsy and EMG sites and for the better clinical interpretation of these findings.

Skeletal-muscle CT, with special reference to polymyositis and myasthenia gravis

International Nuclear Information System (INIS)

Higashi, Yasuto; Ono, Shimato; Yasuda, Takeshi; Morimoto, Kenji; Terao, Akira; Shirabe, Teruo; Yokobayashi, Tsuneo

1984-01-01

We here report on skeletal-muscle CT at the thigh level as studied using a whole-body CT scanner, with special reference to polymyositis (PM) and myasthenia gravis (MG). Early diseased muscles appeared homogenous and were likely to be almost normal. The first sign of muscular atrophy was the apperance of small, patchy or linear, low-density tissues in several muscles. These low-density tissues gradually increased in number until finally the diseased muscles were totally replaced by low-density tissue. These pathological findings were more severe in PM than in MG. There was a maldistribution of low-density tissue in several cases of PM. According to these findings, skeletal-muscle CT was thought to be of great help for the recognition of the general condition of muscles and for the follow-up on the patients. We think skeletal-muscle CT has a very practical application for the better selection of suitable muscular biopsy and EMG sites and for the better clinical interpretation of these findings. (author)

Impact of radiotherapy on myasthenia gravis in patients with malignant thymomas

International Nuclear Information System (INIS)

Hou Xiuyu; Xu Yonggang; Gao Hong; Li Ming; Li Gaofeng; Liu Mingyuan

2006-01-01

Objective: To evaluate the change of myasthenia gravis(MG) during radiotherapy for patients with malignant thymomas. Methods: Forty-five with malignant thymomas patients with were analyzed. The median total dose was DT54.2 Gy in 1.8-2.0 Gy/fraction, 5 clays a week. Anti-cholinesterase, such as pyridostigmine was used to control the MG symptoms. Results: Forty-five patients completed radiotherapy on schedule except one from whom the treatment was was with drawn because of respiratory muscle involvement. Among these 44 patients, myasthenic symptom was relieved in 4 to various degrees, 4 progressed, 34 no change and 2 developed cholinergic crisis. Myasthenic symptom was not changed in one patient for whom radiotherapy had been standed before operation nor during the course of postoperative radiotherapy. Conclusions: A course of radiotherapy of DT54.2 Gy, on fractionation of DT1.8-2.0 Gy modal would not aggravate myasthenia. However, proper use of anti-cholinesterase, careful observation and timely drug-adjustment are necessary. (authors)

Anestesia em paciente com Distrofia Muscular de Duchenne: relato de caso

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Tonelli Deoclécio

2003-01-01

Full Text Available JUSTIFICATIVA E OBJETIVOS: A distrofia muscular de Duchenne é uma afecção recessiva ligada ao cromossomo X, geralmente diagnosticada na infância, acentuando-se progressivamente até agravar a função respiratória. O objetivo deste relato é apresentar um caso de um paciente com distrofia muscular de Duchenne diagnosticada há 2 anos, submetido à postectomia, sob anestesia geral com cetamina S. RELATO DO CASO: Paciente com 9 anos de idade com Distrofia Muscular de Duchenne diagnosticada há 2 anos, submetido à anestesia geral com levo-cetamina (1,5 mg.kg-1, por via venosa, sob ventilação espontânea assistida manualmente por sistema de Baraka (Mapleson A e bloqueio peniano com bupivacaína a 0,5% (25 mg. Foram usados monitores de pressão arterial não invasiva, oximetria de pulso, cardioscopia e temperatura esofagiana. No decorrer da cirurgia, o caso evoluiu sem intercorrências, sendo que no período pós-operatório o paciente apresentou alguns episódios de vômitos sem outras alterações significativas. Permaneceu internado por 24 horas, tendo alta hospitalar assintomático. CONCLUSÕES: A avaliação pré-anestésica cuidadosa, o uso de monitorização adequada e medicações que não predisponham o aparecimento de complicações tornam seguro o procedimento em pacientes portadores de Distrofia Muscular de Duchenne e seu pós-operatório.

Patient registries: useful tools for clinical research in myasthenia gravis.

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Baggi, Fulvio; Mantegazza, Renato; Antozzi, Carlo; Sanders, Donald

2012-12-01

Clinical registries may facilitate research on myasthenia gravis (MG) in several ways: as a source of demographic, clinical, biological, and immunological data on large numbers of patients with this rare disease; as a source of referrals for clinical trials; and by allowing rapid identification of MG patients with specific features. Physician-derived registries have the added advantage of incorporating diagnostic and treatment data that may allow comparison of outcomes from different therapeutic approaches, which can be supplemented with patient self-reported data. We report the demographic analysis of MG patients in two large physician-derived registries, the Duke MG Patient Registry, at the Duke University Medical Center, and the INNCB MG Registry, at the Istituto Neurologico Carlo Besta, as a preliminary study to assess the consistency of the two data sets. These registries share a common structure, with an inner core of common data elements (CDE) that facilitate data analysis. The CDEs are concordant with the MG-specific CDEs developed under the National Institute of Neurological Disorders and Stroke Common Data Elements Project. © 2012 New York Academy of Sciences.

Total body irradiation for myasthenia gravis with thymoma: case report

International Nuclear Information System (INIS)

Kang, Ki Mun; Choi, Ihl Bohng; Kim, In Ah

1999-01-01

Myasthenia Gravis (MG) is relatively rare occuring as one of important autoimmune disease to affect neuromuscular junction. This study was clinically to evaluate total body irradiation (TBI) against two patients including 33-year and 39-year females for chronic MG with thymoma who hospitalized in the St. Mary's Hospital, Catholic University since 1994 as well as who showed no response by thymectomy, immunotherapy and hormonal therapy. TBI designed by the dose of 150-180 cGy consisting of 10 cGy per fraction, three times a week, for 5-6 weeks using linear accelerator of 6 MV. During the treatment of TBI, they did complain acute side effect such as vomiting and also appear improved physical condition from 4-6 weeks after TBI. Through the follow-up period of 18 or 42 months after TBI, they did not have any symptomatic recurrence. Consequently, the results suggest that TBI can be used as an alternative tool for the patients concurrently for MG with thymoma who had been refractory to various conventional therapies like thymectomy, immunotherapy and hormonal therapy

CT findings of lymphofollicular thymic hyperplasia in adult myasthenia gravis

International Nuclear Information System (INIS)

Liu Fugeng; Wei Jiahu; Pan Jishu; Zhou Cheng; Chen Qihang; Yu Jingying; Wu Guogeng; Xu Xianhao

2006-01-01

Objective: To evaluate the CT findings of lymphofollicular thymic hyperplasia in adult myasthenia gravis (MG). Methods: The CT findings of thymus area of 134 adult patients with lymphofollicular thymic hyperplasia in MG were reviewed, all of them with surgically and histologically proven diagnosis, and compared with the CT findings of 165 normal subjects. Results: In the group of patient, CT showed enlargement of thymus in 31 patients, 5 patients had nodule or mass ( 3 cm) and 9 patients (6.7%) had normal size thymus with soft-tissue density, it can considered with thymic hyperplasia. The spotty or streak shadow showed in other patients, though it could not be certain diagnosed as thymic hyperplasia, but could not be except it. The thymus area tissue complete replacement by fatty density were not found in patient group. The CT findings of patients had marked difference when compared with group of normal subjects (P<0.01), except the spotty or streak shadows. Conclusion: CT scan is an important method in diagnosing thymic lymphofollicular hyperplasia of MG in adult. (authors)

Total body irradiation for myasthenia gravis with thymoma: case report

Energy Technology Data Exchange (ETDEWEB)

Kang, Ki Mun; Choi, Ihl Bohng; Kim, In Ah [College of Medicine, Catholic Univ., Seoul (Korea, Republic of)

1999-06-01

Myasthenia Gravis (MG) is relatively rare occuring as one of important autoimmune disease to affect neuromuscular junction. This study was clinically to evaluate total body irradiation (TBI) against two patients including 33-year and 39-year females for chronic MG with thymoma who hospitalized in the St. Mary's Hospital, Catholic University since 1994 as well as who showed no response by thymectomy, immunotherapy and hormonal therapy. TBI designed by the dose of 150-180 cGy consisting of 10 cGy per fraction, three times a week, for 5-6 weeks using linear accelerator of 6 MV. During the treatment of TBI, they did complain acute side effect such as vomiting and also appear improved physical condition from 4-6 weeks after TBI. Through the follow-up period of 18 or 42 months after TBI, they did not have any symptomatic recurrence. Consequently, the results suggest that TBI can be used as an alternative tool for the patients concurrently for MG with thymoma who had been refractory to various conventional therapies like thymectomy, immunotherapy and hormonal therapy.

Visualization of the thymus in myasthenia gravis

International Nuclear Information System (INIS)

Komatsu, Midori; Tanaka, Makoto; Morimatsu, Mitsunori; Hirai, Shunsaku; Heshiki, Atsuko

1982-01-01

We investigated whether CT had any advantage over pneumomediastinography (PMG) for the visualization of thymus in twenty-one patients with myasthenia gravis (MG). In two cases of thymoma which had been suspected with chest radiography, CT demonstrated an anterior mediastinal tumor distinguishable from other mediastinal organs, and for these cases PMG was not performed. Excluding three patients who were submitted to PMG only, CT of the anterior mediastinum was carried out in sixteen patients of MG, revealing thymic shadows in seven (44%). PMG followed by conventional tomography was done subsequently in three of these seven cases, for whom thymectomy was indicated because of uncontrollable myasthenic symptoms, and in all patients finger-like thymic shadows were disclosed. The vertical extension of thymus was more easily demonstrable by PMG than CT. PMG was carried out in six of nine patients in whom CT was negative, and in all cases thymic shadows were obvious with subsequent conventional tomography. Consequently, false negative rate of CT was at least 38% (6/16) with regard to the visualization of the nontumorous thymus. Although CT of the anterior mediastinum is useful as a screening method because of its non-invasiveness, its negative result does not rule out an absence of the pathologic thymus in view of its high false negative rate. In this regard, PMG is still necessary for the final determination of the thymic configuration in the MG patients. (J.P.N.)

FALANDO SOBRE SEXUALIDADE NA ADOLESCÊNCIA: RELATO DE EXPERIÊNCIA

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Eliane Goldberg Rabin

2014-09-01

Full Text Available Trata-se de um relato de experiência sobre atividade de extensão realizada por docentes e acadêmicos do Curso de Graduação em Enfermagem sobre sexualidade na adolescência. Atividade realizada com jovens de 10 a 19 anos, estudantes do ensino fundamental e médio de escolas públicas de Porto Alegre. Foram utilizadas diferentes estratégias e dinâmicas para o desenvolvimento das atividades, com vistas à maior participação dos jovens escolares. Os temas abordados foram as mudanças corporais, por meio da demonstração de moldes anatômicos, métodos contraceptivos, gravidez na adolescência e doenças sexualmente transmissíveis. A atividade constitui-se num espaço de esclarecimento de dúvidas e discussão, possibilitando a ampliação de conhecimentos para todos os participantes. Palavras-chave: adolescente, anatomia humana, ensino básico. Talking on sexuality in adolescence: experience report Abstract: This is an experience report on extension activities undertaken by teachers and students of the Undergraduate Nursing Course on adolescent sexuality. The activity was carried out with young people from 10 to 19 years of age, students from elementary and secondary public schools in Porto Alegre. Different strategies and dynamics for the development of activities were applied, seeking for greater participation of young scholars. The topics covered were the body changes through the demonstration of anatomical models, contraceptive methods, teenage pregnancy and sexually transmitted diseases. The activity constitutes a space for discussion and clarifying doubts, enabling the acquisition of information for all participants. Keywords: adolescent, human anatomy, primary education. Hablar de la sexualidad en la adolescencia: un relato de experiencia Resumen: se trata de un relato de experiencia en actividades de extensión realizadas por profesores y estudiantes de Enfermería sobre la sexualidad del adolescente. Actividad realizada con j

Raquitismo carencial: relato de caso = Vitamin D-dependent Rickets: Case report

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Porto, Juliana Antola

2005-01-01

Introdução: O raquitismo é uma doença do tecido ósseo em formação, onde há falha na calcificação e conseguinte acúmulo de tecido osteóide. O raquitismo carencial é decorrente da deficiência dos precursores da vitamina D, sendo a causa de raquitismo mais facilmente evitável e tratável. Objetivo: Relatar um caso, revisar a etiopatogenia e o tratamento do raquitismo carencial. Relato de caso: Paciente masculino com 2 anos de idade, previamente hígido, consulta com dificuldade de deambular ...

Raquitismo carencial: relato de caso = Vitamin D-dependent rickets: case report

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Porto, Juliana Antola; Piccoli, Cláudia; Salerno, Margareth Rodrigues; Henrique, Iara

2005-01-01

Introdução: O raquitismo é uma doença do tecido ósseo em formação, onde há falha na calcificação e conseguinte acúmulo de tecido osteóide. O raquitismo carencial é decorrente da deficiência dos precursores da vitamina D, sendo a causa de raquitismo mais facilmente evitável e tratável. Objetivo: Relatar um caso, revisar a etiopatogenia e o tratamento do raquitismo carencial. Relato de caso: Paciente masculino com 2 anos de idade, previamente hígido, consulta com dificuldade de deambular progre...

The spectrum of myopathies in the city of São Paulo

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José A. Levy

1976-12-01

Full Text Available A review of all myopathic patients treated at the Neurologic Clinic of the Medical School of the University of São Paulo during the past 15 years is reported. A total of 466 cases were examined and distributed as follows: 56% of progressive muscular dystrophy; 31% of myasthenia gravis; 6% of polymyositis; 4% of myotonic dystrophy; and the remainder of several different diseases (central core disease, Kearns-syndrome, myotonia congenita, adynamia episodica hereditaria, diabetic myopathy and Eaton-Lambert syndrome. Enzymatic dosages, electromyography, muscle biopsy, electrocardiography and genetic counselling are also reported.Os autores fazem uma revisão de todos os casos de miopatias tratados na Clínica Neurológica da F.M.U.S.P. durante os últimos 15 anos. Foram examinados 466 casos, assim distribuídos: 56% de distrofia muscular progressiva; 31% de miastenia grave; 6% de polimiosite; 4% de distrofia miotônica e, o restante, de várias outras moléstias (Central core disease, síndrome de Kearns, miotonia congênita, adinamia episódica hereditária, miopatia diabética e síndrome de Eaton-Lambert. São relatadas também as dosagens enzimáticas, eletromiografia, biópsia muscular, eletrocardiografia e aconselhamento genético.

Definiendo a los otros: relatos académicos sobre la diversidad en la escuela

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María Luisa Jiménez-Rodrigo

2016-01-01

Full Text Available Este artículo indaga en los usos académicos del concepto diversidad en el ámbito escolar a partir del análisis de la literatura científica española. El método se basa en el análisis documental partiendo de una exhaustiva búsqueda y revisión de 218 artículos publicados entre 2006 y 2012 en revistas indexadas en CSIC-ISOC y DIALNET. Se aplicó un esquema estructural de análisis que permitió identificar diferentes configuraciones narrativas en torno a la definición del “otro” en la escuela. Los resultados señalan dos relatos hegemónicos: institucional, basado en las clasificaciones legislativas del alumnado en función de sus capacidades y rendimientos escolares; e intercultural, sustentado en categorizaciones en torno a las diferencias culturales derivadas de la condición inmigrante-extranjera. Concluimos que en ambos relatos se proyecta predominantemente una visión esencializadade la diversidad construida desde parámetros dicotómicos y excluyentes, sin apenas considerar otras categorías sociales y económicas en la conformación de las diferencias y desigualdades educativas.

La función del relato en la producción social de sentido

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Cristina Palomar Verea

2001-01-01

Full Text Available Este artículo es una reflexión sobre la producción de sentido en las prácticas sociales a partir de la discusión en torno a la relación entre la historia, la investigación antropológica y la narrativa, intentando acercar esta discusión al análisis de las prácticas discursivas, entendidas como la puesta en acto del discurso en tanto vehículo primario a través del cual las relaciones sociales son producidas y reproducidas. La discursividad, entonces, hace referencia al conjunto de fenómenos en y a través de los cuales tiene lugar la producción de sentido que constituye a una sociedad como tal, mediante distintos relatos de las prácticas sociales que pueden ser vistas como textos narrativos más allá de la escritura. En este punto se retoman los planteamientos hermenéuticos de Paul Ricoeur sobre la noción de “texto”. Ricoeur se refiere al uso que hace Freud de esta noción: El relato del sueño es un texto ininteligible que el análisis sustituye por otro texto más inteligible.

DISCRIMINACION ETNICA EN RELATOS DE LA EXPERIENCIA ESCOLAR MAPUCHE EN PANGUIPULLI (CHILE)

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Poblete S,María Pía

2003-01-01

El propósito de este artículo es indagar en las percepciones y valoraciones de la experiencia escolar en un contexto interétnico. Presentamos información proveniente de 30 relatos sobre la educación y la experiencia escolar de personas mapuches de la precordillera de la Comuna de Panguipulli. Los resultados nos muestran que las experiencias en la escuela estuvieron marcadas por la discriminación étnica en la mayoría de los entrevistados. La discriminación se vivió en la interacción con profes...

Computed tomography of the thymus after pneumomediastinography in patients with myasthenia gravis

International Nuclear Information System (INIS)

Hirose, Genjiro; Yamamoto, Teiji; Kosoegawa, Hiroshi; Saeki, Mitsuo; Hayashi, Toshiharu

1982-01-01

Nine patients with myasthenia gravis (MG) had plain chest X-ray, tomography, computed tomography (CT) of the thorax, pneumomediastinography (PMG) and post-PMG CT scans. Routine chest radiography detected mediastinal masses in two patients and conventional laminogram provided no additional informations. CT yielded anterior mediastinal increased densities in seven patients (78%), whereas post-PMG CT delineated masses (thymoma or thymic hyperplasia) or thymic gland in all cases (100%), but these procedures could not distinguish thymic tumor from nontumorous conditions. PMG and post-PMG CT scans are superior to other radiographic techniques in detecting mediastinal abnormalities with more clear delineation of thymoma or thymic gland. When an abnormal density is present in the mediastinum of a patient with MG, PMG and post-PMG CT scans should be done to outline the mediastinal anatomy, to determine the size, extent, invasiveness of thymoma or thymic gland prior to thymectomy. (author)

Computed tomography of the thymus after pneumomediastinography in patients with myasthenia gravis

Energy Technology Data Exchange (ETDEWEB)

Hirose, Genjiro; Yamamoto, Teiji; Kosoegawa, Hiroshi; Saeki, Mitsuo; Hayashi, Toshiharu (Kanazawa Medical Univ., Ishikawa (Japan))

1982-09-01

Nine patients with myasthenia gravis (MG) had plain chest X-ray, tomography, computed tomography (CT) of the thorax, pneumomediastinography (PMG) and post-PMG CT scans. Routine chest radiography detected mediastinal masses in two patients and conventional laminogram provided no additional information. CT yielded anterior mediastinal increased densities in seven patients (78%), whereas post-PMG CT delineated masses (thymoma or thymic hyperplasia) or thymic gland in all cases (100%), but these procedures could not distinguish thymic tumor from nontumorous conditions. PMG and post-PMG CT scans are superior to other radiographic techniques in detecting mediastinal abnormalities with more clear delineation of thymoma or thymic gland. When an abnormal density is present in the mediastinum of a patient with MG, PMG and post-PMG CT scans should be done to outline the mediastinal anatomy, to determine the size, extent, invasiveness of thymoma or thymic gland prior to thymectomy.

Bloqueio do nervo isquiático via médio lateral da coxa como opção anestésica no trauma: relato de caso

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Geier, Karl Otto

2006-01-01

JUSTIFICATIVA E OBJETIVOS: Nos pacientes com trauma de membros inferiores e com estômago cheio, tantos os bloqueios de plexos nervosos como os bloqueios de nervos periféricos isolados são procedimentos incomuns, prevalecendo os bloqueios peridural e subaracnóideo como primeira indicação. Este relato de caso registrou a escolha do bloqueio do nervo isquiático, como melhor indicação para anestesia em paciente de estômago cheio e traumatismo grave de pé. RELATO DO CASO: Paciente do sexo masculin...

APLICAÇÃO DE ELETROLIFTING EM RUGAS FACIAIS: RELATO DE CASO

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Paola Emanuele Bragato; Lorena Pohl Fornazari; Keila Cristiane Deon

2013-01-01

As rugas são sulcos ou pregas cutâneas que se apresentam de forma progressiva pelo declínio da junção dermoepidérmica, em que ocorre um distúrbio nos componentes do tecido conjuntivo. O colágeno torna-se mais rígido e a elastina perde a sua elasticidade natural com a redução do número de fibras elásticas e de outros componentes do tecido. O objetivo do estudo foi verificar osefeitos da aplicação de eletrolifting em rugas. É um relato de caso, com uma participante do sexo feminino, de 54 anos,...

Safety and efficacy of eculizumab in anti-acetylcholine receptor antibody-positive refractory generalised myasthenia gravis (REGAIN): a phase 3, randomised, double-blind, placebo-controlled, multicentre study.

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Howard, James F; Utsugisawa, Kimiaki; Benatar, Michael; Murai, Hiroyuki; Barohn, Richard J; Illa, Isabel; Jacob, Saiju; Vissing, John; Burns, Ted M; Kissel, John T; Muppidi, Srikanth; Nowak, Richard J; O'Brien, Fanny; Wang, Jing-Jing; Mantegazza, Renato

2017-12-01

Complement is likely to have a role in refractory generalised myasthenia gravis, but no approved therapies specifically target this system. Results from a phase 2 study suggested that eculizumab, a terminal complement inhibitor, produced clinically meaningful improvements in patients with anti-acetylcholine receptor antibody-positive refractory generalised myasthenia gravis. We further assessed the efficacy and safety of eculizumab in this patient population in a phase 3 trial. We did a phase 3, randomised, double-blind, placebo-controlled, multicentre study (REGAIN) in 76 hospitals and specialised clinics in 17 countries across North America, Latin America, Europe, and Asia. Eligible patients were aged at least 18 years, with a Myasthenia Gravis-Activities of Daily Living (MG-ADL) score of 6 or more, Myasthenia Gravis Foundation of America (MGFA) class II-IV disease, vaccination against Neisseria meningitides, and previous treatment with at least two immunosuppressive therapies or one immunosuppressive therapy and chronic intravenous immunoglobulin or plasma exchange for 12 months without symptom control. Patients with a history of thymoma or thymic neoplasms, thymectomy within 12 months before screening, or use of intravenous immunoglobulin or plasma exchange within 4 weeks before randomisation, or rituximab within 6 months before screening, were excluded. We randomly assigned participants (1:1) to either intravenous eculizumab or intravenous matched placebo for 26 weeks. Dosing for eculizumab was 900 mg on day 1 and at weeks 1, 2, and 3; 1200 mg at week 4; and 1200 mg given every second week thereafter as maintenance dosing. Randomisation was done centrally with an interactive voice or web-response system with patients stratified to one of four groups based on MGFA disease classification. Where possible, patients were maintained on existing myasthenia gravis therapies and rescue medication was allowed at the study physician's discretion. Patients, investigators

Decreased bone mineral density in experimental myasthenia gravis in C57BL/6 mice.

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Oshima, Minako; Iida-Klein, Akiko; Maruta, Takahiro; Deitiker, Philip R; Atassi, M Zouhair

2017-09-01

Experimental autoimmune myasthenia gravis (EAMG), an animal model of myasthenia gravis (MG), can be induced in C57BL/6 (B6, H-2  b ) mice by 2-3 injections with Torpedo californica AChR (tAChR) in complete Freund's adjuvant. Some EAMG mice exhibit weight loss with muscle weakness. The loss in body weight, which is closely associated with bone structure, is particularly evident in EAMG mice with severe muscle weakness. However, the relationship between muscle weakness and bone loss in EAMG has not been studied before. Recent investigations on bone have shed light on association of bone health and immunological states. It is possible that muscle weakness in EAMG developed by anti-tAChR immune responses might accompany bone loss. We determined whether reduced muscle strength associates with decreased bone mineral density (BMD) in EAMG mice. EAMG was induced by two injections at 4-week interval of tAChR and adjuvants in two different age groups. The first tAChR injection was either at age 8 weeks or at 15 weeks. We measured BMD at three skeletal sites, including femur, tibia, and lumbar vertebrae, using dual energy X-ray absorptiometry. Among these bone areas, femur of EAMG mice in both age groups showed a significant decrease in BMD compared to control adjuvant-injected and to non-immunized mice. Reduction in BMD in induced EAMG at a later-age appears to parallel the severity of the disease. The results indicate that anti-tAChR autoimmune response alone can reduce bone density in EAMG mice. BMD reduction was also observed in adjuvant-injected mice in comparison to normal un-injected mice, suggesting that BMD decrease can occur even when muscle activity is normal. Decreased BMD observed in both tAChR-injected and adjuvant-injected mice groups were discussed in relation to innate immunity and bone-related immunology involving activated T cells and tumour necrosis factor-related cytokines that trigger osteoclastogenesis and bone loss.

Keep An Eye Out For Myasthenia Gravis Patients With An Eye Out

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A Arturo eLeis

2014-07-01

Full Text Available Eye trauma and blindness are common in the United States, with an incidence of over 2 million cases per year and 25 million blind adults, respectively. However, literature is surprisingly scarce on the potential confounding effect of eye trauma or blindness on the diagnosis of myasthenia gravis (MG, an autoimmune neuromuscular disease in which fluctuating ocular symptoms are the most distinguishing feature. We present the case of a 75 year-old man with eye enucleation referred for electrodiagnostic evaluation of the right upper limb after an accidental fall. Neurological examination showed proximal muscle weakness, but MG was not initially considered because the patient lacked the classic ocular symptoms of MG. The delay in diagnosis resulted in worsening of systemic MG symptoms, although in other patients it may have precipitated MG crisis or possibly death. Greater awareness that eye trauma or blindness can prevent expression of ocular symptoms in neuromuscular disorders is needed to avoid morbidity associated with an erroneous or delayed diagnosis.

Caspase-1 inhibitor regulates humoral responses in experimental autoimmune myasthenia gravis via IL-6- dependent inhibiton of STAT3.

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Wang, Cong-Cong; Zhang, Min; Li, Heng; Li, Xiao-Li; Yue, Long-Tao; Zhang, Peng; Liu, Ru-Tao; Chen, Hui; Li, Yan-Bin; Duan, Rui-Sheng

2017-08-24

We have previously demonstrated that Cysteinyl aspartate-specific proteinase-1 (caspase-1) inhibitor ameliorates experimental autoimmune myasthenia gravis (EAMG) by inhibited cellular immune response, via suppressing DC IL-1 β, CD4 + T and γdT cells IL-17 pathways. In this study, we investigated the effect of caspase-1 inhibitor on humoral immune response of EAMG and further explore the underlying mechanisms. An animal model of MG was induced by region 97-116 of the rat AChR α subunit (R97-116 peptide) in Lewis rats. Rats were treated with caspase-1 inhibitor Ac-YVAD-cmk intraperitoneally (i.p.) every second day from day 13 after the first immunization. Flow cytometry, western blot, immunofluorescence, and enzyme-linked immunosorbent assay (ELISA) were performed to evaluate the neuroprotective effect of caspase-1 inhibitor on humoral immune response of EAMG. The results showed that caspase-1 inhibitor reduced the relative affinity of anti-R97-116 IgG, suppressed germinal center response, decreased follicular helper T cells, and increased follicular regulatory T cells and regulatory B cells. In addition, we found that caspase-1 inhibitor inhibited humoral immunity response in EAMG rats via suppressing IL-6-STAT3-Bcl-6 pathways. These results suggest that caspase-1 inhibitor ameliorates EAMG by regulating humoral immune response, thus providing new insights into the development of myasthenia gravis and other autoimmune diseases therapies. Copyright © 2017 Elsevier B.V. All rights reserved.

[Myasthenia gravis with the electrographic response of a myasthenic syndrome. Report of a case].

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Werneck, L C; Bittencourt, P C; Nóvak, E M

1985-06-01

It is reported a case of a 61 years-old man with progressive asthenia, disfagia, disphonia and diplopia, of variable intensity during the day, who had a very good response to anticolinesterasic drugs and corticosteroids. The repetitive stimulation tests at low frequency, resulted in large increment (maximum 275%) of the basal voltage after exercise. At high frequency he also had a large increment. Radiological and laboratory investigation three times in a seven-year period was normal, without evidence of any neoplasia. Muscle biopsy showed a type II muscle fiber atrophy. The repetitive stimulation tests repeated three times, was typical of myasthenia gravis in one occasion and in the other two, typical of myasthenic syndrome (increment of 418%). A discussion about other cases with similar findings is made after a review of the literature.

Treatment of epilepsy in patients with myasthenia gravis: Is really harder than it looks?

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Lorenzoni, Paulo José; Ducci, Renata Dal-Prá; Tensini, Tallulah Spina; Dalledone, Giuliano; Kay, Claudia Suemi Kamoi; de Paola, Luciano; Werneck, Lineu Cesar; Scola, Rosana Herminia; Silvado, Carlos

2017-10-01

The relationship between myasthenia gravis (MG) and epilepsy has been rarely reported. As consequence, there are no specific guidelines for the management of these conditions when they mutually occur. We reported on three patients in whom epilepsy and MG are coexisting, but in different clinical settings. Two patients were treated with antiepileptic drugs which improved their symptoms. One patient has controlled the seizures after a successful anterior temporal lobectomy with no appreciable consequences to her MG. We discuss the difficulties in the management of epilepsy in patients with MG. In addition, we report on the first epileptic surgery in a MG patient, indicating that this surgical procedure as a safe option for the treatment of intractable epilepsy in patients with MG. Copyright © 2017. Published by Elsevier Ltd.

The Clinical Outcome and Therapeutic Treatment of a Patient with Double Seronegative Myasthenia Gravis

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Azanjac Ana

2014-03-01

Full Text Available Prikazali smo slučaj 22-godišnjeg muskarca sa kliničkom slikom otežanog gutanja i dvoslika. Bolest myasthenia gravis (MG je dijagnostikovana na osnovu pozitivnog faramakološkog testa, pozitivnog testa neuromišićne transmisije, kompjuterizovane tomografije medijastinuma. Antitela protiv acetilholinskog receptora (AChR i antitela protiv mišićno specifičnog receptora za tirozin kinazu (MuSK. bila su negativna. Zabeleženo je delimično poboljšanje u neurološkom nalazu na ordiniranu terapiju Piridostigminom, Ciklosporinom, kortikosteroidima. Bolesnik je imao povoljan odgovor na izmenu plazme sa povlačenjem svih kliničkih siimptoma što potvrdjuje hipotezu o učešću humoralnih faktora u patogenezi dvostruko negativne MG.

Manejo nutricional em paciente cirúrgico com adenocarcinoma de pâncreas: Um relato de caso

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Caroline Otoni da Silva

2018-03-01

Full Text Available O objetivo desse relato de caso é demonstrar como o manejo nutricional pode ser realizado em pacientes idosos com diagnóstico de adenocarcinoma de pâncreas e submetidos a cirurgia eletiva. Resultados: A dieta proposta supria a necessidade energética e calórica da paciente, o objetivo de recuperação do estado nutricional e preparo da paciente para o procedimento cirúrgico foi atingido, tendo em vista o prazo em que ocorreram os fatos. Foi ofertado o suplemento com imunonutrientes por um período de 12 dias no pré-operatório, sendo continuada no pós-operatório, conforme recomendado pelo DITEN, 2011. Nota se através desse relato a importância do uso das ferramentas de triagem e diagnóstico nutricional, para detecção precoce dos indivíduos desnutridos ou em risco nutricional, otimizando assim o planejamento para a oferta de calorias e nutrientes específicos a fim de melhorar o estado nutricional e prepara-los para a cirurgia. Descritores: Terapia nutricional; Neoplasias pancreáticas; Dietoterapia.

Juvenile myasthenia gravis in Norway: Clinical characteristics, treatment, and long-term outcome in a nationwide population-based cohort.

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Popperud, T H; Boldingh, M I; Rasmussen, M; Kerty, E

2017-09-01

This study aimed to characterize juvenile myasthenia gravis in a national population-based cohort in Norway, and to evaluate long-term outcome and potential differences correlated with prepubertal versus postpubertal disease onset. Patients with onset of myasthenia gravis aged ≤18 years were identified through multiple strategies. Retrospective clinical data were collected by means of medical charts. All patients had an updated clinical examination. Cases were divided into prepubertal and postpubertal onset using age 12 years as the cut off. In total, 75 patients were identified of whom 63 were included in the study: 21 in the prepubertal and 42 in the postpubertal onset group. There was a female preponderance in both groups. In total, 59% presented with ocular symptoms, but the great majority of patients in both groups generalized during the two first years of the disease. Myasthenic crisis was more frequent in the prepubertal onset group. All patients were initially treated with pyridostigmine, 26 with steroids, and 17 with other immunosuppressive treatment. The postpubertal cases were more often treated with immunosuppressive therapy. Fifty patients (79%) underwent thymectomy. The general outcome was favourable: 57% became asymptomatic and only four subjects failed to attain clinical improvement. One-third had at least one additional autoimmune disease. Despite frequent symptom generalization and a subgroup of prepubertal onset with severe disease, the long-term outcome was good, especially in the thymectomized prepubertal onset group. Polyautoimmunity occurred in both groups in one-third. Copyright © 2017 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved.

RELATOS DE VIDA Y RELIGIOSIDAD POPULAR. ORIGEN Y SENTIDOS DE LA FIESTA DE LA VIRGEN DE URKUPIÑA EN BAHÍA BLANCA.

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Graciela Hernández

2010-10-01

Full Text Available Proponemos conocer el origen e ir identificando los sentidos de una de las manifestaciones de la religiosidad popular que se producen en Bahía Blanca: las festividades en honor a la Virgende Urkupiña. Es una devoción mariana surgida en Bolivia y profesada por migrantes bolivianos y de las provincias del noroeste argentino, como jujeños, salteños y tucumanosradicados en esta ciudad y la zona de influencia.Se trata de una investigación realizada con metodología cualitativa etnográfica. Del trabajo de campo hemos seleccionados relatos de vida que nos resultan significativos en tanto encontramos en ellos las explicaciones que los sujetos entrevistados dan de los procesos sociales. El relato de vida es el fragmento del texto oral que da cuenta de aquellos temas que nos hemos propuesto conocer.Realizamos un breve recorrido por la bibliografía de la última década que toma el tema la fiesta de la Virgen de Urkupiña en distintos lugares del país. Hemos visto que cada uno de los autores focaliza en distintos aspectos de la festividad o en las distintas perspectivas teóricas;nosotros queremos aportar nuevas explicaciones sobre el origen de estas celebraciones y rituales a partir de la interpretación de los relatos de vida seleccionados

Intussuscepção jejunojejunal em potro: relato de caso

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Paulo Ari Tiethböl Leiria

2017-03-01

Full Text Available As intussuscepções são acidentes intestinais incomuns e que podem resultar em desconforto abdominal agudo e progressivo ou recorrente em potros. Esta condição afeta potros neonatos de forma assintomática e de 3 a 12 meses são emergências cirúrgicas quando envolvem o intestino delgado, principalmente o jejuno. A avaliação ultrassonográfica transabdominal é um importante para o exame desses animais devido às limitações de seu tamanho para avaliações do abdômen agudo. O presente relato de caso mostra a importância da utilização do ultrassom transabdominal em potros com abdômen agudo

Anestesia em paciente com síndrome de Marshall-Smith: relato de caso

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Mandim, Beatriz Lemos da Silva; Fonseca, Neuber Martins; Ruzi, Roberto Araújo; Temer, Paulo Cezar Silva

2007-01-01

JUSTIFICATIVA E OBJETIVOS: A síndrome Marshall-Smith é uma doença rara, caracterizada por dismorfismo facial, acelerada maturação óssea, atraso no desenvolvimento neuropsicomotor e anormalidade das vias aéreas. Os pacientes com essa síndrome apresentam grande probabilidade de complicações anestésicas, sobretudo com relação ao manejo das vias aéreas. Há poucos dados na literatura anestésica a respeito desta síndrome. O objetivo deste relato foi apresentar as dificuldades e a conduta anestésica...

Presentación del Libro: Cuasi una Fantasía. Cuentos y Relatos.

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Efraím Otero Ruiz

2006-01-01

Meses y varios huesos rotos después puedo, al fin, presentar ante ustedes mi libro de cuentos y relatos, salido de las prensas de Kimpres -con el generoso apoyo de esta Academia desde septiembre del año pasado. Afortunadamente, por tratarse de “Cuasi una fantasía” son narraciones que solo están parcialmente ancladas en el tiempo y comprenden una vivencia de medio siglo, no sólo por los personajes-hoy casi todos desaparecidos- sino por los sucesos, que a veces -aunque tomados ...

Síndrome de Isaacs: relato de um caso

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Hélio Ghizoni Teive

1988-06-01

Full Text Available Relato do caso de paciente do sexo feminino com 40 anos de idade, com quadro progressivo de diminuição de força, disfonia, disfagia, dispnéia e hiperidrose. O exame físico revelava contratura em flexão das mãos, déficit de força muscular, fasciculações, mioquimia facial e pseudomiotonia nas mãos, A eletromiografia mostrou atividade elétrica contínua em repouso. O estudo histoquímico muscular revelou atrofia de fibras do tipo II, enquanto a microscopia eletrônica mostrou dilatação importante das cisternas do retículo sarcoplasmático. O diagnóstico de síndrome de Isaacs foi firmado. Excelente resposta clínica ao emprego de carbamazepina foi observada. São discutidos e revistos vários aspectos relacionados a essa rara síndrome.

Maison de verre / Zonestraal. Relato de dos edificios

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Jan Molema

2014-01-01

Full Text Available Desde los años sesenta del siglo XX el sanatorio Zonnestraal de Hilversum y la Maison de Verre en París han sido descritos extensamente en la literatura de la arquitectura moderna. Sin embargo, no se ha hecho ningún esfuerzo por colocarlos juntos en un mismo relato. Este ensayo es un esfuerzo para enmendar ese vacío. Ambos están relacionados íntimamente por sus autores: Bernard Bijvoet, como codiseñador de los planos de la Maison, y Johannes Duiker, quien trabajó con el anterior en los preliminares de Zonnestraal, aunque lo finalizó en solitario, al mismo tiempo que se terminó el diseño de la casa. En el trabajo se cuestiona además, ofreciendo fundadas conjeturas, la creencia establecida de que se precisó una nueva estructura metálica para sustentar la inalterada vivienda situada encima de la Maison de Verre.

El relato corto en el cine. Literatura en 3-D: ‘Memento' de Christopher Nolan (2000

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David Caldevilla Domínguez

2012-07-01

Full Text Available Normal 0 21 false false false /* Style Definitions */ table.MsoNormalTable {mso-style-name:"Tabla normal"; mso-tstyle-rowband-size:0; mso-tstyle-colband-size:0; mso-style-noshow:yes; mso-style-parent:""; mso-padding-alt:0cm 5.4pt 0cm 5.4pt; mso-para-margin:0cm; mso-para-margin-bottom:.0001pt; mso-pagination:widow-orphan; font-size:10.0pt; font-family:"Times New Roman"; mso-ansi-language:#0400; mso-fareast-language:#0400; mso-bidi-language:#0400;} La narrativa literaria y la cinematográfica se erigen como dos lenguajes separados por la sustancia expresiva en la que se basan. En el caso de las adaptaciones literarias, estas diferencias se manifiestan otorgando una especial ventaja al cine ya que existen licencias expresivas imposibles para el texto. En el presente artículo se analiza una de las adaptaciones más sorprendentes, innovadoras y rupturantes de la historia del cine, ‘Memento’, que explota, como ninguna otra antes, la posibilidad de romper el hilo narrativo llamado convencional o lineal, para pasar a generar una trama en la que el espectador se erige en lecto-autor. Este tropo cinematográfico halla sus mejores capacidades de expresión en dos géneros (entendiendo género no sólo en su aspecto formal, sino en el temático tan antiguos como el cine mismo: el de ciencia-ficción y el negro, género este último al que pertenece la película analizada, elegida para mejor demostrar esas posibilidades que el cine ofrece a la literatura a través de las adaptaciones. El hecho de  que ‘Memento’ esté basada en un relato corto permite que, además, se consideren las ventajas que este tipo de relatos ofrece a los adaptadores-guionistas frente a los relatos largos como las novelas o las obras teatrales.

Caesarean section in a patient with Myasthenia Gravis: A bigger challenge for the anesthesiologist than the obstetrician

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Manoj K Sanwal

2012-01-01

Full Text Available Myasthenia Gravis (MG is an acquired, autoimmune disorder affecting neuromuscular junction presenting with easy fatigability, progressive weakness, diplopia, difficulty in speaking and swallowing and even ventilatory failure in severe cases. During pregnancy the disease may go into remission or may exacerbate at any time during first, second and third trimesters or postpartum period. We are reporting the case of a 28 year old primigravida, known case of MG, who underwent caesarean section and developed muscular weakness on third postoperative day. Her neonate also had tachypnoea and hypotonia, Both, the mother and the baby were managed aggressively and responded well to therapy.

Los huicholes, de Fernando Benítez: un relato de viaje

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Lucero Margarita Aguirre-Valdés

2015-01-01

Full Text Available El género relato de viaje cuenta con una nueva apertura para su campo de estudio gracias a las investigaciones del español Luis Alburquerque García. Haciendo uso de sus aportaciones teóricas, este trabajo busca aplicarlas al texto Los huicholes , del escritor y periodista Fernando Benítez. Se demostró que en la construcción de dicha obra participan de igual manera lo referencial y la literatura gracias al constante uso del lenguaje poético; al mismo tiempo que se mantiene en el límite entre dos disciplinas, una objetiva e informativa, y otra, subjetiva y estética.

Anestesia em paciente com síndrome de Gilbert: relato de caso Anestesia en paciente con síndrome de Gilbert: relato de caso Anesthesia in a patient with Gilbert's syndrome: case report

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Fabiano Timbó Barbosa

2004-06-01

Full Text Available JUSTIFICATIVA E OBJETIVOS: A síndrome de Gilbert é uma doença crônica benigna, a qual leva à icterícia recorrente com grande aumento da bilirrubina não conjugada, que pode levar à toxicidade após o uso de medicações utilizadas na prática diária. O objetivo deste relato é descrever a conduta anestésica em uma paciente com síndrome de Gilbert, submetida à cirurgia videolaparoscópica. RELATO DO CASO: Paciente do sexo feminino, com 22 anos, portadora da síndrome de Gilbert, submetida à cirurgia videolaparoscópica sob anestesia geral com propofol, alfentanil, succinilcolina, atracúrio e isoflurano. Não houve sinais de toxicidade durante a anestesia. A paciente apresentou recuperação pós-operatória sem intercorrências e recebeu alta hospitalar após três dias. CONCLUSÕES: O paciente portador da síndrome de Gilbert pode ser submetido à anestesia geral de forma segura sem o aparecimento de toxicidade desde que sejam evitados os fatores que possam levar à diminuição da atividade da glicuroniltransferase.JUSTIFICATIVA Y OBJETIVOS: La síndrome de Gilbert es una enfermedad crónica benigna la cual lleva a la ictericia recurrente con grande aumento de la bilirrubina no conjugada, que puede llevar a la toxicidad después del uso de medicamentos utilizadas en la práctica diaria. El objetivo de este relato es describir la conducta anestésica en una paciente con síndrome de Gilbert, sometida a cirugía videolaparoscópica. RELATO DEL CASO: Paciente del sexo femenino, con 22 años, portadora de la síndrome de Gilbert fue sometida a cirugía videolaparoscópica bajo anestesia general con propofol, alfentanil, succinilcolina, atracúrio e isoflurano. No hubo señales de toxicidad durante la anestesia. La paciente presentó recuperación pós-operatoria sin intercurrencias y recibió alta hospitalar después de tres días. CONCLUSIONES: El paciente portador del síndrome de Gilbert puede ser sometido a la anestesia general de

Anestesia para paciente portador da equência de moebius: relato de caso Anestesia para Paciente Portador de la Secuencia de Moebius: relato de Caso Anesthesia in a patient with moebius sequence: case report

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Adriano Bechara de Souza Hobaika

2009-06-01

Full Text Available JUSTIFICATIVA E OBJETIVOS: A sequência de Moebius (SM é uma rara paralisia do VI e VII nervos cranianos. Alterações craniofaciais estão presentes em aproximadamente 90% destes pacientes, o que pode tornar a intubação traqueal muito difícil. RELATO DO CASO: Paciente do sexo masculino, 2 anos e 5 meses, portador de SM, submetido à broncoscopia flexível para avaliação de laringotraqueomalácia. Comorbidades: crises de broncoespasmo e comunicação interventricular. Realizou-se indução anestésica com sevoflurano em O2 a 100% e venóclise. Introduziu-se máscara laríngea AMBU® número 2,5 e o fibrobroncoscópio foi introduzido através da mesma. O procedimento foi realizado sem complicações e a broncoscopia revelou-se normal. O paciente recebeu alta para casa após duas horas. CONCLUSÕES: O controle das vias aéreas é o grande desafio nestes pacientes, havendo relato de falha ou dificuldade de intubação em 13 pacientes de uma série de 41 casos analisados. Micrognatia, retrognatia, hipoplasia mandibular e fenda palatina são algumas características destes pacientes. Em outra grande série com 106 anestesias em pacientes com SM há descrição do uso da máscara laríngea em um caso. Parece não haver contra-indicação a realizar o procedimento em regime ambulatorial. Há relato de aspiração pulmonar e obstrução respiratória na sala de recuperação devido à dificuldade em deglutir e eliminar as secreções da boca e recomenda-se administrar antisialogogos.JUSTIFICATIVA Y OBJETIVOS: La secuencia de Moebius (SM es una rara parálisis del VI y VII nervios cranianos. Las alteraciones craneofaciales están presentes en aproximadamente un 90% de esos pacientes, lo que puede hacer con que la intubación traqueal sea muy difícil. RELATO DEL CASO: Paciente del sexo masculino, 2 años y 5 meses, portador de SM, sometido a la broncoscopia flexible para la evaluación de laringotraqueomalacia. Comorbidades: crisis de broncoespasmo

Anestesia em paciente portadora de síndrome de Mckusick-Kaufman: relato de caso

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Hobaika,Adriano Bechara de Souza; Borges,Ziltomar Donizetti; Teixeira,Vera Coelho

2004-01-01

JUSTIFICATIVA E OBJETIVOS: A síndrome de Mckusick-Kaufman é uma doença rara, caracterizada tipicamente por hidrometrocolpos, polidactilia e defeitos cardíacos congênitos. Pacientes portadores desta doença podem ser submetidos a diversos procedimentos cirúrgicos durante a sua vida e o anestesiologista deve estar preparado para possíveis alterações. O objetivo deste artigo é relatar a conduta anestésica adotada em uma paciente portadora desta síndrome. RELATO DO CASO: Paciente do sexo feminino ...

Abdome agudo obstrutivo pela veia porta - relato de caso

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Celeste Gomes Sardinha Oshiro

2016-10-01

Full Text Available Introdução: A veia porta pré-duodenal é uma anomalia congênita rara, sintomática em apenas 50% dos casos, sendo que seu diagnóstico é feito por laparotomia exploradora. O tratamento de escolha é cirúrgico, com bom prognóstico. Objetivo: Relatar um caso de Abdome Agudo Obstrutivo por Veia Porta Pré Doudenal (VPPD no período neonatal no Conjunto Hospitalar de Sorocaba. Metodologia: Descrição do referido caso e revisão de literatura. Relato de Caso: Recém-nascido de F.S.S., feminino, de parto normal em 30/05/2016, cuja mãe com 21 anos, apresentou durante pré-natal Diabetes Mellitus Gestacional e polihidrâmnio; negou consanguinidade, vícios e infecções. Ao nascimento, idade gestacional 38 1/7 semanas, peso 2865g, comprimento 47 cm, Apgar 9/9. Durante rotinas de sala de parto, à aspiração gástrica, saída de 55 ml de líquido claro com grumos (LCCG. No 4o dia de vida, episódios de vômito com sangue e distensão abdominal. No 11° dia de vida, realizada Laparotomia Exploradora que identificou dilatação gástrica e duodenal, principalmente na 3° porção, onde passa anteriormente a Veia Porta, comprimindo parcialmente a borda antimesentérica duodenal, o que comprometia seu esvaziamento. Realizada anastomose duodeno-jejunal. Recebeu alimentação parenteral por 15 dias. RN apresentou infecções fúngica e bacteriana, tratadas durante internação. Recebe alta com 46 dias de vida em aleitamento materno e boa recuperação clínica. Conclusão: Existem poucos relatos sobre a formação anômala da veia porta e suas consequências. O diagnóstico pré-natal ou pré-operatório de VPPD raramente é feito. Boa evolução pós correção cirúrgica.

Insuficiência respiratória aguda durante anestesia pediátrica: atelectasia e pneumotórax hipertensivo: relato de caso

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Joel Massari Rezende

2012-02-01

Full Text Available JUSTIFICATIVA E OBJETIVOS: A tarefa primordial do anestesiologista é garantir a adequada oxigenação do paciente. O objetivo deste relato é des crever o diagnóstico e conduta em um caso de insuficiência respiratória aguda durante anestesia, com finalidade didática. RELATO DO CASO: Criança de três anos submetida à anestesia para cirurgia urológica apresentou insuficiência respiratória por obstruções de brônquios por secreção, evoluindo com atelectasia e pneumotórax hipertensivo. Apresentamos o desenrolar do caso, e os meios de tratamento aplicado, com destaque para a urgência e técnica da drenagem no pneumotórax hipertensivo. CONCLUSÕES: A atenção do anestesiologista para o diagnóstico precoce de complicações respiratórias e o conhecimento das medidas prioritárias em cada momento pode evitar efeitos adversos graves

Standardization of the experimental autoimmune myasthenia gravis (EAMG) model by immunization of rats with Torpedo californica acetylcholine receptors — Recommendations for methods and experimental designs

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Losen, Mario; Martinez-Martinez, Pilar; Molenaar, Peter C.; Lazaridis, Konstantinos; Tzartos, Socrates; Brenner, Talma; Duan, Rui-Sheng; Luo, Jie; Lindstrom, Jon; Kusner, Linda

2015-01-01

Myasthenia gravis (MG) with antibodies against the acetylcholine receptor (AChR) is characterized by a chronic, fatigable weakness of voluntary muscles. The production of autoantibodies involves the dysregulation of T cells which provide the environment for the development of autoreactive B cells. The symptoms are caused by destruction of the postsynaptic membrane and degradation of the AChR by IgG autoantibodies, predominantly of the G1 and G3 subclasses. Active immunization of animals with AChR from mammalian muscles, AChR from Torpedo or Electrophorus electric organs, and recombinant or synthetic AChR fragments generates a chronic model of MG, termed experimental autoimmune myasthenia gravis (EAMG). This model covers cellular mechanisms involved in the immune response against the AChR, e.g. antigen presentation, T cell-help and regulation, B cell selection and differentiation into plasma cells. Our aim is to define standard operation procedures and recommendations for the rat EAMG model using purified AChR from the Torpedo californica electric organ, in order to facilitate more rapid translation of preclinical proof of concept or efficacy studies into clinical trials and, ultimately, clinical practice. PMID:25796590

A case of syndrome involving the inappropriate secretion of antidiuretic hormone developed during radiation therapy in a patient with invasive thymoma complicated with myasthenia gravis

International Nuclear Information System (INIS)

Chikuie, Naoki; Ishida, Simon; Sato, Tomohiko; Furutama, Daisuke; Sugino, Shyouichi; Kimura, Humihiro; Hanafusa, Toshiaki

2007-01-01

We present the case of a 56-year-old male with a syndrome involving the inappropriate secretion of antidiuretic hormone (SIADH), which developed during radiation therapy for invasive thymoma, complicated with myasthenia gravis (MG). Chest computed tomography revealed a huge mediastinal mass lesion spreading to the pulmonary artery, vena cava and pericardium. He was diagnosed with invasive thymoma, based on the pathological findings of a mediastinal tumor biopsy under computed tomography guidance. He received outpatient radiotherapy for the invasive thymoma, and two weeks after the initiation of radiation at a dose of 22 Gy, was admitted to our hospital because of hypercapnea due to weakness of the diaphragm and disturbance of consciousness. Laboratory examinations of the patient showed hyponatremia, plasma hypoosmolarity in the presence of concentrated urine and inappropriately increased concentration of the plasma antidiuretic hormone. He was also diagnosed as having myasthenia gravis, based on the existence of an anti-acetylcholine receptor antibody. The SIADH was treated by fluid restriction and sodium chloride, and MG was treated with plasma exchange and prednisolone. He recovered from respiratory failure, and his hyponatremia was improved. To our knowledge, this is a rare description of an invasive thymoma associated with SIADH. (author)

Immunization with Recombinantly Expressed LRP4 Induces Experimental Autoimmune Myasthenia Gravis in C57BL/6 Mice.

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Ulusoy, Canan; Çavuş, Filiz; Yılmaz, Vuslat; Tüzün, Erdem

2017-07-01

Myasthenia gravis (MG) is an autoimmune disease of the neuromuscular junction (NMJ), characterized with muscle weakness. While MG develops due to acetylcholine receptor (AChR) antibodies in most patients, antibodies to muscle-specific receptor tyrosine kinase (MuSK) or low-density lipoprotein receptor-related protein 4 (LRP4) may also be identified. Experimental autoimmune myasthenia gravis (EAMG) has been previously induced by both LRP4 immunization and passive transfer of LRP4 antibodies. Our aim was to confirm previous results and to test the pathogenic effects of LRP4 immunization in a commonly used mouse strain C57BL/6 (B6) using a recombinantly expressed human LRP4 protein. B6 mice were immunized with human LRP4 in CFA, Torpedo Californica AChR in CFA or only CFA. Clinical and pathogenic aspects of EAMG were compared among groups. LRP4- and AChR-immunized mice showed comparable EAMG clinical severity. LRP4-immunized mice displayed serum antibodies to LRP4 and NMJ IgG and complement factor C3 deposits. IgG2 was the dominant anti-LRP4 isotype. Cultured lymph node cells of LRP4- and AChR-immunized mice gave identical pro-inflammatory cytokine (IL-6, IFN-γ and IL-17) responses to LRP4 and AChR stimulation, respectively. Our results confirm the EAMG-inducing action of LRP4 immunization and identify B6 as a LRP4-EAMG-susceptible mouse strain. Demonstration of complement fixing anti-LRP4 antibodies in sera and complement/IgG deposits at the NMJ of LRP4-immunized mice indicates complement activation as a putative pathogenic mechanism. We have thus developed a practical LRP4-induced EAMG model using a non-conformational protein and a widely available mouse strain for future investigation of LRP4-related MG.

Psicologia e Práticas Restaurativas na Socioeducação: Relato de Experiência

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Iara da Silva Ferrão

Full Text Available Resumo Este artigo tem como objetivo apresentar um relato de experiência de profissional da área da Psicologia, dentro de uma Unidade de Internação de adolescentes em conflito com a lei. Ainda pretendeu-se relacionar práticas restaurativas com possibilidade de experiência exitosa ao sistema socioeducativo. O relato tem como cenário uma unidade de Atendimento Socioeducativo de uma cidade no interior do estado do Rio Grande do Sul. Os dados obtidos são frutos da experiência profissional da autora e fazem parte de um estudo maior intitulado “Um Estudo dos Fatores de Risco e de Proteção em Jovens que Cumprem Medida Socioeducativa”. Os resultados mostraram que as práticas restaurativas realizadas nesta instituição foram apenas os círculos de compromisso. O tema da Justiça Restaurativa (JR, por ser novo e por não ter sido originalmente um modelo criado no cenário brasileiro, ainda é difícil de avaliar possíveis resultados e impactos. As experiências apresentadas sugerem que há possibilidade da JR ser uma experiência exitosa para o Sistema Socioeducativo. Indica-se a necessidade de realização de mais pesquisas com relação a essa temática.

A bricolage na pesquisa em educação em enfermagem: Relato de experiência

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Leonara Raddai Gunther de Campos

2016-01-01

Full Text Available RESUMO Introdução: Este artigo apresenta um relato de experiência sobre a utilização da bricolage em uma pesquisa educacional em enfermagem. Objetivo: Relatar a experiência do uso da bricolage. Métodos: Primeiramente, foi realizado um levantamento bibliográfico sobre o termo e sua utilização em meio científico, em seguida, as autoras apresentam a experiência da aplicação do método em uma pesquisa de Mestrado em Enfermagem. A bricolage possibilitou a combinação de estratégias para a construção dos dados, como a observação participante, entrevista semiestruturada, análise documental e gravação em áudio das sessões tutoriais. A análise dos dados foi uma etapa bem peculiar e criativa, onde as autoras, amparadas pela bricolage criaram seu próprio processo de análise. Conclusões: Dentre os principais resultados são ressaltados as vantagens proporcionadas pela utilização da bricolage, como a captação dos aspectos multilógicos dos fenômenos e desafios. Implicações para a prática: Este relato contribui com novas opções metodológicas para estudos em enfermagem.

Diurnal and day-to-day variation of isometric muscle strength in myasthenia gravis.

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Vinge, Lotte; Jakobsen, Johannes; Pedersen, Asger Roer; Andersen, Henning

2016-01-01

In patients with myasthenia gravis (MG), muscle strength is expected to decrease gradually during the day due to physical activities. Isometric muscle strength at the shoulder, knee, and ankle was determined in 10 MG patients (MGFA class II-IV) who were receiving usual medical treatment and in 10 control subjects. To determine diurnal and day-to-day variation, muscle strength was measured 4 times during day 1 and once at day 2. Knee extension strength decreased during the day in both patients and controls. Neither diurnal nor day-to-day variation of muscle strength was higher in patients compared with controls. Patients with mild to moderate MG did not have increased variation of isometric muscle strength during the day or from day-to-day compared with controls. This suggests that isometric muscle performance can be determined with high reproducibility in similar groups of MG patients without regard to time of day. © 2015 Wiley Periodicals, Inc.

The Role of Osteopontin and Its Gene on Glucocorticoid Response in Myasthenia Gravis

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Yanchen Xie

2017-05-01

Full Text Available Biomarkers that assess treatment response for patients with the autoimmune disorder, myasthenia gravis (MG, have not been evaluated to a significant extent. We hypothesized the pro-inflammatory cytokine, osteopontin (OPN, may be associated with variability of response to glucocorticoids (GCs in patients with MG. A cohort of 250 MG patients treated with standardized protocol of GCs was recruited, and plasma OPN and polymorphisms of its gene, secreted phosphoprotein 1 (SPP1, were evaluated. Mean OPN levels were higher in patients compared to healthy controls. Carriers of rs11728697*T allele (allele definition: one of two or more alternative forms of a gene were more frequent in the poorly GC responsive group compared to the GC responsive group indicating an association of rs11728697*T allele with GC non-responsiveness. One risk haplotype (AGTACT was identified associated with GC non-responsiveness compared with GC responsive MG group. Genetic variations of SPP1 were found associated with the response to GC among MG patients.

The usefulness of MRI for detection of the thymus gland in myasthenia gravis

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Hokezu, Youichi; Kaseda, Syun; Arimura, Kimiyoshi; Osame, Mitsuhiro; Baba, Kuniaki (Kagoshima Univ. (Japan). Faculty of Medicine); Ohkubo, Koichi; Hagiwara, Hiroshi

1989-08-01

Seven patients with myasthenia gravis (MG) were examined to find thymus or thymoma employing chest radiographs, computed tomography (CT), pneumomediastinography (PMG), computed tomography after pneumomediastinography (PMG-CT) and magnetic resonance imaging (MRI). X-ray CT examination could reveal thymus only in half out of 6 cases scanned. On the other hand, MRI confirmed thymus or thymoma in 6 out of 7 patients. PMG and PMG-CT confirmed thymus or thymoma clearly in all of the 4 cases studied. PMG and PMG-CT examinations revealed thymus or thymoma more clearly than MRI. MRI is, however, an examination causing no pain to the patients and also more superior to X-ray CT in distinguishing between a thymus and mediastinal fat or vascular structure. In addition, MRI could reveal even capsules in thymoma which were never revealed by X-ray CT. We concluded that MRI could be an alternative method to CT and PMG in detection of thymus or thymoma in MG. (author).

The usefulness of MRI for detection of the thymus gland in myasthenia gravis

International Nuclear Information System (INIS)

Hokezu, Youichi; Kaseda, Syun; Arimura, Kimiyoshi; Osame, Mitsuhiro; Baba, Kuniaki; Ohkubo, Koichi; Hagiwara, Hiroshi.

1989-01-01

Seven patients with myasthenia gravis (MG) were examined to find thymus or thymoma employing chest radiographs, computed tomography (CT), pneumomediastinography (PMG), computed tomography after pneumomediastinography (PMG-CT) and magnetic resonance imaging (MRI). X-ray CT examination could reveal thymus only in half out of 6 cases scanned. On the other hand, MRI confirmed thymus or thymoma in 6 out of 7 patients. PMG and PMG-CT confirmed thymus or thymoma clearly in all of the 4 cases studied. PMG and PMG-CT examinations revealed thymus or thymoma more clearly than MRI. MRI is, however, an examination causing no pain to the patients and also more superior to X-ray CT in distinguishing between a thymus and mediastinal fat or vascular structure. In addition, MRI could reveal even capsules in thymoma which were never revealed by X-ray CT. We concluded that MRI could be an alternative method to CT and PMG in detection of thymus or thymoma in MG. (author)

Current and emerging therapies for the treatment of myasthenia gravis

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Mantegazza, Renato; Bonanno, Silvia; Camera, Giorgia; Antozzi, Carlo

2011-01-01

Myasthenia gravis (MG) is an autoimmmune disease in which autoantibodies to different antigens of the neuromuscular junction cause the typical weakness and fatigability. Treatment includes anticholinesterase drugs, immunosuppression, immunomodulation, and thymectomy. The autoimmune response is maintained under control by corticosteroids frequently associated with immunosuppressive drugs, with improvement in the majority of patients. In case of acute exacerbations with bulbar symptoms or repeated relapses, modulation of autoantibody activity by plasmapheresis or intravenous immunoglobulins provides rapid improvement. Recently, techniques removing only circulating immunoglobulins have been developed for the chronic management of treatment-resistant patients. The rationale for thymectomy relies on the central role of the thymus. Despite the lack of controlled studies, thymectomy is recommended as an option to improve the clinical outcome or promote complete remission. New videothoracoscopic techniques have been developed to offer the maximal surgical approach with the minimal invasiveness and hence patient tolerability. The use of biological drugs such as anti-CD20 antibodies is still limited but promising. Studies performed in the animal model of MG demonstrated that several more selective or antigen-specific approaches, ranging from mucosal tolerization to inhibition of complement activity or cellular therapy, might be feasible. Investigation of the transfer of these therapeutic approaches to the human disease will be the challenge for the future. PMID:21552317

Sindrome peri-silviana bilateral não relacionada a malformações: relato de dois casos

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Grasel, Ralf Paulo; Carvalho Neto, Arnolfo; Bruck, Isac; Antoniuk, Sérgio A.

1996-01-01

In this case report we present the neuroimaging findings and clinical features of two patients with a bilateral perisylvian syndrome not related to malformations, but probably to ischemic etiology. Evaluations including history, general and neurologic examinations, electroencephalograms, and imaging data were reviewed as recent literature about the subject.Neste relato de caso, apresentamos os achados de neuroimagem e os aspectos clínicos de dois pacientes com síndrome peri-silviana bilateral...

Del metafísico al parapsicólogo: el estereotipo del chino en dos relatos modernistas

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Siwen Ning

2017-02-01

Full Text Available El presente trabajo examina los recursos narrativos y el tratamiento de dos personajes chinos que aparecen en dos relatos de la revista La Ilustración Española y Americana, publicados a finales del siglo xix el primero, a principios del xx el segundo. Este estudio tiene como finalidad comprobar la evolución del estereotipo chino en la literatura española en el contexto del fin de siglo.

Exotismo e autenticidade: relatos de viajantes à procura de sexo Exoticism and authenticity: tales of travelers in search of sex

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Adriana Piscitelli

2002-01-01

Full Text Available A produção sócio-antropológica que trata do turismo discute a oposição entre o caráter de massa atribuído ao olhar dos turistas contemporâneos e o caráter individual vinculado à "viagem". Este texto dialoga com argumentos presentes nessa oposição, a partir da análise etnográfica de relatos de viajantes à procura de sexo que freqüentam pontos do circuito internacional de turismo sexual. Centrando-se em narrativas ficcionais/experienciais, Plateforme, romance de Michel Houellebecq, recentemente publicado, os relatos de viagem de Mattioli Ross e histórias contadas por viajantes à procura de sexo, no marco de uma pesquisa sobre turismo sexual internacional no Nordeste do Brasil, o texto estabelece esse diálogo considerando as noções centrais presentes nesse conjunto de relatos atravessados por gênero: exotismo, sexualidade e autenticidade.The socio-anthropological production on tourism opposes the mass character imputed to the views of contemporary tourists to the individual character attributed to the "trip". This text establishes a dialogue with arguments present in that opposition, based on ethnographic analyses of tales by travelers in search of sex used to places in the international circuit of sexual tourism. Centering on fictional/experiential accounts, Plateforme, recently published novel by Michel Houellebecq, Mattioli Ross' travel accounts and tales told by travelers in search of sex, framed by a research on international sexual tourism in Brazil's Northeast, the text establishes such a dialogue considering the notions central to those accounts crisscrossed by gender: exoticism, sexuality, authenticity.

Co-construyendo historias: a la búsqueda de hechos luminosos en los relatos familiares sobre el consumo de drogas

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Ayme Pacheco Trejo

2008-01-01

Full Text Available La presente investigación se ubica bajo el marco teórico del construccionismo social. Los relatos familiares sobre el consumo de drogas se exploraron con un enfoque comprensivo-interpretativo, ya que se parte del supuesto de que las personas se relacionan y construyen sus realidades a través de la interacción dialógica entre los participantes, donde se incluye al propio investigador. El propósito del presente estudio fue identificar los hechos que iluminan las contradicciones y potenciar la emergencia de historias alternativas, por medio de puntuar aspectos poco atendidos de la experiencia, y cuestionar prácticas y discursos sociales hegemónicos. Para tal fin se presenta el caso de una familia con un miembro consumidor. Los espacios conversacionales fueron tres y se utilizo la entrevista semi-estructurada con base en la propuesta narrativa de Michael White (2002a, 2002b. Los resultados permiten observar el quebranto del relato dominante; un relato plagado de problemas, de etiquetas que devienen de una relación de saber-poder que se articulan con una red de controles y normas sociales. Un aprendizaje cultural que se materializa en formas de consumo, formas de relacionarse, formas de enfermar, curarse, y morir; esto es, un aprendizaje que penetra la conciencia y la relación con el consumidor, sin que necesariamente se perciba como un mecanismo de control negativo. Se concluye sobre la importancia de una reconstrucción colectiva, que representa una visión de la realidad como producto de una acción reflexiva de los participantes, a través de saberes sociales compartidos, que dan cuenta de la riqueza y complejidad de las narraciones familiares sobre el consumo de drogas.

Archivo y relato en el Río de la Plata Colonial. El difícil discurso de la violencia

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Loreley El Jaber

2013-11-01

Full Text Available La propuesta en esta ocasión es la de abordar la relación entre archivo y relato en el Río de la Plata durante el siglo XVI y, para esto, se analizará una información levantada por Cabeza de Vaca en su defensa, fechada en 1544, porque en ella se da lugar a la aparición de un conjunto de voces de españoles que denuncian los atropellos, gravámenes y “vejaciones” sufridas por parte de sus pares en procura de la cobranza del quinto. Estos discursos, no reproducidos por otros textos impresos durante el mencionado siglo, plantean una problemática compleja: la relación entre ley, violencia y espacio conquistado; pero también, otra de distinto orden: los alcances y efectos de discursividades como estas, devenires de un relato cruel detenido en el tiempo del archivo judicial. El presente trabajo busca abordar ambas problemáticas así como darle un lugar a esta serie de voces olvidadas.

Rare association of thymoma, myasthenia gravis and sarcoidosis : a case report

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Kurukumbi Mohankumar

2008-07-01

Full Text Available Abstract Introduction The association of thymoma with myasthenia gravis (MG is well known. Thymoma with sarcoidosis however, is very rare. We presented an interesting case with coexisting thymoma, MG and sarcoidosis. Case presentation A 59-year-old female patient with a history of sarcoidosis was admitted to the hospital with a one-day history of sudden onset of right-sided partial ptosis and diplopia. Neurosarcoidosis with cranial nerve involvement was considered, but was ruled out by the clinical findings, and MG was confirmed by the positive tensilon test, electrophysiological findings and positive acetylcholine receptor binding antibodies. On further evaluation, a CT chest scan showed a left anterior mediastinal mass and bilateral lymphadenopathy. Post surgical diagnosis confirmed the thymoma and sarcoidosis in the lymph nodes. Conclusion When two or more diseases of undetermined origin are found together, several interesting questions are raised. It is important to first confirm the diagnoses individually. Immunologic mechanisms triggering the occurrence of these diagnoses together, are difficult to address. Although the coexistence of thymoma, MG and sarcoidosis may be coincidental, it is noteworthy to report this case because of the multiple interesting features observed as well as the rarity of occurrence.

Evaluation of repetitive stimulation test (RST in 30 patients with Myasthenia Gravis, who were previously confirmed by clinical sign and tensilon test 1996-99

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"Ghabaee M

2001-07-01

Full Text Available est (RST is the most commonly used electrodiagnostic test to asses the defect of neuromuscular transmission, which is reported to be positive in the diffuse and restricted ocular forms 60-95% and 14-50%, respectively. In a cross-sectional study, to determine the efficacy of repetitive stimulation test in myasthenia gravis, we evaluated the results in 30 cases who were hospitalized in Imam Khomeini Hospital during 1996-1999. Patients were first selected clinically and then confirmed by Tensilon test.Various clinical types including generalized and restricted ocular forms with different severity and duration were entered in this study. Considering the fact that the positiveness of the test is enhanced by assessment of more muscle groups, we evaluated decremental response in the facial, proximal and distal muscles of limbs. 90% of patients had the generalized form of the disease, whereas ocular myasthenia gravis was seen only in 10% of the cases. 74% of females and 73% of males showed positive response (overall: 73.3%. No significant association was found between the positive response, and age and sex. Peaks of incidences of the disease for the males were in fourth and sixth decades and for the females in thired decades

Infecção cutânea rara por Acinetobacter baumannii em imunocompetente: relato de um caso

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Cirino,Pablo Vitoriano; Guimarães,Newton Sales; Follador,Ivonise

2008-01-01

O Acinetobacter baumanni é patógeno oportunista antigamente considerado de baixa virulência. Atualmente está envolvido em processos infecciosos que acometem pacientes imunocomprometidos,grandes queimados e pacientes em unidades de terapia intensiva que fazem uso de ventilação mecânica. Esse relato de caso chama atenção para infecção cutânea rara por essa bactéria em paciente imunocompetente.

Plasmocitoma extramedular em bulbo peniano de cão: relato de caso

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T.G. Gorenstein

2016-04-01

Full Text Available O objetivo deste relato de caso é descrever a ocorrência de plasmocitoma em bulbo peniano de um cão, classificado como uma doença extramedular não cutânea de localização rara e casuística inédita. Um cão, sem raça definida, com sete anos de idade e pesando 15kg, não castrado, apresentou histórico clínico de anorexia, vômitos, anúria e constipação. Ao exame específico da genitália externa, foi encontrada uma massa em bulbo peniano durante a inspeção do prepúcio, aderida à pele e encapsulada, extremamente firme e arredondada, medindo cerca de 6cm de diâmetro. Por meio da ultrassonografia dessa estrutura, foi observado aumento do volume regional com ecotextura heterogênea e ecogenicidade mista, além de neovascularização tecidual ao Doppler colorido. Foi realizada biópsia da massa, sendo verificada a presença de neoplasia de células redondas. A caracterização do tumor foi realizada pela imuno-histoquímica, e as células neoplásicas foram imunoexpressas para CD79a e MUM1, indicando o diagnóstico de plasmocitoma extramedular. Embora os tumores penianos em cães sejam os predominantemente venéreos transmissíveis (TVT, e os plasmocitomas sejam neoplasias raras nessa localização, este relato de caso fornece com ineditismo a ocorrência de plasmocitoma extramedular em bulbo peniano de cão, condição ainda não descrita em veterinária.

Ruy González de Clavijo. La embajada a Tamorlán. Relato del viaje hasta Samarcanda y regreso (1403-1406

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López Estrada, Francisco

2005-04-01

Full Text Available El presente relato es una narración, puesta en boca de los embajadores enviados por Enrique III, rey de Castilla y León, que cuenta el viaje que hicieron hasta Samarcanda, capital del Imperio logrado por Tamorlán al cabo de su vida. Este viaje fue en parte por mar, hasta Trebisonda; luego, por tierra, hasta Samarcanda, y duró cerca de tres años. Los embajadores llegaron a su destino, pero poco después murió Tamorlán, y los enviados del rey castellano regresaron a Alcalá de Henares, donde informaron acerca de su cometido. La narración es uno de los primeros viajes que se cuenta como tal en la literatura española, y representa una obra lograda, situada en los comienzos del siglo XV, en lengua castellana; en lo histórico es un relato verídico del hecho acontecido.…

LA COSA Y LA PALABRA. RELATO Y EMOCIONALIDAD EN UN MUSEO POLICIAL / The thing and the words. Story and emotion in a police museum

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Mariana Sirimarco

2014-11-01

Full Text Available Es a todas luces claro que un museo brinda un relato: vuelve lo material en patrón cultural significativo. Idéntico ejercicio le cabe al Museo de la Policía Federal Argentina. Los artefactos que en él se despliegan ponen en escena discursos, vivencias y valorizaciones a partir de los cuales pensarse como institución. Este trabajo pretende bucear en la construcción de algunos de estos relatos, entendiéndolos como narrativas que no sólo involucran un universo de sentidos políticos y sociales, sino un universo ético y moral. Un relato no se conforma por la descripción aséptica de un evento, sino por la conversión de un acontecimiento en un mensaje. Esto es, por una narrativización que porta carga emocional. Desarmar estos relatos en su clave emotiva supone, en el contexto de un museo, prestar atención a su carácter de registros pluri-modales, donde la semantización de un evento interpela diversos modos comunicativos. ¿Qué elementos se seleccionan para comportar sentimientos significativos? ¿Cómo se vinculan, estos distintos registros, en la producción de la emotividad? Y sobre todo: ¿cómo se construye lo emotivo desde la materialidad de un artefacto? ¿Cómo logra conmover un objeto?   Palabras clave: museo policial, relato, emocionalidad, objetos, palabras    Abstract It is clear that a museum offers stories: turns what it is material into a cultural significant pattern. The same exercise carries out the Museum of Argentine Federal Police. The artifacts there stage discourses, experiences and values that allow police agency to think about itself as institution. This paper intends to analyze the construction of some of these stories, understanding them as narratives that imply not only political and social senses, but also ethic and moral ones. A story is not shaped by the aseptic description of an event, but by the transformation of it into a message. That is to say, by a narrative operation that carries emotional charge

Avaliação funcional de relatos de disfunção erétil.

OpenAIRE

Domingos, Vania Gomes Machado

2014-01-01

O presente estudo teve como objetivo analisar funcionalmente o comportamento verbal de uma pessoa diagnosticado com disfunção erétil. O participante, do sexo masculino de 37anos é casado. Para o controle dos procedimentos foram empregados dois delineamentos experimentais: o de múltiplos elementos e o de tratamentos alternados. Para avaliar os antecedentes e consequentes dos relatos de disfunção erétil, foi usado processo de avaliação funcional que incluiu (1) observação indiret...

Using Herbs and Spices/Preparing Sauces and Gravies. Learning Activity Pack and Instructor's Guide 5.11. Commercial Foods and Culinary Arts Competency-Based Series. Section 5: Basic Food Preparation.

Science.gov (United States)

Florida State Univ., Tallahassee. Center for Studies in Vocational Education.

This document consists of a learning activity packet (LAP) for the student and an instructor's guide for the teacher. The LAP is intended to acquaint occupational home economics students with herbs and spices and the selection and preparation of sauces and gravies. Illustrated information sheets and learning activities are provided in these areas:…

RELATO DE CASO: RESPOSTA COMPLETA COM QUIMIOTERAPIA DE SEGUNDA LINHA EM CÂNCER DE BEXIGA ESTADIO IV

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Carla Simone Moreira de FREITAS

2018-04-01

Full Text Available O câncer de bexiga é a neoplasia mais comum do sistema urinário, sendo raro ocorrer casos de resposta completa a quimioterapia em tumores avançados, quando não realizado cirurgia com intuito curativo. Objetivo: relatar o caso de um paciente que apresentou resposta completa da neoplasia vesical em estadio IV após quimioterapia de 2ª linha. Metodologia: relato de caso. 

Síndrome de Cogan: relato de caso Cogan's Syndrome: case report

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Fabricio Witzel de Medeiros

2005-12-01

Full Text Available Ceratite intersticial não luética, surdez e vertigem caracterizam a síndrome de Cogan. Os achados oculares mais comuns no início da síndrome são opacidades corneanas numulares periféricas bilaterais. O tratamento desta rara doença utiliza imunossupressão sistêmica com agentes citotóxicos e corticosteróides. O relato de caso apresenta uma paciente com a evolução da forma clássica da síndrome de Cogan.Non-luetic interstitial keratitis, deafness and vertigo characterize Cogan's syndrome. The most common ocular findings in early Cogan's syndrome are bilateral, peripheral, subepithelial numular corneal opacities. The report presents a patient with the evolution of the classic form of Cogan's syndrome.

Cistopexia videoassistida em cadela com cistocele após prolapso uterino: relato de caso

OpenAIRE

Krebs, T.; Brun, M.V.; Linhares, M.T.; Dalmolin, F.; Pohl, V.H.; Feranti, J.P.S.

2015-01-01

O prolapso de vesícula urinária associado ao prolapso uterino é condição rara em cães e gatos. O tratamento preconizado para essa afecção é o reposicionamento anatômico das estruturas afetadas. No presente relato é descrito o manejo por meio de ovariossalpingo-histerectomia por celiotomia e, após dois dias, cistopexia videoassistida. Essa condição, conforme o conhecimento dos autores, não havia sido previamente descrita. A operação apresentou-se como possibilidade viável de tratamento, sem co...

Computed tomography of the anterior mediastinum in myasthemia gravis: a radiologic-pathologic correlative study

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Fon, G.T.; Bein, M.E.; Mancuso, A.A.; Keesey, J.C.; Lupetin, A.R.; Wong, W.S.

1982-01-01

Chest radiographs and computed tomographic (CT) scans of the mediastinum were correlated with pathologic findings of the thymus following thymectomy in 57 patients with myasthenia gravis. Based on the patient's age and the overall morphology of the anterior mediastinum, CT scans were assigned one of four grades in an attempt to predict thymus pathologic findings. Using this grading, 14 of 16 cases of thymoma were suspected or definitely diagnosed. One of the two cases not diagnosed on CT was a microscopic tumor. There were no false-positive diagnoses in 11 cases graded as definitely thymoma. We conclude that thymoma can be sensitively diagnosed in patients older than 40 years of age. However, thymoma cannot be predicted with a high level of confidence in patients younger than 40 because of the difficulty in differentiating normal thymus or hyperplasia from thymoma. Recommendations for the use of CT in the preoperative evaluation of myasthenic patients are presented.

Computed tomography of the anterior mediastinum in myasthemia gravis: a radiologic-pathologic correlative study

International Nuclear Information System (INIS)

Fon, G.T.; Bein, M.E.; Mancuso, A.A.; Keesey, J.C.; Lupetin, A.R.; Wong, W.S.

1982-01-01

Chest radiographs and computed tomographic (CT) scans of the mediastinum were correlated with pathologic findings of the thymus following thymectomy in 57 patients with myasthenia gravis. Based on the patient's age and the overall morphology of the anterior mediastinum, CT scans were assigned one of four grades in an attempt to predict thymus pathologic findings. Using this grading, 14 of 16 cases of thymoma were suspected or definitely diagnosed. One of the two cases not diagnosed on CT was a microscopic tumor. There were no false-positive diagnoses in 11 cases graded as definitely thymoma. We conclude that thymoma can be sensitively diagnosed in patients older than 40 years of age. However, thymoma cannot be predicted with a high level of confidence in patients younger than 40 because of the difficulty in differentiating normal thymus or hyperplasia from thymoma. Recommendations for the use of CT in the preoperative evaluation of myasthenic patients are presented

Effect of low-dose irradiation on growth of and toxin production by Staphylococcus aureus and Bacillus cereus in roast beef and gravy.

Science.gov (United States)

Grant, I R; Nixon, C R; Patterson, M F

1993-03-01

The effect of irradiation (2 kGy) on growth of and toxin production by Staphylococcus aureus and Bacillus cereus in roast beef and gravy during storage at abuse temperatures (15 and 22 degrees C) was assessed by inoculation studies. Irradiation resulted in a 3-4 log10 reduction in numbers of both pathogens. Whenever B. cereus and S. aureus numbers reached 10(6) and 10(7) cfu/g, respectively, during storage their toxins were detectable. As the time taken to attain these levels was longer in irradiated than in unirradiated samples, toxin production by both pathogens was delayed by irradiation. When samples initially containing low levels (10(2)/g) of S. aureus were irradiated no toxin was produced during subsequent storage at 15 or 22 degrees C. Diarrhoeal toxin produced by B. cereus was detected after 2 days at 22 degrees C, but not at 15 degrees C, in samples containing 10(2) cells/g prior to irradiation. When higher numbers (10(6)/g) of either pathogen were present prior to irradiation, toxins were produced by both pathogens at 22 degrees C, but not at 15 degrees C. Microbial competition had an effect on the growth of B. cereus and S. aureus after irradiation when a low initial inoculum was applied. However, when a higher inoculum was used the pathogens outnumbered their competitors and competition effects were less important. It was concluded that low-dose irradiation would improve the microbiological safety of roast beef and gravy.

O que se faz e o que se diz: auto-relatos emitidos por terapeutas comportamentais

OpenAIRE

Oliveira, Wilton de

2008-01-01

O presente trabalho tem o objetivo de verificar as relações entre os auto-relatos emitidos por terapeutas comportamentais sobre seus próprios comportamentos na interação com seus clientes e os respectivos comportamentos que de fato ocorreram. Como sujeitos da pesquisa foram selecionados três terapeutas analítico-comportamentais com tempo de experiência distintos (seis meses Ta, cinco anos Tb, e oito anos Tc). Cada terapeuta atendeu três clientes (uma sessão cada), de modo a perfazer um total ...