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Sample records for con miastenia gravis

  1. Miastenia gravis juvenil Juvenile myasthenia gravis

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    Oscar Papazian; Israel Alfonso; Nayle Araguez

    2009-01-01

    La miastenia gravis juvenil (MGJ) es un trastorno crónico auto inmune en el cual existen anticuerpos séricos que al unirse a los receptores de acetilcolin nicotínicos de la membrana muscular de la placa motora alteran la transmisión neuromuscular. El resultado es fatiga muscular precoz con progresión a la parálisis durante estados de contracción muscular iterativos (movimientos) o sostenidos (posturas) y más raramente parálisis permanente durante el reposo. Los músculos inervados por los nerv...

  2. Miastenia gravis diagnostic in dogs

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    Adriana Patricia Suraniti

    2010-12-01

    Full Text Available Miastenia Gravis is a neuromuscular disease caused by auto antibodies. Early Clinical and biochemical diagnosis and treatment is demanded in the assurementof quality and time of life in all dogs. In this study we describe the conventional diagnosis methods and therapy in 32 dogs with suspected myasthenia gravis and propose the administration of bromide of piridostigmin as another use full diagnosis method in dogs.

  3. Miastenia gravis juvenil Juvenile myasthenia gravis

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    Oscar Papazian

    2009-01-01

    Full Text Available La miastenia gravis juvenil (MGJ es un trastorno crónico auto inmune en el cual existen anticuerpos séricos que al unirse a los receptores de acetilcolin nicotínicos de la membrana muscular de la placa motora alteran la transmisión neuromuscular. El resultado es fatiga muscular precoz con progresión a la parálisis durante estados de contracción muscular iterativos (movimientos o sostenidos (posturas y más raramente parálisis permanente durante el reposo. Los músculos inervados por los nervios craneales, especialmente los extraoculares y elevadores de los párpados, tienen más tendencia a la debilidad muscular persistente que los inervados por otros pares craneales y las extremidades. Las formas clínicas de presentación son generalizadas, oculares y respiratorias. El diagnóstico se sospecha mediante la anamnesia, la fatiga anormal se comprueba mediante el examen físico y la estimulación eléctrica iterativa del nervio que inerva al músculo afectado pero no paralizado. Se corrobora mediante la administración de inhibidores de la acetilcolin esterasa (IACE que al aumentar la cantidad de acetilcolin en la hendidura sináptica, corrigen la fatiga o la debilidad muscular transitoriamente. Se hace el diagnóstico de certeza mediante la demostración sérica de anticuerpos contra los receptores de acetilcolin (ACRA. El tratamiento es a largo plazo sintomático con IACE y etiopatogénico con inmunosupresores, plasmaféresis, gamma globulina endovenosa y timectomía. El curso es crónico. La remisión espontánea o después de tratamiento sintomático o etiopatogénico ocurre entre 1-10 años respectivamente. La mortalidad es prácticamente nula aun durantes las crisis miastenias gracias a la educación de padres, pacientes y público en general sobre el tema, al desarrollo del sistema de respuesta rápida de auxilio domiciliario y las unidades de cuidados intensivos y el empleo de la ventilación asistida profiláctica, plasmaféresis y

  4. 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.

  5. Miastenia gravis familiar: registro de dois irmãos

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    Marcos R. G. de Freitas

    1985-03-01

    Full Text Available A forma familiar da miastenia gravis é condição relativamente rara, ocorrendo em cerca de 3,4% dos pacientes miastênicos. Os autores registram os casos de dois irmãos não-gêmeos, apresentando miastenia gravis com acometimento predominantemente ocular desde o nascimento. Um terceiro irmão faleceu na infância, provavelmente com a mesma afecção. Os pais eram consanguíneos. Estudo da incidência da forma familiar da miastenia e de seus aspectos clínicos, genéticos e terapêuticos complementa os registros.

  6. Síndrome de Usher asociado a miastenia gravis y esquizofrenia Syndrome of Usher associated to miastenia gravis and schizophrenia

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    Francisca Santisteban Aguilera

    2009-04-01

    Full Text Available 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 alteraciones clínicas presentes en este enfermo hacen sospechar la asociación de un síndrome de Usher, con miastenia gravis y una esquizofrenia.The case of a patient attended at "Saturnino Lora" Provincial Hospital and at the Pigmentary Retinosis Center in Santiago de Cuba with audiology characteristics, moderate neurosensorial hypoacusia, ophthalmologic affections, generalized fatigue, dysphagia, asymmetric bilateral palpebral ptosis with prevalence in the left side, affection of the oblique muscle and of the internal rectum muscle and absence of the gag reflex, besides delusion and hallucinations is presented. The clinical changes in this patient make the suspect of an association of the Usher`s syndrome, with miastenia gravis and a schizophrenia.

  7. Anestesia peridural torácica para cirurgia plástica de mama em paciente portadora de miastenia gravis: relato de caso Anestesia peridural torácica para cirugía plástica de mama en paciente portadora de miastenia gravis: relato de caso Thoracic epidural anesthesia for mammaplasty in myasthenia gravis patient: case report

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    Fabiano Timbó Barbosa

    2005-06-01

    Full Text Available JUSTIFICATIVA E OBJETIVOS: A miastenia gravis é uma doença crônica, auto-imune, caracterizada pela fraqueza da musculatura esquelética em decorrência da diminuição dos receptores de acetilcolina na junção neuromuscular. O objetivo deste relato é mostrar um caso de paciente com miastenia gravis submetida a anestesia peridural torácica para cirurgia plástica de mama. RELATO DO CASO: Paciente com 51 anos, portadora de miastenia gravis foi submetida a anestesia peridural torácica com bupivacaína e fentanil. Não houve sinais de depressão respiratória. A paciente recebeu alta hospitalar após 36 horas. CONCLUSÕES: O presente caso sugere como conduta anestésica para o paciente portador de miastenia gravis a anestesia peridural como técnica única, sem a obrigatoriedade de intubação orotraqueal.JUSTIFICATIVA Y OBJETIVOS: La miastenia gravis es una enfermedad crónica, auto-inmune, caracterizada por la debilidad de la musculatura esquelética resultante de la disminución de los receptores de acetilcolina en la unión neuromuscular. El objetivo de este relato es mostrar el caso de una paciente con miastenia gravis sometida a anestesia peridural torácica para una cirugía plástica de mama. RELATO DEL CASO: Paciente del sexo femenino, 51 años, portadora de miastenia gravis fue sometida a anestesia peridural torácica con bupivacaína y fentanil. No hubo señales de depresión respiratoria. La paciente recibió alta hospitalaria después de 36 horas. CONCLUSIONES: Este actual caso sugiere como conducta anestésica para el paciente portador de miastenia gravis la anestesia peridural como única técnica, sin la obligatoriedad de intubación orotraqueal.BACKGROUND AND OBJECTIVES: Myasthenia gravis is a chronic autoimmune disease characterized by skeletal muscles weakness promoted by decreased acetylcholine receptors in the neuromuscular junction. This report aimed at describing a case of myasthenia gravis patient submitted to thoracic

  8. Miastenia gravis congênita e oftalmoplegia externa

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    Moreira Ana Tereza Ramos

    2001-01-01

    Full Text Available A miastenia gravis é uma doença crônica, caracterizada por fatigabilidade anormal de músculos estriados, podendo acometer grupos musculares isolados ou tornar-se generalizada. Os autores descrevem um caso de miastenia gravis congênita generalizada e oftalmoplegia parcial em um paciente de 10 anos de idade, portador de sintomas sistêmicos motores e de ausência na adução, abdução e elevação em ambos os olhos e com ptose palpebral bilateral, sendo reduzida à função de infraversão. O paciente foi diagnosticado aos dois anos e seis meses, sendo iniciado tratamento com piridostigmina em doses subterapêuticas, sem alteração importante no quadro clínico. Aos quatro anos de idade, procurou esta instituição, sendo ajustada à dose da medicação, seguindo-se melhora significativa dos sintomas motores sistêmicos, melhora parcial da ptose palpebral e sem alteração na oftalmoplegia externa.

  9. Anestesia ambulatorial para radioterapia em paciente portador de miastenia gravis: relato de caso Anestesia ambulatorial para radioterapia en paciente portador de miastenia gravis: relato de caso Outpatient anesthesia for radiotherapy in a patient with myasthenia gravis: case report

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    Raquel Marcondes Bussolotti

    2006-08-01

    Full Text Available JUSTIFICATIVA E OBJETIVOS: A miastenia gravis (MG é doença auto-imune rara, caracterizada pela redução de receptores nicotínicos na junção neuromuscular, com incidência de 14/100.000. O objetivo deste relato foi descrever o caso de paciente portador de miastenia gravis submetido à anestesia geral balanceada, para tratamento radioterápico de carcinoma espinocelular de parótida, em regime ambulatorial. RELATO DO CASO: Paciente do sexo masculino, 87 anos, 87 kg, estado físico ASA III, com história de miastenia gravis; acidente vascular encefálico prévio (AVE; marca-passo por bloqueio atrioventricular total; hipertensão arterial sistêmica (HAS. Foram programadas sete sessões de radioterapia de 20 minutos para tratamento de tumor de parótida, sob anestesia geral. Na sala de radioterapia foi monitorizado com cardioscópio, monitor de pressão arterial não-invasiva, oxímetro de pulso, e submetido à anestesia geral com propofol e sevoflurano. Após a indução, foi mantido em ventilação espontânea com cânula de Guedel e cateter nasofaríngeo com O2 /sevoflurano, para acomodação da máscara imobilizadora. Na sala de recuperação pós-anestésica, não apresentou complicações. CONCLUSÕES: A escolha dos anestésicos e o acompanhamento clínico criterioso permitiram a boa evolução do paciente, com AVE prévio, cardiopata e idoso, submetido à anestesia geral balanceada para procedimento de aplicação de radioterapia, em regime ambulatorial.JUSTIFICATIVA Y OBJETIVOS: La Miastenia Gravis (MG es una enfermedad auto inmune rara, caracterizada por la reducción de receptores nicotínicos en la junción neuromuscular, con incidencia de 14/100.000. El objetivo de este relato fue el de describir el caso del paciente portador de miastenia gravis sometido a la anestesia general balanceada, para tratamiento radioterápico de carcinoma espinocelular de parótida en régimen ambulatorial. RELATO DEL CASO: Paciente del sexo masculino

  10. Miastenia gravis análise de 90 casos tratados com timectomia myasthenia gravis

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    Almeida Fábio Henrique Souza

    2000-01-01

    Full Text Available A Miastenia Gravis é uma doença auto-imune caracterizada por auto-anticorpos contra receptores nicotínicos de acetilcolina da placa neural. O tratamento clínico básico para as formas generalizadas é feito com drogas anticolenesterásicas, mas em algumas fases podem ser necessários outras modalidades de tratamento, tais como a corticoterapia e a plasmaferese. O tratamento cirúrgico, a timectomia, é uma forma de abordar a causa base da doença e tem resultados bastante encorajadores, visto que é atribuída ao timo a produção dos auto-anticorpos. Apresentamos neste trabalho o quadro clínico, os resultados cirúrgicos e as alteração anátomo-patológicas dos 90 casos estudados.

  11. Miastenia gravis infantil: relato de caso diagnosticado clinicamente

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    J. Rodrigo Lopes

    1985-09-01

    Full Text Available Registro de caso de miastenia infantil (forma congênita, em criança com 16 meses de idade. Ênfase ao diagnóstico diferencial das várias formas de miastenia na infância é dada durante a discussão do caso. O diagnóstico baseou-se na resposta imediata a teste com Neostigmina, seguido de terapêutica satisfatória com Mestinon.

  12. Lupus eritematoso sistêmico associado a miastenia gravis: relato de caso Systemic lupus erythematosus and myasthenia gravis: case report

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    MARCIO F. DE CARVALHO

    1998-03-01

    Full Text Available Os autores descrevem o caso de uma mulher branca de 24 anos de idade admitida com lupus eritematoso sistêmico (com 4 anos de evolução de doença e início recente de miastenia gravis. São discutidos os principais diagnósticos diferenciais para a fraqueza muscular e a fadiga apresentadas por esta paciente. Uma revisão de literatura abordando a associação de miastenia gravis e lupus eritematoso é feita, com ênfase às características clínicas desses pacientes e ao papel do timoma e timectomia no desenvolvimento de lupus eritematoso em pacientes previamente miastênicos.We report the case of a 24-year-old white woman admitted with a four year diagnosis of systemic lupus erythematosus and the recent onset of myasthenia gravis discussing the main differential diagnosis of weakness and fatigue in this patient. A review of literature approaching the association of myasthenia gravis and systemic lupus erythematosus is also done with emphasis on the clinical characteristics of these patients and the role of thymoma and thymectomy in the development of systemic lupus erythematosus in myasthenic patients.

  13. Familial myasthenia gravis: report of four cases Miastenia grave familial: registro de quatro casos

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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.Os autores registram dois pares de gêmeos com miastenia grave, pertencentes a duas famílias diferentes. Este é o único registro de miastenia familial durante os últimos 20 anos, num total de 145 pacientes examinados na Clínica Neurológica da FMUSP. Apesar do fato de a miastenia grave não ter características hereditárias, os aspectos peculiares dos quatro pacientes justificam o presente registro. Os dois pares de gêmeos nasceram de pais não miastênicos e sem consanguinidade. A doença iniciou-se no nascimento, evoluindo com ptose bilateral parcial da pálpebra superior precocemente em todos os pacientes. O curso da moléstia tem sido favorável. Não havia timoma.

  14. 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.

  15. 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

  16. Anestesia venosa total para timectomia em paciente com Miastenia Gravis: relato de caso

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    Rezer Gabrielle

    2003-01-01

    Full Text Available JUSTIFICATIVA E OBJETIVOS: Pacientes com doenças neuromusculares, como Miastenia Gravis, respondem de maneira anormal aos anestésicos, conforme a técnica e as drogas administradas. O objetivo deste relato é mostrar um caso de paciente portadora de Miastenia Gravis, submetida a timectomia sob anestesia venosa total com propofol e remifentanil. RELATO DO CASO: Paciente feminina, 52 anos, 72 kg, com história de Miastenia Gravis submetida a timectomia transesternal, sob anestesia venosa total, com o uso de propofol em infusão alvo controlada (3 g.ml-1 e remifentanil contínuo (0,3 µg.kg-1.min-1. Para a intubação traqueal foi utilizada succinilcolina (50 mg, sendo realizada sem dificuldade. Com a finalidade de realizar a analgesia pós-operatória, foram utilizados tramadol (50 mg, cetoprofeno (100 mg e dipirona (1 g, no per-operatório. Após a cirurgia, a infusão de propofol e remifentanil foi encerrada e, 15 minutos depois, a paciente foi extubada. A paciente apresentava-se eupnéica, acordada, sem dor, movimentando membros, com freqüência respiratória de 14 mpm e mantendo saturação de oxigênio de 97%. Permaneceu com cateter nasal de O2 a 2 L.min-1 na UTI intermediária, durante 36 horas, e recebeu alta hospitalar no 4º dia do pós-operatório. CONCLUSÕES: A anestesia venosa total, com agentes anestésicos de curta duração e sem metabólitos ativos, favoreceu a recuperação e a extubação precoce da paciente

  17. Teste do gelo no diagnóstico de miastenia gravis Ice pack test in the diagnosis of myasthenia gravis

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    Nilson Lopes da Fonseca Junior

    2010-04-01

    Full Text Available OBJETIVO: Demonstrar a sensibilidade e especificidade do teste do gelo no diagnóstico diferencial de ptose palpebral por miastenia gravis. MÉTODOS: Estudo prospectivo tipo ensaio clínico com grupo controle. Foi realizado o teste do gelo em pacientes portadores de ptose palpebral. Os pacientes foram divididos em 2 grupos, sendo o grupo I constituído por pacientes com miastenia gravis e o grupo II (controle formado por pacientes portadores de ptose congênita, miogênica não-miastênica ou aponeurótica. RESULTADOS: Todos os pacientes do grupo I tiveram aumento da fenda palpebral de, no mínimo, 3 mm após a aplicação do gelo. Nenhum paciente do grupo II apresentou incremento da fenda palpebral após o teste. CONCLUSÃO: O teste do gelo mostrou-se específico para detecção de ptose palpebral de causa miastênica.PURPOSE: To demonstrate the sensitivity and the specificity of the ice test in the differential diagnosis of ptosis in myasthenia gravis. METHODS: Prospective trial with a control group. The patients were instructed to hold a frozen ice pack on the closed ptotic eyelid. They were divided into 2 groups, with group I consisting of patients with myasthenia gravis and group II (control consisting of patients with congenital, non-myasthenic myogenic or aponeurotic ptosis. RESULTS: All patients in group I had increased palpebral fissure for at least 3 mm after the application of ice pack. No patient in group II showed increased palpebral fissure after the ice test. CONCLUSION: Ice test proved to be specific for the detection of myasthenic ptosis.

  18. 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.

  19. 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.

  20. 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

  1. Invasive medullary thymoma associated with myasthenia gravis: an unusual case Miastenia gravis em um paciente com timoma medular invasivo: relato de caso

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    JORGE S. REIS FILHO

    2000-12-01

    Full Text Available Thymomas are tumors characterized by a remarkable morphological heterogeneity and variable clinical behavior. This tumor has unique clinical associations, most notably with hematological abnormalities and myasthenia gravis. According with the Müller-Hermelink criteria, there are significant differences between the histological types of thymomas and the association with myasthenia gravis. Among the different histological types, medullary thymoma is the least frequent variant associated with this autoimmune disease. In this report we describe a case of medullary thymoma presenting in a 71-year- old woman with a myasthenic syndrome.Os timomas são tumores caracterizados por grande heterogeneidade morfológica e comportamento clínico variável. Este tumor apresenta associações clínicas singulares, principalmente com doenças hematológicas e com a miastenia gravis. De acordo com a classificação de Müller-Hermelink, existem diferenças significativas entre as variedades histológicas dos timomas e sua associação com a miastenia gravis. Entre os diferentes tipos histológicos, o timoma medular é a variante menos frequentemente associada com esta doença autoimune. Neste relato, nós descrevemos caso de timoma medular em uma paciente de 71 anos de idade com síndrome miastênica.

  2. 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

  3. Miastenia gravis: relato de dois casos e revisão da literatura

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    Ana Laura Colle Kauling

    2011-12-01

    Full Text Available JUSTIFICATIVA E OBJETIVOS: A Miastenia gravis (MG é uma doença neurológica autoimune que afeta a porção pós-sináptica da junção neuromuscular. Trata-se de um desafio ao anestesiologista, pela diversidade das manifestações da doença e pela possibilidade de complicações ventilatórias no pós-operatório. O objetivo deste trabalho é demonstrar a importância da monitoração adequada ao bloqueio neuromuscular (BNM em virtude das múltiplas formas de apresentação da MG. CONTEÚDO: Neste artigo serão descritos dois casos de pacientes com MG - um que apresentou a forma clássica de sensibilidade ao bloqueador neuromuscular (BNM e outro com resposta semelhante à de um paciente normal. A revisão da literatura será restrita às características da doença e a descrição de sua fisiopatologia estará voltada às reações aos BNM. CONCLUSÕES: Como conclusão, sugere-se que, em decorrência das múltiplas formas de apresentação e de tratamento da MG, é fundamental o uso de monitores da transmissão neuromuscular quando se usa BNM.

  4. 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.

  5. 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

  6. 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.

  7. Lúpus Eritematoso Sistêmico com fraqueza muscular por Miastenia Gravis Systemic Lupus Erythematosus with muscle weakness due to Myasthenia Gravis

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    Sâmia Araújo de Sousa Studart

    2011-06-01

    Full Text Available O Lúpus Eritematoso Sistêmico (LES e a Miastenia Gravis (MG são doenças autoimunes cuja associação em um mesmo paciente é raramente descrita. Essas patologias compartilham algumas características como acometimento de mulheres jovens, positividade para anticorpos antinucleares, evolução em períodos de exacerbações e remissões. O presente relato de caso analisa as possíveis hipóteses diagnósticas para um quadro clínico de ptose palpebral e fraqueza muscular proximal em uma paciente portadora de lúpus recente que evoluiu com MG associada.Systemic lupus erythematosus (SLE and myasthenia gravis (MG are autoimmune diseases, whose association in the same patient is rarely reported. Both pathologies share the following characteristics: affect mainly young women; alternate exacerbation and remission periods; and have positive antinuclear antibody (ANA test. This case report assesses possible diagnostic hypotheses for the clinical findings of eyelid ptosis and proximal muscle weakness in a female patient recently diagnosed with SLE, who evolved with associated MG.

  8. 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

  9. 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.

  10. 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.

  11. 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.

  12. 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.

  13. 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

  14. 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

  15. 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.

  16. 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...

  17. Doença de Castleman associada a sarcoma de células dendríticas foliculares e miastenia gravis Castleman's disease associated with follicular dendritic cell sarcoma and myasthenia gravis

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    Fernando Luiz Westphal

    2010-12-01

    Full Text Available A doença de Castleman é um distúrbio linfoproliferativo atípico, de etiologia desconhecida, que pode estar associada a uma série de condições clínicas, inclusive doenças de caráter autoimune e neoplasias malignas. No presente relato, uma paciente de 72 anos foi encaminhada ao serviço de cirurgia torácica do Hospital Universitário Getúlio Vargas, localizado na cidade de Manaus (AM para a ressecção de um tumor de mediastino posterior. Três meses antes, havia sido internada em UTI com um quadro de dispneia intensa, ocasião na qual foi diagnosticada miastenia gravis. Após a ressecção da massa mediastinal, a análise histopatológica revelou doença de Castleman hialino-vascular complicada por sarcoma de células dendríticas foliculares. Até o momento da redação deste estudo, a paciente utilizava um anticolinesterásico e corticoides para o controle da miastenia gravis.Castleman's disease is an atypical lymphoproliferative disorder of unknown etiology, which might be associated with various clinical conditions, including autoimmune diseases and malignant neoplasms. We report the case of a 72-year-old female patient who was referred to the thoracic surgery department of Getúlio Vargas University Hospital, in the city of Manaus, Brazil, for the resection of a posterior mediastinal tumor. Three months prior, the patient had been admitted to the ICU with signs of severe dyspnea, at which time she was diagnosed with myasthenia gravis. After the resection of the mediastinal tumor, the histopathological examination revealed hyaline vascular-type Castleman's disease, complicated by follicular dendritic cell sarcoma. At this writing, the patient was being treated with an anticholinesterase agent and corticosteroids for the control of myasthenia gravis.

  18. 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

  19. 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

  20. 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.

  1. 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

  2. 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

  3. 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

  4. Miastenia grave en la adolescencia: A propósito de un caso Myasthenia gravis in adolescence: Apropos of a case

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    Mildrey Jiménez López

    2008-06-01

    Full Text Available En este trabajo se presenta a un adolescente masculino que inició un cuadro de fatigabilidad muscular tras el ejercicio, que lo llevó a desaprobar la asignatura de preparación física. Posteriormente comienza a presentar dificultades para ingerir alimentos, para pronunciar palabras y asimetría en la fuerza muscular de ambos hemicuerpos. Es ingresado, y a los pocos días comienza a presentar alteraciones respiratorias, por lo que se decide su traslado a la sala de cuidados intensivos donde fue necesario acoplarlo a un ventilador mecánico. En esta oportunidad no hubo respuesta a la administración de anticolinesterásicos, sin embargo comenzó a mejorar con el tratamiento con esteroides. Evolucionó satisfactoriamente con recuperación lenta y progresiva de la fuerza muscular. Se restableció el tratamiento con anticolinesterásicos paulatinamente con buena respuesta en esta oportunidad.A male adolescent that started with a picture of muscle weakness after doing exercises that led him to fail the physical education subject is presented in this paper. Later on, he began to have difficulties to eat and to pronounce words, and to show asymmetry in the muscular strength of both hemibodies. He was admitted and a few days after he had respiratory alterations and it was decided to transfer him to an intensive care unit and to couple him to a mechanical ventilator. In that opportunity, there was no response to the administration of anticholinesterase agents; however, he began to improve with steroids. His evolution was satisfactory with a slow and progressive recovery of the muscle strength. The treatment with anticholinesterase agents was gradually re-established with a good response this time.

  5. 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

    Directory of Open Access Journals (Sweden)

    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

  6. 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.

  7. Miastenia grave familiar: relato de caso em gêmeas idênticas

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    Elza Dias-Tosta

    1989-06-01

    Full Text Available Trata-se do sétimo relato da literatura mundial de casos de miastenia gravis em gêmeos homozigóticos em que ambos são acometidos. O homozigotismo foi provado com certeza por estudo de HLA e a forma adquirida da doença foi provada por determinação de níveis elevados de anticorpos anti-receptor de acetilcolina, havendo também níveis elevados de anticorpos antimúsculo estriado, sem outras evidências de timoma.

  8. 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

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    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

  9. 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 ...

  10. Miastenia gravis: resultados de timectomia em 52 pacientes

    Directory of Open Access Journals (Sweden)

    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.

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

    Directory of Open Access Journals (Sweden)

    Lokman Soyoral

    2017-03-01

    Full Text Available 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. Resumo: Miastenia grave (MG é uma doença autoimune caracterizada por fraqueza muscular que flutua, piorando com o esforço físico e melhorando com o repouso. O diagnóstico de MG é feito após exame clínico e físico e confirmado por imunoensaios séricos para medir os níveis de autoanticorpos. MG, especialmente quando associada à gravidez, é uma doença de alto risco e de curso imprevisível. Descrevemos o segundo relato sobre o uso de sugamadex após rocurônio para um parto cesário com miastenia grave, mas, ao contrário de nosso caso que foi previamente diagnosticado com miastenia grave, a paciente foi extubada com sucesso no pós-operatório sem qualquer problema respiratório. Keywords: Myasthenia gravis, Sugammadex, Pregnancy, Palavras-chave: Miastenia grave, Sugammadex, Gravidez

  12. 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 ...

  13. 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 ...

  14. 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- ...

  15. 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.

  16. 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...

  17. 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 ...

  18. 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 ...

  19. Treatment for Myasthenia Gravis (MG)

    Science.gov (United States)

    ... 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.

  20. [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.

  1. 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

  2. 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.

  3. 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

  4. 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

  5. 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)

  6. 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...

  7. 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

    Directory of Open Access Journals (Sweden)

    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

  8. 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

  9. Multiple Thymoma with Myasthenia Gravis

    Directory of Open Access Journals (Sweden)

    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.

  10. 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

  11. 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

  12. Thymus irradiation for myasthenia gravis

    Energy Technology Data Exchange (ETDEWEB)

    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.

  13. Estudo dos linfócitos circulantes por anticorpos monoclonais na miastenia grave

    Directory of Open Access Journals (Sweden)

    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.

  14. 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. ...

  15. 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...

  16. 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

  17. Ocular myasthenia gravis: Side effect of urografin

    Directory of Open Access Journals (Sweden)

    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.

  18. 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.

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

    Directory of Open Access Journals (Sweden)

    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.

  20. 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)

  1. [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.

  2. 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)

  3. 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.

  4. 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 ...

  5. Isolated laryngeal myasthenia gravis for 26 years.

    Science.gov (United States)

    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.

  6. Tratamento das formas severas de miastenia pelo ACTH por via intravenosa

    Directory of Open Access Journals (Sweden)

    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

  7. Psychiatric disorders in myasthenia gravis

    Directory of Open Access Journals (Sweden)

    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.

  8. 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

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

    Science.gov (United States)

    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.

  10. 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...

  11. 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)

  12. 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 ...

  13. Myasthenia Gravis and Its Aeromedical Implications.

    Science.gov (United States)

    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.

  14. 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.).

  15. 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

  16. Multiple thymoma in a patient with myasthenia gravis: case report

    Energy Technology Data Exchange (ETDEWEB)

    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.

  17. [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.

  18. 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.

  19. 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

  20. 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.

  1. Autoimmune Thyroiditis and Myasthenia Gravis

    Directory of Open Access Journals (Sweden)

    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.

  2. 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

  3. 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.

  4. Familial myasthenia gravis: report of four cases

    Directory of Open Access Journals (Sweden)

    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.

  5. 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

  6. Clinical predictors for the prognosis of myasthenia gravis.

    Science.gov (United States)

    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.

  7. 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

  8. Vegetables, Soups, Sauces, Gravies and Beverages.

    Science.gov (United States)

    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…

  9. 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

  10. CT evaluation of thymus in myasthenia gravis

    Energy Technology Data Exchange (ETDEWEB)

    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.

  11. 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

  12. Ocular myasthenia gravis in a setting of thyrotoxicosis

    Directory of Open Access Journals (Sweden)

    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.

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

    Directory of Open Access Journals (Sweden)

    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.

  14. 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

  15. SPECIFIC FEATURES OF ANESTHESIA IN PATIENTS WITH MYASTHENIA GRAVIS.

    Science.gov (United States)

    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

  16. [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.

  17. 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

  18. Myasthenia Gravis in Pregnancy: A Case Report

    Directory of Open Access Journals (Sweden)

    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.

  19. 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 ...

  20. 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 ...

  1. 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 ...

  2. 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.

  3. Autoimmune Myasthenia Gravis after Sternal Fracture

    Directory of Open Access Journals (Sweden)

    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.

  4. 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

  5. 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.

  6. 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.

  7. 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

  8. Juvenile Myasthenia Gravis: A Paediatric Perspective

    Directory of Open Access Journals (Sweden)

    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.

  9. 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.

  10. Myopathy in Childhood Muscle-Specific Kinase Myasthenia Gravis.

    Science.gov (United States)

    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.

  11. Infantile Onset Myasthenia Gravis with MuSK Antibodies

    Directory of Open Access Journals (Sweden)

    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.

  12. 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...

  13. Myasthenia Gravis – a beginning with no end

    Directory of Open Access Journals (Sweden)

    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.

  14. Diagnostico y tratamiento de la myasthenia gravis estudio de una poblacion hospitalaria

    Directory of Open Access Journals (Sweden)

    Roberto D. Rey

    1990-09-01

    Full Text Available Durante la observacion de 50 pacientes con Myasthenia gravis entre los anos 1974 y 1987 se encontro un predominio del sexo femenino en la proporcion de 2,5 a 1 y mayor frecuencia de presentacion en Ias 3ª y 5ª décadas de la vida. Luego de la evaluacion clínica, el diagnóstico fué confirmado mediante: (1 prueba dei edrofonio, (2 estímulo nervioso repetitivo, (3 dosaje de anticuerpos antirreceptor colinérgico y (4 transferencia pasiva del suero al raton con ulterior niedicion de la amplitud de mepp's. La positividad diagnostica vario entre el 90 y el 100%, segun el tipo de prueba empleada. El estúdio radiológico del timo fué hecho con neumomediastinografía. obteniendose excelente correlación con la descripcion histológica de la glândula, y con tomografia computada, que demostro menor eficiência diagnostica. El tratamiento fué implementado en base a anticolinesterásicos, timectomía y corticoterapia inmunosupresora, evidenciandose con esta última mejores resultados. Nueve pacientes desarrollaron peoria transitória del cuadro muscular al iniciarse el tratamiento esteroideo, 6 de ellos exhibieron posteriormente evolucion desfavorable. Esta observacion aparenta tener valor pronóstico en la evolucion de la MG.

  15. 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

  16. 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.

  17. 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.

  18. Myasthenia gravis and pregnancy: clinical implications and neonatal outcome

    Directory of Open Access Journals (Sweden)

    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.

  19. 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.

  20. 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.

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

    Directory of Open Access Journals (Sweden)

    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.

  2. 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.

  3. 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.

  4. 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.)

  5. 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.

  6. Myasthenia Gravis: Unusual Presentations and Diagnostic Pitfalls.

    Science.gov (United States)

    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.

  7. 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

  8. 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.

  9. 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 ...

  10. 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.

  11. 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 ...

  12. 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 ...

  13. 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.

  14. 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

  15. Ectopic cervical thymoma in a patient with Myasthenia gravis

    Directory of Open Access Journals (Sweden)

    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.

  16. Myasthenia gravis exacerbation and diarrhea associated with erythromycin treatment

    Directory of Open Access Journals (Sweden)

    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.

  17. 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.

  18. Prognosis of thymectomy in myasthenia gravis patients with thymus hyperplasia.

    Science.gov (United States)

    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.

  19. 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.

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

    Science.gov (United States)

    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.

  1. 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.

  2. 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.

  3. 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

  4. 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.

  5. 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)

  6. 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.

  7. 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.

  8. 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

  9. 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.

  10. 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

  11. [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.

  12. Vocal cord palsy: An uncommon presenting feature of myasthenia gravis

    Directory of Open Access Journals (Sweden)

    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.

  13. Myasthenia gravis: new developments in research and treatment.

    Science.gov (United States)

    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.

  14. 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.

  15. Home sleep study for patients with myasthenia gravis.

    Science.gov (United States)

    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.

  16. 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.

  17. A conceptual framework for evaluating impairments in myasthenia gravis.

    Directory of Open Access Journals (Sweden)

    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.

  18. 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 ...

  19. 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

  20. 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.

  1. 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.

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

    Science.gov (United States)

    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.

  3. 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

  4. 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

  5. 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.

  6. 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.

  7. 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.

  8. [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.

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

    Science.gov (United States)

    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.

  10. A Genome-wide Association Study of Myasthenia Gravis

    Science.gov (United States)

    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

  11. Clinical Characteristics of Juvenile Myasthenia Gravis in Southern China

    Directory of Open Access Journals (Sweden)

    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.

  12. 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.

  13. 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

  14. 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

  15. 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.)

  16. 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.

  17. 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.

  18. 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

  19. 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″...

  20. 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...

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

    Directory of Open Access Journals (Sweden)

    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.

  2. 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.

  3. 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

  4. 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

  5. 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

  6. 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.

  7. 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.

  8. 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.

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

    Science.gov (United States)

    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.

  10. 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)

  11. Early onset bilateral juvenile myasthenia gravis masquerading as simple congenital ptosis

    Directory of Open Access Journals (Sweden)

    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.

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

    Science.gov (United States)

    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

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

    Science.gov (United States)

    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.

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

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    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.

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

    Science.gov (United States)

    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.

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

    Directory of Open Access Journals (Sweden)

    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.

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

    Science.gov (United States)

    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.

  18. 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.

  19. 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

  20. Severe Preeclampsia in the Setting of Myasthenia Gravis

    Directory of Open Access Journals (Sweden)

    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.

  1. 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.

  2. 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.

  3. 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.

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

    Science.gov (United States)

    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.

  5. Informes clínicos breves

    Directory of Open Access Journals (Sweden)

    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

  6. Endocardite acuta da Staphylococcus haemolyticus meticillino sensibile, con recidiva dopo trattamento da ceppo meticillino resistente

    Directory of Open Access Journals (Sweden)

    Giacomo Magnani

    2005-03-01

    Full Text Available Le endocarditi su valvole native (NVE da Stafilococchi coagulasi negativi (CoNS presentano un andamento aggressivo simile a quello da Staphylococcus aureus e sono difficili da trattare per l’alta incidenza da parte dei CoNS di resistenza a oxacillina.Viene riportato un caso di NVE da Staphylococcus haemolyticus meticillino sensibile, con risposta iniziale favorevole al trattamento con oxacillina, ma con successiva recidiva a distanza di tre mesi legata comparsa di ceppo oxacillino-resistente. L’ipotesi è che l’infezione sia stata sostenuta da una popolazione batterica con fenotipo eteroresistente, che in seguito al trattamento antibiotico ha sviluppato resistenza omogenea a oxacillina. Nell’impostare un trattamento per infezioni gravi da Stafilococchi sensibili in vitro ad oxacillina è necessario considerare sempre la presenza di un eteroresistenza.

  7. 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

  8. 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.

  9. [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.

  10. Regulatory T cells in multiple sclerosis and myasthenia gravis.

    Science.gov (United States)

    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

  11. 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

  12. 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

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

    Directory of Open Access Journals (Sweden)

    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.

  14. 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.

  15. 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.

  16. Patient registries: useful tools for clinical research in myasthenia gravis.

    Science.gov (United States)

    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.

  17. 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

  18. 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)

  19. DIAGNOSIS OF MYASTHENIA GRAVIS USING FUZZY GAZE TRACKING SOFTWARE

    Directory of Open Access Journals (Sweden)

    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.

  20. Current and emerging therapies for the treatment of myasthenia gravis

    Science.gov (United States)

    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

  1. Computed tomography of the thymus in myasthenia gravis

    Energy Technology Data Exchange (ETDEWEB)

    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).

  2. 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.

  3. Three Types of Striational Antibodies in Myasthenia Gravis

    Directory of Open Access Journals (Sweden)

    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.

  4. 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)

  5. 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.

  6. 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)

  7. 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.

  8. Myasthenia gravis in children: analysis of 18 patients

    Directory of Open Access Journals (Sweden)

    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.

  9. 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

  10. 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

  11. 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.

  12. 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.).

  13. Assessment of cognitive function in patients with myasthenia gravis

    Directory of Open Access Journals (Sweden)

    Sherifa A Hamed

    2014-12-01

    Full Text Available Aim: During the past decade, there has been an increasing interest in the evaluation of cognitive function in myasthenia gravis (MG, neuromuscular transmission disorder caused by acetylcholine receptor auto-antibodies. However, the results of previous studies on cognition and MG are inconsistent and controversial. This study aimed to evaluate cognition in patients with mild/moderate grades of MG. Methods: This study included 20 patients with MG with a mean age of 28.45 ± 8.89 years and duration of illness of 3.52 ± 1.15 years. Cognition was tested using a sensitive battery of psychometric testing (Mini-mental State Examination [MMSE], Stanford-Binet Intelligence Scale 4 th edition [SBIS] and Wechsler Memory Scale-Revised [WMS-R] and by recording P300 component of event-related potentials (ERPs, a neurophysiological analog for cognitive function. Results: Compared with healthy subjects (n = 20, patients had lower total scores of cognitive testing (MMSE, SBIS and WMS-R (P = 0.001, higher Beck Depression Inventory 2 nd edition scores (P = 0.0001 and prolonged latencies (P = 0.01 and reduced amplitudes (P = 0.001 of P300 component of ERPs. Correlations were identified between total scores of cognitive testing and age (r = -0.470, P = 0.010, duration of illness (r = -0.788, P = 0.001 and depression scores (r = -0.323, P = 0.045. Using linear regression analysis and after controlling for age and depression scores, a significant correlation was identified between total scores of cognitive testing and duration of illness (β = -0.305, P = 0.045. Conclusion: Patients with mild/moderate MG may have cognitive dysfunction. This is important to determine prognosis and managing patients.

  14. 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...

  15. 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.

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

    Science.gov (United States)

    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.

  17. 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...

  18. 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

  19. 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...

  20. 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...

  1. “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, ...

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

    Science.gov (United States)

    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.

  3. 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.…

  4. 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.

  5. 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.

  6. 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)

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

    Science.gov (United States)

    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.

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

    Science.gov (United States)

    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.

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

    Science.gov (United States)

    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

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

    Science.gov (United States)

    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.

  11. 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

  12. 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...

  13. 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.

  14. 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 ...

  15. Complete stable remission and autoantibody specificity in myasthenia gravis.

    Science.gov (United States)

    Baggi, Fulvio; Andreetta, Francesca; Maggi, Lorenzo; Confalonieri, Paolo; Morandi, Lucia; Salerno, Franco; Bernasconi, Pia; Montomoli, Cristina; Barberis, Massimo; Mantegazza, Renato; Antozzi, Carlo

    2013-01-08

    Patients with myasthenia gravis (MG) are subgrouped as acetylcholine receptor (AChR)-positive, muscle-specific kinase (MuSK)-positive, and AChR/MuSK-negative MG (or double negative [DN]) on the basis of autoantibody assay. We investigated the relationships between autoantibody specificity, main clinical features, and outcome of the disease, in particular the occurrence of complete stable remission (CSR), by means of a retrospective study on a cohort of 677 Italian patients with MG. A total of 517 (76%) patients with AChR-positive MG, 55 (8%) patients with MuSK-positive MG, and 105 (16%) patients with DN MG were included in the study. Kaplan-Meier and Cox proportional hazard regression analyses were used to evaluate associations between baseline characteristics, antibody specificity, and CSR. Clinical stage at onset and at maximal worsening was more severe for MuSK-positive patients: bulbar impairment at maximal worsening was found in 83.6% of MuSK-positive patients compared with 58.6% of AChR-positive patients and 43.8% of DN patients (p CSR was observed in 3.6% of MuSK-positive patients compared with 22.2% of AChR-positive and 21.9% of DN patients. In the whole MG cohort, onset before age 40 (hazard ratio [HR] = 1.96, 95% confidence interval [CI] 1.27-3.02, p = 0.002) and ocular and generalized clinical stages at maximal worsening were associated with CSR (ocular, HR = 8.05, 95% CI 1.88-34.53, p = 0.005; generalized, HR = 3.71, 95% CI 1.16-11.90, p = 0.023; bulbar, HR = 3.16, 95% CI 1.00-10.05, p = 0.051). MuSK antibodies identify a clinically distinguishable, more severe form of MG since the disease onset, with a lower occurrence of CSR. These features should be considered by the clinician in the management of this particular form of MG.

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

    Directory of Open Access Journals (Sweden)

    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.

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

    Directory of Open Access Journals (Sweden)

    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.

  18. 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.

  19. [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.

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

    Directory of Open Access Journals (Sweden)

    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.

  1. 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.

  2. 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.

  3. 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

  4. 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.

  5. 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.

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

    Directory of Open Access Journals (Sweden)

    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.

  7. 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.

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

    Directory of Open Access Journals (Sweden)

    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.

  9. 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.

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

    Science.gov (United States)

    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.

  11. [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.

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

    Science.gov (United States)

    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.

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

    Science.gov (United States)

    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.

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

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    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.

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

    Directory of Open Access Journals (Sweden)

    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.

  16. 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.

  17. Differential Cytokine Changes in Patients with Myasthenia Gravis with Antibodies against AChR and MuSK.

    Directory of Open Access Journals (Sweden)

    Vuslat Yilmaz

    Full Text Available Neuromuscular transmission failure in myasthenia gravis (MG is most commonly elicited by autoantibodies (ab to the acetylcholine receptor or the muscle-specific kinase, constituting AChR-MG and MuSK-MG. It is controversial whether these MG subtypes arise through different T helper (Th 1, Th2 or Th17 polarized immune reactions and how these reactions are blunted by immunosuppression. To address these questions, plasma levels of cytokines related to various Th subtypes were determined in patients with AChR-MG, MuSK-MG and healthy controls (CON. Peripheral blood mononuclear cells (PBMC were activated in vitro by anti-CD3, and cytokines were quantified in supernatants. In purified blood CD4+ T cells, RNA of various cytokines, Th subtype specific transcription factors and the co-stimulatory molecule, CD40L, were quantified by qRT-PCR. Plasma levels of Th1, Th2 and Th17 related cytokines were overall not significantly different between MG subtypes and CON. By contrast, in vitro stimulated PBMC from MuSK-MG but not AChR-MG patients showed significantly increased secretion of the Th1, Th17 and T follicular helper cell related cytokines, IFN-γ, IL-17A and IL-21. Stimulated expression of IL-4, IL-6, IL-10 and IL-13 was not significantly different. At the RNA level, expression of CD40L by CD4+ T cells was reduced in both AChR-MG and MuSK-MG patients while expression of Th subset related cytokines and transcription factors were normal. Immunosuppression treatment had two effects: First, it reduced levels of IL12p40 in the plasma of AChR-MG and MuSK-MG patients, leaving other cytokine levels unchanged; second, it reduced spontaneous secretion of IFN-γ and increased secretion of IL-6 and IL-10 by cultured PBMC from AChR-MG, but not MuSK-MG patients. We conclude that Th1 and Th17 immune reactions play a role in MuSK-MG. Immunosuppression attenuates the Th1 response in AChR-MG and MuSK-MG, but otherwise modulates immune responses in AChR-MG and Mu

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

    Directory of Open Access Journals (Sweden)

    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.

  19. 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

  20. [Thymus surgery in a general surgery department].

    Science.gov (United States)

    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.

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

    Science.gov (United States)

    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

  2. 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

  3. 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

  4. 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

  5. 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.

  6. 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.

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

    Directory of Open Access Journals (Sweden)

    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.

  8. 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.

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

    Science.gov (United States)

    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.

  10. 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.

  11. [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.

  12. 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.

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

    Science.gov (United States)

    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.

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

    Science.gov (United States)

    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.

  15. 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

  16. 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

  17. 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.

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

    Science.gov (United States)

    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.

  19. [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.

  20. 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.

  1. Teste de estimulação repetitiva no músculo ancôneo para diagnóstico da miastenia grave: mapeamento da sua área de placa motora Repetitive stimulation test on the anconeus muscle for the diagnosis of myasthenia gravis: the mapping of its motor end-plate area

    Directory of Open Access Journals (Sweden)

    Maria das Graças Wanderley S. Coriolano

    2007-06-01

    Full Text Available OBJETIVO: Mapear a área de placa motora do músculo ancôneo para definir a melhor localização dos eletrodos de registro em testes de estimulação repetitiva (TER no diagnóstico dos distúrbios da transmissão neuromuscular. MÉTODO: Registramos o potencial de ação composto do músculo ancôneo sobre a pele que o recobre, após estimulação do ramo que o inerva. Analisando as formas de onda registradas em cada ponto da pele foi possível definir a área de placa. RESULTADOS: A área de placa motora do ancôneo é uma linha paralela à borda da ulna. O melhor local de colocação do eletrodo "ativo" de registro situa-se cerca de 2 cm distal ao olécrano e 1 cm lateral à borda da ulna. CONCLUSÃO: A realização de TER no músculo ancôneo é simples e bem tolerada. Com a estimulação do ancôneo o antebraço praticamente não se move, sendo o procedimento livre de artefatos de movimento.PURPOSE: To map the motor end-plate area of the anconeus muscle and define the best place for positioning the recording electrodes in repetitive stimulation tests (RST for the diagnosis of neuromuscular transmission disorders. METHOD: The compound muscle action potential of the anconeus was recorded after stimulating the motor branch of the radial nerve that innervates it. By analyzing the waveforms registered at each point of the skin we were able to define the motor end-plate area. RESULTS: The motor end-plate area of the anconeus is a line parallel to the ulna border. The best place for placing the "active" recording electrode is about 2cm distal to the olecranon and 1 cm lateral to the border of the ulna. CONCLUSION: Performing RST in the anconeus muscle is simple and well tolerated. Stimulation of the anconeus almost doesn't move the forearm and the procedure is virtually free of movement artifacts.

  2. 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.

  3. 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

  4. 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

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

    Science.gov (United States)

    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.

  6. 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.

  7. 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

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

    Directory of Open Access Journals (Sweden)

    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

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

    Directory of Open Access Journals (Sweden)

    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

  10. 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)

  11. [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.

  12. [Myasthenia gravis with the electrographic response of a myasthenic syndrome. Report of a case].

    Science.gov (United States)

    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.

  13. 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.

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

    Science.gov (United States)

    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.

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

    Directory of Open Access Journals (Sweden)

    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.

  16. 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.

  17. 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.

  18. 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.

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

    Science.gov (United States)

    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.

  20. Treatment of epilepsy in patients with myasthenia gravis: Is really harder than it looks?

    Science.gov (United States)

    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.

  1. 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.

  2. 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.

  3. 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.

  4. 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)

  5. Computed tomography of the anterior mediastinum in myasthemia gravis: a radiologic-pathologic correlative study

    Energy Technology Data Exchange (ETDEWEB)

    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.

  6. The Clinical Outcome and Therapeutic Treatment of a Patient with Double Seronegative Myasthenia Gravis

    Directory of Open Access Journals (Sweden)

    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.

  7. 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

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

    Science.gov (United States)

    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.

  9. 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

  10. Muscle satellite cells are functionally impaired in myasthenia gravis: consequences on muscle regeneration.

    Science.gov (United States)

    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.

  11. 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.

  12. 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.

  13. 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.

  14. 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...

  15. Paciente con esquizofrenia tratado con ziprasidona + clozapina

    Directory of Open Access Journals (Sweden)

    Pol Yanguas E.

    2013-05-01

    Full Text Available P es un paciente diagnosticado de esquizofrenia, sigue en un piso tutelado un programa de rehabilitación, está medicado con clozapina 500 mg/día y ziprasidona 280 mg/ día. Padece hipercolesterolemia, tabaquismo y sus hábitos alimenticios no son buenos. La medicación que utiliza desde 2007 hasta ahora se refleja en la tabla 1. El último tratamiento se le introdujo el 7 de agosto de 2012, habiendo presentado un electro cardiograma (ECG normal, pero con ligera taquicardia ventricular y prolactinemia de 44,8 ng/ml (valores normales: 2-18 ng/ml.

  16. 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.

  17. 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.

  18. 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

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

    Directory of Open Access Journals (Sweden)

    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

  20. Decreased bone mineral density in experimental myasthenia gravis in C57BL/6 mice.

    Science.gov (United States)

    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.

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

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    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.

  2. 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.

  3. Treatment of velopharyngeal inadequacy in a patient with submucous cleft palate and myasthenia gravis.

    Science.gov (United States)

    Rikihisa, Naoaki; Udagawa, Akikazu; Yoshimoto, Shinya; Ichinose, Masaharu; Kimura, Tomoe; Shimizu, Sara

    2009-09-01

    To describe the clinical course and management of a patient with submucous cleft palate who developed myasthenia gravis (MG) as an adult and suffered recurrent hypernasality. Few reports have described MG patients undergoing pharyngeal flap surgery for velopharyngeal incompetence, and these have described only slight speech improvement in such patients. Case report. The patient underwent primary pushback palatoplasty and superiorly based pharyngeal flap surgery for submucous cleft and short palate at age 7. Hypernasality showed major improvement after initial surgery. At age 19, the patient developed MG that triggered the recurrence of velopharyngeal incompetence. After MG was treated, revision pushback palatoplasty was performed for velopharyngeal incompetence when the patient was 24 years old. Preoperatively and postoperatively, the patient was evaluated by the same speech-language-hearing therapists, each with at least 5 years of clinical experience in cleft palate speech. After the second pushback palatoplasty, hypernasality and audible nasal air emission during speech decreased to mild. Primary pushback palatoplasty and pharyngeal flap surgery were performed for the submucous cleft palate. Revision pushback palatoplasty improved velopharyngeal inadequacy induced by MG. Decreased perceived nasality positively influenced the patient's quality of life. Combined pushback palatoplasty and pharyngeal flap surgery is thus an option in surgical treatment for velopharyngeal inadequacy to close the cleft and the velopharyngeal orifice in cases of cleft palate and MG.

  4. Thymic lesions and myasthenia gravis. Diagnosis based on mediastinal imaging and pathological findings

    International Nuclear Information System (INIS)

    Pirronti, T.; Rinaldi, P.; Marano, P.; Batocchi, A.P.; Evoli, A.; Di Schino, C.

    2002-01-01

    Purpose: To achieve a better understanding of the role of CT and MR imaging in the study of the mediastinum in patients with myasthenia gravis (MG). Material and Methods: Mediastinal CT and MR findings were correlated with the histopathological results in 104 thymectomized MG patients. Results: CT was performed in 104 patients; in 11 of them, MR was also carried out. 44 patients had hyperplasia at histology. On CT, thymic hyperplasia was confirmed in 16 cases, thymoma was diagnosed in 10 and a normal thymus in 18 (sensitivity 36%, specificity 95%). Of 52 patients with thymoma at histology, CT showed thymoma in 46, hyperplasia in 1, and normal thymus in 5. CT showed 88.5% sensitivity and 77% specificity for thymoma. In 10 patients with invasive thymoma, CT was indiscriminate, while invasiveness was detected in 7 cases at MR (70% sensitivity) and at CT in 1 case. Both CT and MR detected tumor recurrence in 5 cases, but the exact localization and degree of invasion were best defined by MR. Conclusion: In MG patients CT is a sensitive, specific and efficient modality for detecting thymoma, but is less so for detecting thymic hyperplasia. MR was shown to be accurate in detecting invasive thymoma both preoperatively and in postoperative follow-up

  5. The effect of pale, soft and exudative meat on the quality of canned pork in gravy.

    Science.gov (United States)

    Florowski, Tomasz; Florowska, Anna; Chmiel, Marta; Adamczak, Lech; Pietrzak, Dorota; Ruchlicka, Magdalena

    2017-01-01

    The objective of the study was to evaluate the use of PSE meat in the production of sterilized pork type canned meat in its own gravy. Canned meat products were produced with 50% of PSE meat as well as with 100% PSE meat, and compared with canned meat products of good quality (RFN). It was found that decreased quality of PSE meat had a small impact on the quality of canned meat products. Substitution of both 50% as well as the total quantity of RFN meat with PSE meat did not affect the course of the sterilization process, neither increase the quantity of excreted fat and jelly in canned meat. It also had no effect on the instrumentally-measured parameters of texture and neither did it affect different sensory quality features, including the overall desirability of the product. The PSE canned meat product were characterized by higher values of L* and b* color parameters. Copyright © 2016 Elsevier Ltd. All rights reserved.

  6. Association between myasthenia gravis and cognitive function: A systematic review and meta-analysis

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    Zhifeng Mao

    2015-01-01

    Full Text Available The course of myasthenia gravis (MG is complicated by increased reports of cognitive defects in both human and animal models, which suggests potential central nervous system (CNS damage. We conducted a systematic review of the relationships between MG and cognitive function. This systematic review followed Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA guidelines. Major databases were searched to examine the neuropsychological studies of adults with MG. Weighted effect sizes were pooled by cognitive domain. Eight studies representing 300 subjects were included. Eight cognitive domain categories were identified: (i Mini-Mental State Examination (MMSE, (ii language, (iii processing speed, (iv verbal learning and memory, (v visual learning and memory, (vi attention span, (vii response fluency, and (viii motor performance. Nine (cognitive domain categories, MMSE, language, processing speed, verbal learning and memory (except for delayed recall memory, and motor performance of 16 cognitive tasks revealed significant moderate effect sizes. Verbal logical-delayed memory, finger tapping with the preferred hand, and the Symbol Digit Modalities Test showed a greater magnitude relationship to cognitive function than did other specific cognitive domains. Verbal learning and memory seems to be the most significant affected according to cognitive domain categories. For MG, the ability of attention, response fluency, visual learning, and memory seems to be reserved. The MG patients seem to perform significantly worse than the non-MG controls in a range of cognitive domains. Our findings should be interpreted with caution because of the clinical and methodological heterogeneity of included studies.

  7. Intravenous flurbiprofen for post-thymectomy pain relief in patients with myasthenia gravis

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    Su Chunhua

    2012-09-01

    Full Text Available Abstract Background Post-thymectomy pain in myasthenia gravis (MG patients can inhibit breathing and coughing. Inappropriate usage of analgesics may exacerbate respiratory inhibition and even cause myasthenic crisis. Flurbiprofen is a non-steroidal anti-inflammatory drug (NSAID that is commonly used to control moderate postoperative pain and is not associated with respiratory inhibition. We hypothesized that flurbiprofen may provide post-thymectomy pain relief without increasing the risk of complications in MG patients. Methods Two hundred MG patients underwent extended thymectomy from March 2006 to December 2010 and were randomly allocated to a flurbiprofen group (110 patients, 50 mg intravenous flurbiprofen axetil or a control group (90 patients, 100 mg intramuscular tramadol as postoperative analgesia. Visual analog scale (VAS pain score, heart rate, blood pressure, respiratory rate, pulse oximetry (SpO2, and adverse effects were recorded before and up to 24 h after drug administration. Results There were no significant differences in the preoperative clinical characteristics of the flurbiprofen and control (tramadol groups. Both flurbiprofen and tramadol significantly alleviated post-thymectomy pain (p p 2 in either group at all time points. Conclusions Post-thymectomy intravenous administration of flurbiprofen axetil provides safe and effective analgesia for MG patients.

  8. Intravenous flurbiprofen for post-thymectomy pain relief in patients with myasthenia gravis

    Science.gov (United States)

    2012-01-01

    Background Post-thymectomy pain in myasthenia gravis (MG) patients can inhibit breathing and coughing. Inappropriate usage of analgesics may exacerbate respiratory inhibition and even cause myasthenic crisis. Flurbiprofen is a non-steroidal anti-inflammatory drug (NSAID) that is commonly used to control moderate postoperative pain and is not associated with respiratory inhibition. We hypothesized that flurbiprofen may provide post-thymectomy pain relief without increasing the risk of complications in MG patients. Methods Two hundred MG patients underwent extended thymectomy from March 2006 to December 2010 and were randomly allocated to a flurbiprofen group (110 patients, 50 mg intravenous flurbiprofen axetil) or a control group (90 patients, 100 mg intramuscular tramadol) as postoperative analgesia. Visual analog scale (VAS) pain score, heart rate, blood pressure, respiratory rate, pulse oximetry (SpO2), and adverse effects were recorded before and up to 24 h after drug administration. Results There were no significant differences in the preoperative clinical characteristics of the flurbiprofen and control (tramadol) groups. Both flurbiprofen and tramadol significantly alleviated post-thymectomy pain (p flurbiprofen group had significantly lower VAS pain scores at 0.5 h, 2 h, 4 h, and 8 h after surgery (p flurbiprofen axetil provides safe and effective analgesia for MG patients. PMID:23020939

  9. Study of the prevalence of familial autoimmune myasthenia gravis in a Spanish cohort.

    Science.gov (United States)

    Salvado, Maria; Canela, Merce; Ponseti, Jose Maria; Lorenzo, Laura; Garcia, Cecilia; Cazorla, Sonia; Gili, Gisela; Raguer, Nuria; Gamez, Josep

    2016-01-15

    Myasthenia gravis (MG) is an autoimmune disease caused by a failure of neuromuscular transmission. Familial clustering has been reported despiteMG usually manifesting as a sporadic condition presumed not to be inherited. Our study investigated the prevalence of FAMG in a Spanish cohort, characterizing their phenotype,antibody titres and thymus findings. We investigated the presence of familial cases in 462 MG patients, characterizing by age and MGFA class at debut, quantitative MG score, antibody titres, MGFA post-intervention status and thymus pathology. Sixteen cases from8 unrelated pedigrees were identified. The prevalence of FAMG caseswas 3.46%.Mean age at onset was 57.8 ± 17.4 years (range=23–82). Distribution at debut was: 6 ocular, 4 IIa, 4IIb, 1 IIIa and 1 IIIb. Thymoma was identified in two of the 7 thymectomized individuals. The prevalence of FAMG in Spain is similar to other populations. Post-intervention status did not differ from sporadic autoimmune MG. As in other neuromuscular disorders, phenotype and inheritance heterogeneity are present in FAMG. In addition to the interfamilial heterogeneity observed, members of the same family affected with FAMG may even present different ages of onset, severity and thymus involvement. Further studies are necessary to clarify the role of genetic risk factors in this form of autoimmune MG.

  10. Effective humoral immunity against diphtheria and tetanus in patients with systemic lupus erythematosus or myasthenia gravis.

    Science.gov (United States)

    Csuka, Dorottya; Czirják, László; Hóbor, Renáta; Illes, Zsolt; Bánáti, Miklós; Rajczy, Katalin; Tordai, Attila; Füst, George

    2013-07-01

    Controversy exists about the effectiveness of vaccine-induced immune response in patients with immunoregulatory disorders. Our aim was to determine the antibody titers to diphtheria and tetanus in patients with either of two autoimmune diseases. 279 patients with SLE (205 females, aged 45.0 ± 13.8 years), 158 patients with myasthenia gravis (MG) (101 females, aged 55 ± 18.7 years) and 208 healthy subjects (122 females, aged 48 ± 14.6 years) were enrolled. Serum concentrations of diphtheria-antitoxin-IgG (A-DIPHTH) and tetanus-antitoxoid-IgG (A-TET) were determined with ELISA. Equal proportions of healthy subjects, as well as patients with SLE or MG exhibited proper antibody responses and immune protection against diphtheria and tetanus. In all three test groups, serum concentration of A-DIPHTH decreased significantly (p60-years-old) subjects. There were no significant differences among the groups in the age-related changes of A-TET and A-DIPHTH except that in diphtheria and tetanus infections in patients with SLE or MG is comparable to the healthy population. Copyright © 2013 Elsevier Ltd. All rights reserved.

  11. Preoperative High-Dose Steroid Has Long-Term Beneficial Effects for Myasthenia Gravis

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    Syuichi Tetsuka

    2013-01-01

    Full Text Available Previous studies addressing preoperative steroid treatment have revealed that control of myasthenia gravis (MG with steroids prior to surgery appeared to stabilize postoperative status. The purpose of our study was to clarify the clinical benefits of the preoperative programmed high-dose steroid treatment on the long-term outcomes of MG patients. We retrospectively reviewed the records of 171 MG patients who were followed up after undergoing thymectomy in our hospital between 1988 and 2006. One hundred and thirteen patients in the programmed treatment group had received preoperative steroid treatment, while 58 patients received no steroid treatment during the preoperative period. Clinical remission, which was defined as the achievement of the modified pharmacologic remission (PR for at least 1 year, and clinical benefits were compared between the two groups. With regard to the remission after thymectomy, Kaplan-Meier life-table curves for patients in the preoperative steroid treatment group versus those for patients in the no steroid preoperative treatment group revealed a significantly higher probability of the PR in the preoperative steroid treatment group (log-rank test, P<0.01. This study might be the first, as per our knowledge, to indicate that preoperative programmed high-dose steroid treatment has long-term beneficial effects for MG patients.

  12. 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.

  13. Binding affinities of anti-acetylcholine receptor autoantibodies in myasthenia gravis

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    Bray, J.J.; Drachman, D.B.

    1982-01-01

    Antibodies directed against acetylcholine (ACh) receptors are present in the sera of nearly 90% of patients with myasthenia gravis (MG), and are involved in the pathogenesis of this autoimmune disease. However, the antibody titers measured by the standard radioimmunoassay correspond poorly with the clinical severity of the disease. To determine whether this disparity could be accounted for by differences in the binding affinities of anti-ACh receptor antibodies in different patients, we have measured the binding affinities of these autoantibodies in 15 sera from MG patients. The affinity constants (K/sub o/), as determined by Scatchard analysis, were all in the range of 10/sup 10/ M/sup -1/, comparable to the highest values reported in immunized animals. The affinity constants were truly representative of the population of autoantibodies detected by the radioimmunoassay, as shown by the remarkable linearity of the Scatchard plots (r/sup 2/>0.90) and the close correlation between the antibody titers determined by extrapolation of the Scatchard plots and by saturation analysis (r = 0.99; p < 0.001). There was only a 6-fold variation in affinity constants measured in this series of patients despite widely differing antibody titers and severity of the disease. Factors other than the titer and affinity of anti-ACh receptor antibodies may correlate better with the clinical manifestations of MG.

  14. Immunization of mice with LRP4 induces myasthenia similar to MuSK-associated myasthenia gravis.

    Science.gov (United States)

    Mori, Shuuichi; Motohashi, Norio; Takashima, Rumi; Kishi, Masahiko; Nishimune, Hiroshi; Shigemoto, Kazuhiro

    2017-11-01

    Since the first report of experimental animal models of myasthenia gravis (MG) with autoantibodies against low-density lipoprotein receptor-related protein 4 (LRP4), there have not been any major reports replicating the pathogenicity of anti-LRP4 antibodies (Abs). Recent clinical studies have cast doubt on the specificity and pathogenicity of anti-LRP4 antibodies for MG, highlighting the need for further research. In this study, we purified antigens corresponding to the extracellular region of human LRP4 stably expressed with chaperones in 293 cells and used these antigens to immunize female A/J mice. Immunization with LRP4 protein caused mice to develop myasthenia having similar electrophysiological and histological features as are observed in MG patients with circulating Abs against muscle-specific kinase (MuSK). Our results clearly demonstrate that active immunization of mice with LRP4 proteins causes myasthenia similar to the MG induced by anti-MuSK Abs. Further experimental and clinical studies are required to prove the pathogenicity of anti-LRP4 Abs in MG patients. Copyright © 2017 The Authors. Published by Elsevier Inc. All rights reserved.

  15. Electrocardiography as the First Step for the Further Examination of Cardiac Involvement in Myasthenia Gravis

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    Takao Kato

    2016-01-01

    Full Text Available Introduction. Cardiac involvement of myasthenia gravis (MG accompanies a poor prognosis. In the present study, we aimed to investigate the relationship between ECG abnormality and cardiac involvement. Methods. Of 178 patients diagnosed with MG between 2001 and 2013 at our hospital, we retrospectively analyzed consecutive 58 patients who underwent both ECG and echocardiography and without underlying cardiovascular disease. ECG abnormalities were defined by computer-assigned Minnesota-codes. Cardiac damage was defined as either (1 ejection fraction (EF 8 on echocardiography. Results. Thirty-three patients (56.8% had ECG abnormality. An elevated E/e′ was observed in patients with ECG abnormality compared to those without ECG abnormality (11.2±3.2, 8.7±2.2, resp., p=0.03. Among patients with ECG abnormality, 14 of 15 patients showed cardiac damage. Among patients without ECG abnormality, 6 of 33 patients showed cardiac damage (p=0.003. Reduced EF was observed in five patients (8.6% with ECG abnormality and none in patients without ECG abnormality. Conclusions. ECG may aid as the first step for the further examination of cardiac damage in patients with MG.

  16. Effects of combined traditional Chinese medicine with immunosuppressive agents for patients with myasthenia gravis

    Science.gov (United States)

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

    2015-01-01

    Myasthenia gravis (MG) is a kind of autoimmune disease induced by transferring dysfunction of neuromuscular junction. In the present study, we developed an integrated therapy combined with traditional Chinese medicine and immuno suppressive agents to seek for an effective treatment of MG. 220 MG patients were randomly divided into two groups with different therapies. Plasma levels of acetylcholine receptors antibodies (AchRAb) and CD4+CD25+ regulatory T cells (CD4+CD25+Treg) were conducted through ELISA and flow cytometry. The amount of AchRAb (8.52±0.96 vs. 5.22±0.46) and CD4+CD25+Treg (1.94±1.21 vs. 3.21±0.96) in Group A receiving integrated therapy were significantly improved compared with Group B; the clinical performance of group treated with the integrated therapy was also much better. The integrated therapy in the present study could significantly improve the condition of MG with high recovery rate and low recurrence rate, which can be employed in future clinical treatment of MG. PMID:26770531

  17. Structural insights into the molecular mechanisms of myasthenia gravis and their therapeutic implications

    Energy Technology Data Exchange (ETDEWEB)

    Noridomi, Kaori; Watanabe, Go; Hansen, Melissa N.; Han, Gye Won; Chen, Lin (USC)

    2017-04-25

    The nicotinic acetylcholine receptor (nAChR) is a major target of autoantibodies in myasthenia gravis (MG), an autoimmune disease that causes neuromuscular transmission dysfunction. Despite decades of research, the molecular mechanisms underlying MG have not been fully elucidated. Here, we present the crystal structure of the nAChR α1 subunit bound by the Fab fragment of mAb35, a reference monoclonal antibody that causes experimental MG and competes with ~65% of antibodies from MG patients. Our structures reveal for the first time the detailed molecular interactions between MG antibodies and a core region on nAChR α1. These structures suggest a major nAChR-binding mechanism shared by a large number of MG antibodies and the possibility to treat MG by blocking this binding mechanism. Structure-based modeling also provides insights into antibody-mediated nAChR cross-linking known to cause receptor degradation. Our studies establish a structural basis for further mechanistic studies and therapeutic development of MG.

  18. 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.

  19. The usefulness of MRI for detection of the thymus gland in myasthenia gravis

    Energy Technology Data Exchange (ETDEWEB)

    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).

  20. 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)

  1. 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)

  2. 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)

  3. Imaging of thymus in myasthenia gravis: From thymic hyperplasia to thymic tumor

    International Nuclear Information System (INIS)

    Priola, A.M.; Priola, S.M.

    2014-01-01

    Myasthenia gravis (MG) is an autoimmune disorder often associated with thymic abnormalities. At onset, thymic lymphoid hyperplasia (TLH) and thymoma can be found in up to 65% and 15% of patients, respectively. Diagnostic imaging is crucial in this setting in order to detect the presence and type of the thymic abnormality and in the preoperative planning, when indicated. Chest radiography has a minor role due to its low accuracy. Computed tomography is the imaging modality of choice, although the differentiation between a small thymoma and TLH that appears as a focal soft-tissue mass may be not possible. Magnetic resonance imaging (MRI) is not usually employed, but it is useful in equivocal cases, especially in differentiating focal TLH from thymoma by using chemical-shift sequences for defining the proper management. In addition, diffusion-weighted (DW)-MRI can differentiate lipid-poor normal/hyperplastic thymus from thymoma and could be useful in differentiating non-advanced from advanced thymomas. Positron emission tomography (PET)-CT is not helpful in distinguishing early from advanced thymoma but can be used to differentiate thymic carcinoma from thymoma. Hereby, we discuss the imaging features of thymic abnormalities in MG, even focusing on novel aspects of chemical-shift and DW-MRI

  4. Radiographical diagnosis of the thymus in myasthenia gravis. Comparison of pneumomediastinography and computed tomography

    Energy Technology Data Exchange (ETDEWEB)

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

    1987-11-01

    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.

  5. When myasthenia gravis is deemed refractory: clinical signposts and treatment strategies

    Science.gov (United States)

    Mantegazza, Renato; Antozzi, Carlo

    2018-01-01

    The prognosis for patients with myasthenia gravis (MG) has improved significantly over the past half century, including substantial reductions in mortality and morbidity. However, approximately 10% of patients fails to respond adequately to current therapies and are considered treatment refractory, or treatment intolerant, and up to 80% have disease that fails to achieve complete stable remission. Although patients with autoantibodies to muscle-specific tyrosine kinase (anti-MuSK positive) are more likely to become treatment refractory than those with autoantibodies to the acetylcholine receptor (anti-AChR positive), each of these serotypes is substantially represented in the refractory MG population. Other risk factors for becoming treatment refractory include history of thymoma or thymectomy and female sex. A modified treatment algorithm for MG is proposed: patients who have disease that fails to respond to the stepwise approach to therapy, are treatment intolerant, or who require chronic rescue measures despite ongoing therapy, should be considered treatment refractory and emerging therapies should be considered. Three emerging monoclonal antibody-based therapies are discussed: the anti-B-cell agent rituximab; the terminal complement activation inhibitor eculizumab; and belimumab, which targets B-cell activating factor. Increased understanding of molecular pathophysiology and accurate antibody subtyping in MG should lead to the use of new therapeutic agents and successful management of treatment-refractory patients. PMID:29403543

  6. Keep An Eye Out For Myasthenia Gravis Patients With An Eye Out

    Directory of Open Access Journals (Sweden)

    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.

  7. Thymic lesions and myasthenia gravis. Diagnosis based on mediastinal imaging and pathological findings

    Energy Technology Data Exchange (ETDEWEB)

    Pirronti, T.; Rinaldi, P.; Marano, P. [Univ. Cattolica del S. Cuore, Roma (Italy). Inst. of Radiology; Batocchi, A.P.; Evoli, A.; Di Schino, C. [Univ. Cattolica del S. Cuore, Roma (Italy). Inst. of Neurology

    2002-07-01

    Purpose: To achieve a better understanding of the role of CT and MR imaging in the study of the mediastinum in patients with myasthenia gravis (MG). Material and Methods: Mediastinal CT and MR findings were correlated with the histopathological results in 104 thymectomized MG patients. Results: CT was performed in 104 patients; in 11 of them, MR was also carried out. 44 patients had hyperplasia at histology. On CT, thymic hyperplasia was confirmed in 16 cases, thymoma was diagnosed in 10 and a normal thymus in 18 (sensitivity 36%, specificity 95%). Of 52 patients with thymoma at histology, CT showed thymoma in 46, hyperplasia in 1, and normal thymus in 5. CT showed 88.5% sensitivity and 77% specificity for thymoma. In 10 patients with invasive thymoma, CT was indiscriminate, while invasiveness was detected in 7 cases at MR (70% sensitivity) and at CT in 1 case. Both CT and MR detected tumor recurrence in 5 cases, but the exact localization and degree of invasion were best defined by MR. Conclusion: In MG patients CT is a sensitive, specific and efficient modality for detecting thymoma, but is less so for detecting thymic hyperplasia. MR was shown to be accurate in detecting invasive thymoma both preoperatively and in postoperative follow-up.

  8. Visualization of the thymus in myasthenia gravis. Comparison between pneumomediastinography and CT of the anterior mediastinum

    Energy Technology Data Exchange (ETDEWEB)

    Komatsu, Midori; Tanaka, Makoto; Morimatsu, Mitsunori; Hirai, Shunsaku; Heshiki, Atsuko (Gunma Univ., Maebashi (Japan). School of Medicine)

    1982-12-01

    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.

  9. T-cell receptor Vα and Cα alleles associated with multiple sclerosis and myasthenia gravis

    International Nuclear Information System (INIS)

    Oksenberg, J.R.; Cavalli-Sforza, L.L.; Steinman, L.; Sherritt, M.; Bernard, C.C.; Begovich, A.B.; Erlich, H.A.

    1989-01-01

    Polymorphic markers in genes encoding the α chain of the human T-cell receptor (TcR) have been detected by Southern blot analysis in Pss I digests. Polymorphic bands were observed at 6.3 and 2.0 kilobases (kb) with frequencies of 0.30 and 0.44, respectively, in the general population. Using the polymerase chain reaction (PCR) method, the authors amplified selected sequences derived from the full-length TcR α cDNA probe. These PcR products were used as specific probes to demonstrate that the 6.3-kb polymorphic fragment hybridizes to the variable (V)-region probe and the 2.0-kb fragment hybridizes to the constant (C)-region probe. Segregation of the polymorphic bands was analyzed in family studies. To look for associations between these markers and autoimmune diseases, the authors have studied the restriction fragment length polymorphism distribution of the Pss I markers in patients with multiple sclerosis, myasthenia gravis, and Graves disease. Significant differences in the frequency of the polymorphic V α and C α markers were identified between patients and healthy individuals

  10. Factors predicting the outcomes of elderly hospitalized myasthenia gravis patients: a national database study

    Directory of Open Access Journals (Sweden)

    Tiamkao S

    2017-04-01

    Full Text Available Somsak Tiamkao,1,2 Sineenard Pranboon,3 Kaewjai Thepsuthammarat,4 Kittisak Sawanyawisuth1,5,6 1Department of Medicine, Faculty of Medicine, 2The Neuroscience Research and Development Group, 3Nursing Division, Srinagarind Hospital, 4Clinical Epidemiology Unit, Faculty of Medicine, 5Research Center in Back, Neck, Other Joint Pain and Human Performance (BNOJPH, 6Ambulatory Medicine Research Group, Khon Kaen University, Khon Kaen, Thailand Background: Myasthenia gravis (MG in elderly populations is increasing. This study aimed to evaluate predictors for treatment outcomes in elderly hospitalized MG patients using the national database. Methods: We collected data of elderly hospitalized MG patients from the National Health Security Office from October 2009 to September 2010. Predictors for treatment outcomes were examined. Results: During the study period, 1,948 identified MG patients were admitted to hospitals throughout Thailand. Of those, 441 patients (22.64% were aged ≥ 60 years. There were 66 patients (14.97% who had poor outcomes. There were only three significant factors in the final model. Presence of pneumonia, use of mechanical ventilators, and septicemia had adjusted odds ratios (95% confidence interval of 2.83 (1.03, 7.75, 5.33 (2.24, 12.72, and 4.47 (1.86, 10.75, respectively. Conclusion: Pneumonia, being on a mechanical ventilator, and septicemia were independent factors associated with poor treatment outcomes in elderly hospitalized MG patients according to national data. Keywords: pneumonia, ventilator, mortality, predictor 

  11. Rare association of thymoma, myasthenia gravis and sarcoidosis : a case report

    Directory of Open Access Journals (Sweden)

    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.

  12. Presumed Isotretinoin-Induced, Concomitant Autoimmune Thyroid Disease and Ocular Myasthenia Gravis: A Case Report

    Directory of Open Access Journals (Sweden)

    Huseyin Gursoy

    2012-11-01

    Full Text Available Introduction: There are many adverse effects that have been described for isotretinoin. To the best of our knowledge, this is the first report of a possible association of oral isotretinoin intake with autoimmune thyroiditis and ocular myasthenia gravis (OMG. Case Presentation: A 19-year-old Caucasian male, who had used oral isotretinoin for severe acne disease for the previous six months, was referred to our clinic. He had a three-week history of diplopia and variable bilateral ptosis. Physical examination showed moderate periorbital edema and limitations of up- and down-gaze in the left eye. Laboratory findings and thyroid ultrasound were consistent with autoimmune thyroiditis. Antithyroid therapy did not relieve the clinical symptoms. Concomitant OMG was suspected. Variable ptosis and a positive response to oral prednisolone of 40 mg/day and pyridostigmine of 360 mg/day supported the diagnosis of concomitant autoimmune thyroiditis and OMG. Conclusion: Autoimmune disorders may be triggered by oral isotretinoin treatment. Clinicians prescribing isotretinoin should be aware of the possible association between isotretinoin intake and concomitant autoimmune thyroiditis and OMG.

  13. Myasthenia gravis in Ceará, Brazil: clinical and epidemiological aspects

    Directory of Open Access Journals (Sweden)

    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.

  14. Videojuego con Realidad Virtual

    OpenAIRE

    González Mora, César

    2017-01-01

    El objetivo del proyecto es el desarrollo de un videojuego deportivo que utilice realidad mixta. El videojuego se podrá utilizar con dispositivos de tipo cardboard, y utilizará realidad aumentada para la interacción del jugador con el videojuego. En el desarrollo se utilizará el motor Unity para conseguir una aplicación multiplataforma, y la librería Vuforia para implementar realidad mixta.

  15. Sistemas integrados con Arduino

    OpenAIRE

    EL YAKOUTI, MOHAMMED

    2017-01-01

    Design of a robot prototype remotely controllable from Bluetooth using Arduino. Control and testing of sensors and events interacting with Arduino and Bluetooth. Diseño de un prototipo de robot controlable remotamente con Bluetooth utilizando Arduino. Control y verificación de los sensores y eventos que interactúan mediante el Arduino y el Bluetooth. El Yakouti, M. (2017). Sistemas integrados con Arduino. http://hdl.handle.net/10251/89274. TFGM

  16. 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

  17. [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.

  18. Investigando con personas con dificultades de aprendizaje

    Directory of Open Access Journals (Sweden)

    Borja González Luna

    2013-12-01

    Full Text Available El artículo muestra los orígenes de lo que Walmsley (2008 denomina «investigación inclusiva». Para comprender qué se entiende por investigación inclusiva tenemos que remontarnos a los debates epistemológicos sobre las metodologías cuantitativas y cualitativas, acontecidos en la década de los 90, en torno a la revista Disability & Society. A partir de una síntesis de dichos debates, focalizados en el ámbito de la «discapacidad intelectual y del desarrollo», se exponen dos estrategias de colaboración con dicha población: a una aproximación etnográfica (de trabajo grupal, y b una aproximación biográfica (de trabajo individual. A continuación se esboza un posible diseño de trabajo de campo que intenta superar el paradigma cualitativo «clásico» con el objetivo de incluir a dicho colectivo más allá del rol de «sujetos de la investigación». Para finalizar se recoge el debate sobre la accesibilidad de los resultados de la investigación a los participantes en dichas investigaciones, y con ello la necesaria innovación en el ámbito de las «devoluciones» de los resultados, cuando se trata de incluir a personas que presentan limitaciones para la comprensión del lenguaje abstracto oral y/o escrito.

  19. Caesarean section in a patient with Myasthenia Gravis: A bigger challenge for the anesthesiologist than the obstetrician

    Directory of Open Access Journals (Sweden)

    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.

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

    Directory of Open Access Journals (Sweden)

    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.

  1. Giochiamo con i robot

    Directory of Open Access Journals (Sweden)

    Andrea Bonarini

    2009-01-01

    Full Text Available "Giochiamo con i robot" e' un laboratorio interattivo per grandi e piccini realizzato per l'edizione 2007 del Festival della Scienza di Genova. Lungo un percorso che va dalla telerobotica alla robotica evolutiva, il laboratorio sviluppa il tema di dare intelligenza ai robot. Questo percorso, le cui tappe sono le varie installazioni, si conclude nella "bottega" dove e' possibile costruire e programmare i propri robot o smontare e modificare quelli esposti durante il percorso didattico. I visitatori sono coinvolti in attivita' ludiche grazie alle quali possonoentrare in contatto con alcune delle idee potenti della robotica,

  2. disegnare con ... Alberto Pratelli

    Directory of Open Access Journals (Sweden)

    Roberto Mingucci

    2017-12-01

    Full Text Available Con questa breve intervista ad Alberto Pratelli, (non a caso scelto per aprire questa nuova rubrica intendia-mo inaugurare un dialogo con personalità significati-ve del Disegno di Architettura, che consenta riflessioni dedicate alle sue varie dimensioni, oggi più che mai da approfondire. La suggestione a farlo, viene da un’idea di Pablo Rodri-guez Navarro ed abbiamo quindi pensato di avviarla proprio in questo numero, che Pablo ha accettato di curare su un tema a lui particolarmente caro.

  3. Marinospirillum insulare sp. nov., a novel halophilic helical bacterium isolated from kusaya gravy.

    Science.gov (United States)

    Satomi, M; Kimura, B; Hayashi, M; Okuzumi, M; Fujii, T

    2004-01-01

    A novel species that belongs to the genus Marinospirillum is described on the basis of phenotypic characteristics, phylogenetic analysis of 16S rRNA and gyrB gene sequences and DNA-DNA hybridization. Four strains of helical, halophilic, Gram-negative, heterotrophic bacteria were isolated from kusaya gravy, which is fermented brine that is used for the production of traditional dried fish in the Izu Islands of Japan. All of the new isolates were motile by means of bipolar tuft flagella, of small cell size, coccoid-body-forming and aerophilic; it was concluded that they belong to the same bacterial species, based on DNA-DNA hybridization values (>70% DNA relatedness). DNA G+C contents of the new strains were 42-43 mol% and they had isoprenoid quinone Q-8 as the major component. Phylogenetic analysis of 16S rRNA gene sequences indicated that the new isolates were members of the genus Marinospirillum; sequence similarity of the new isolates to Marinospirillum minutulum, Marinospirillum megaterium and Marinospirillum alkaliphilum was 98.5, 98.2 and 95.2%, respectively. Phylogenetic analysis based on the gyrB gene indicated that the new isolates had enough phylogenetic distance from M. minutulum and M. megaterium to be regarded as different species, with 84.7 and 78.7% sequence similarity, respectively. DNA-DNA hybridization showed that the new isolates had <36% DNA relatedness to M. minutulum and M. megaterium, supporting the phylogenetic conclusion. Thus, a novel species is proposed: Marinospirillum insulare sp. nov. (type strain, KT=LMG 21802T=NBRC 100033T).

  4. [Correlation factors of 127 times pre-crisis state in patients with myasthenia gravis].

    Science.gov (United States)

    Ou, C Y; Ran, H; Qiu, L; Huang, Z D; Lin, Z Z; Deng, J; Liu, W B

    2017-10-10

    Objective: To investigate the clinical features of the Pre-Crisis State and analyze the correlated risk factors of Pre-Crisis State of myasthenia crisis. Methods: We included 93 patients with myasthenia gravis (MG) who experienced 127 times Pre-Crisis State between October 2007 and July 2016. Those patients were hospitalized in the MG specialize center, Department of Neurological Science, first Affiliated Hospital of Sun Yat-sen University. The information of the general situation, the clinical manifestations and the blood gas analysis in those patients were collected using our innovated clinical research form. Statistic methods were applied including descriptive analysis, univariate logistic analysis, multivariate correlation logistic analysis, etc. Results: (1)The typical features of MG Pre-Crisis State included: dyspnea (127 times, 100% not requiring intubation or non-invasive ventilation), bulbar-muscle weakness (121 times, 95.28%), the increased blood partial pressure of carbon dioxide (PCO(2)) (94 times, 85.45%), expectoration weakness (99 times, 77.95%), sleep disorders (107 times, 84.25%) and the infection (99 times, 77.95%). The occurrence of dyspnea in combination with bulbar-muscle weakness ( P =0.002) or the increased blood PCO(2) ( P =0.042) often indicated the tendency of crisis. (2) The MG symptoms which were proportion to the occurrence of crisis includes: bulbar-muscle weakness ( P =0.028), fever ( P =0.028), malnutrition ( P =0.066), complications ( P =0.071), excess oropharyngeal secretions ( P =0.005) and the increased blood PCO(2) ( P =0.007). The perioperative period of thymectomy would not increase the risk of crisis. Conclusions: Dyspnea indicates the occurrence of the Pre-Crisis State of MG. In order to significantly reduce the morbidity of myasthenia crisis, the bulbar-muscle weakness, the increased blood PCO(2), expectoration weakness, sleep disorders, infection & fever and excess oropharyngeal secretions should be treated timely.

  5. Negative impact of high cumulative glucocorticoid dose on bone metabolism of patients with myasthenia gravis.

    Science.gov (United States)

    Braz, Nayara Felicidade Tomaz; Rocha, Natalia Pessoa; Vieira, Érica Leandro Marciano; Gomez, Rodrigo Santiago; Barbosa, Izabela Guimarães; Malheiro, Olívio Brito; Kakehasi, Adriana Maria; Teixeira, Antonio Lucio

    2017-08-01

    This current study aimed to evaluate the frequency of low bone mass, osteopenia, and osteoporosis in patients with myasthenia gravis (MG) and to investigate the possible association between bone mineral density (BMD) and plasma levels of bone metabolism markers. Eighty patients with MG and 62 controls BMD were measured in the right femoral neck and lumbar spine by dual-energy X-ray absorptiometry. Plasma concentrations of osteocalcin, osteopontin, osteoprotegerin, tumor necrosis factor (TNF-α), interleukin (IL)-1β, IL-6, dickkopf (DKK-1), sclerostin, insulin, leptin, adrenocorticotropic hormone, parathyroid hormone, and fibroblast growth factor (FGF-23) were analyzed by Luminex®. The mean age of patients was 41.9 years, with 13.5 years of length of illness, and a mean cumulative dose of glucocorticoids 38,123 mg. Patients had significant reduction in BMD of the lumbar, the femoral neck, and in the whole body when compared with controls. Fourteen percent MG patients had osteoporosis at the lumbar spine and 2.5% at the femoral neck. In comparison with controls, patients with MG presented lower levels of osteocalcin, adrenocorticotropic hormone, parathyroid hormone, sclerostin, TNF-α, and DKK-1 and higher levels of FGF-23, leptin, and IL-6. There was a significant negative correlation between cumulative glucocorticoid dose and serum calcium, lumbar spine T-score, femoral neck BMD, T-score, and Z-score. After multivariate analysis, higher TNF-α levels increased the likelihood of presenting low bone mass by 2.62. MG patients under corticotherapy presented low BMD and altered levels of bone markers.

  6. Nivolumab-related myasthenia gravis with myositis and myocarditis in Japan.

    Science.gov (United States)

    Suzuki, Shigeaki; Ishikawa, Nobuhisa; Konoeda, Fumie; Seki, Nobuhiko; Fukushima, Satoshi; Takahashi, Kikuko; Uhara, Hisashi; Hasegawa, Yoshikazu; Inomata, Shinichiro; Otani, Yasushi; Yokota, Kenji; Hirose, Takashi; Tanaka, Ryo; Suzuki, Norihiro; Matsui, Makoto

    2017-09-12

    To report the clinical features of myasthenia gravis (MG) induced by treatment with immune checkpoint inhibitors using 2-year safety databases based on postmarketing surveys in Japan. We studied 10,277 patients with cancer who had received monotherapy with either nivolumab or ipilimumab between September 2014 and August 2016. As the control group, 105 patients with idiopathic MG were used. There were 12 MG cases (0.12%) among 9,869 patients with cancer who had been treated with nivolumab, but none among 408 patients treated with ipilimumab. These 12 patients included 6 men and 6 women with a mean age of 73.5 ± 6.3 years. MG onset occurred in the early phase after nivolumab treatment and rapidly deteriorated. Nivolumab-related MG (nivoMG) included 4 patients with mild involvement and 8 patients with severe involvement. Bulbar symptoms and myasthenic crisis were observed more frequently in nivoMG than idiopathic MG. Ten patients were positive for anti-acetylcholine receptor antibodies. Serum creatine kinase levels were markedly elevated to an average level of 4,799 IU/L. Among the 12 patients with nivoMG, 4 had myositis and 3 had myocarditis, with 1 of these patients having both. Immunosuppressive therapy was effective. Postintervention status showed that pharmacologic remission or minimal manifestations were obtained in 4 patients; however, 2 patients died. Immune-related adverse events triggered by nivolumab impaired the patients' daily living activity. The prompt and correct recognition of MG following treatment with immune checkpoint inhibitors in patients with cancer is important. © 2017 American Academy of Neurology.

  7. Clinical significance of detection of antibodies to fetal and adult acetylcholine receptors in myasthenia gravis

    Institute of Scientific and Technical Information of China (English)

    Qi-Guang Shi; Zhi-Hong Wang; Xiao-Wei Ma; Da-Qi Zhang; Chun-Sheng Yang; Fu-Dong Shi; Li Yang

    2012-01-01

    Objective To evaluate the frequency,distribution and clinical significance of the antibodies to the fetal and/or adult acetylcholine receptor (AChR) in patients with myasthenia gravis (MG).Methods AChR antibodies were detected by cell-based assay in the serum of ocular MG (OMG) (n =90) and generalized MG (GMG) patients (n =110).The fetaltype (2α∶ β∶ γ∶ δ) and adult-type (2α∶ β∶ ε∶ δ) AChR were used as antigens,and their relevance to disease presentation was assessed.Results The overall frequencies of anti-adult and anti-fetal AChR antibodies were similar in all 200 patients examined,with 14 having serum specific to the AChR-γ subunit,and 22 to the AChR-ε subunit.The overall sensitivity when using the fetal and adult AChR antibodies was higher than that when using the fetal AChR antibody only (P =0.015).Compared with OMG patients,the mean age at disease onset and the positive ratio of antibodies to both isoforms of the AChR were significantly higher in patients who subsequently progressed to GMG.Older patients and patients with both anti-fetal and anti-adult AChR antibodies had a greater risk for developing generalized disease [odds ratio (OR),1.03;95% confidence interval (CI),1.01-1.06 and OR,5.09;95% CI,2.23-11.62].Conclusion Using both fetal-and adulttype AChRs as the antigens may be more sensitive than using either subtype.Patients with serum specific to both isoforms are at a greater risk of progressing to GMG.Patients with disease onset at an advanced age appear to have a higher frequency of GMG conversion.

  8. Coexistent Autoimmune Autonomic Ganglionopathy and Myasthenia Gravis Associated with Non-Small Cell Lung Cancer

    Science.gov (United States)

    Peltier, Amanda C.; Black, Bonnie K.; Raj, Satish R.; Donofrio, Peter; Robertson, David; Biaggioni, Italo

    2014-01-01

    We report a case of a 55 year old man with non-small cell lung cancer who underwent radiation, chemotherapy with carbotaxol and paclitaxel, and left upper lobe removal two years prior to evaluation. He was referred for disabling orthostatic hypotension (113/69 supine, 66/47 mmHg standing after 10 minutes without a compensatory heart rate increase (57 to 59 bpm), fatigue, and constipation with episodes of ileus. Clinical examination showed mild ptosis bilaterally, fatiguable neck flexor weakness and hip flexor weakness. Blood pressure response to Valsalva maneuver was abnormal with absence of phase 4 overshoot and a Valsalva heart rate ratio of 1.04, The plasma norepinephrine level was low (79 pg/ml supine to 330 pg/ml standing). Single fiber EMG of the right extensor digitorum communis revealed normal mean MCD (jitter) but several pairs exceeded a jitter of 100 µs. Antibodies against muscle acetylcholine receptor [(AChR) 0.66 nmol/L, normal <0.02] and ganglionic AChR (0.34 nmol/L, normal <0.02) were present. Treatment with plasma exchange normalized responses to standing posture (105/68 supine to 118/82 mmHg standing, 66 to 79 bpm), to Valsalva (normal blood pressure overshoot, HR ratio 1.47), norepinephrine (194 pg/ml supine, 763 standing), and jitter measurements. We conclude that autoimmune autonomic ganglionopathy and myasthenia gravis can coexist and suggest that the latter should be excluded in patients with autoimmune autonomic ganglionopathy who complain of fatigue that is improved with non-supine rest. PMID:19882640

  9. Epidemiological study of myasthenia gravis in the province of Reggio Emilia, Italy.

    Science.gov (United States)

    Guidetti, D; Sabadini, R; Bondavalli, M; Cavalletti, S; Lodesani, M; Mantegazza, R; Cosi, V; Solime, F

    1998-06-01

    We carried out a retrospective incidence, prevalence and mortality survey of myasthenia gravis in the province of Reggio Emilia in Northern Italy. Based on 49 patients, the mean incidence per year for the period 1980 through 1994 was 7.8 per 1,000,000. On 31 December 1994 the prevalence rate was 117.5 per 1,000,000 for all patients, either active or recovered (50 cases in a population of 427,493) and 103.4 per 1,000,000 for the active disease. In the 15-year period 1980-1994 the average mortality rate was 1.0 per 1,000,000 per year. The average age at onset was 44.6 +/- 21.0, and the average age at the time of prevalence determination was 51.1 +/- 19.6 for the active disease. At the time of diagnosis, 21 patients (36.8%) were classed in group I according to Osserman's criteria, 31 in group II (54.4%), (19 in group II-A and 12 in group II-B), and the other 5 (8.8%) in group III. Of all the prevalence cases, 6 (12%) were in remission without therapy and 6 with therapy, while most of the others 16 (32%) were classed in group I, 15 (30%) in group II, and 1 (2%) in group III. Thymectomy was performed in 20 patients (35.1%), 12 (21%) had thymoma (malignant in 4 cases), 6 had thymic hyperplasia while in two patients thymic histology was normal. The relation the grade of Osserman's scale at the time of incidence and the presence of thymoma were significant. Higher grades of Osserman's scale were associated were malignant thymoma. Furthermore the relationship between thymectomy and the grade of Osserman's scale at the date of prevalence was significant for the presence of lower grades of Osserman's scale in the patients submitted to thymectomy.

  10. [Development of patient-reported outcome scale for myasthenia gravis: a psychometric test].

    Science.gov (United States)

    Chen, Xin-lin; Liu, Feng-bin; Guo, Li; Liu, Xiao-bin

    2010-02-01

    To investigate the scientificity of patient-reported outcome (PRO) scale for myasthenia gravis (MG), which was used to evaluate the clinical effects of traditional Chinese and Western medicine treatment on MG patients. Psychometric performance of the MG-PRO scale was also expected to be evaluated in this study. A total of 100 MG patients and 100 healthy people were face-to-face interviewed by well-trained investigators, and the data of MG-PRO scale were collected. The classical theory test (CTT) and item response theory (IRT) methods were used to analyze the psychometric performance such as validity, reliability, person separation index (PSI) and differential item functioning (DIF) in the MG-PRO scale. The results of CTT analysis showed that the split-half reliabilities of the MG-PRO scale and each dimension were greater than 0.7. In the analysis of internal consistency of each dimension, the Cronbach's alpha was greater than 0.8. Each facet had greater correlation with its dimension than the other dimensions. Four principal components were extracted by exploratory factor analysis, which represented all dimensions of the scale, and the cumulative variance was 55.54%. The scores of each of the 8 facets between MG patients and healthy people were different (Pdefinition and connotation of quality of life and contains special issues of MG patients as well, and shows good reliability (split-half reliability, Cronbach's alpha), validity (content validity, construct validity, discriminate validity) from the results of CTT, and good psychometric performance from the results of IRT.

  11. Validity of Forced Eyelid Closure Test: A Novel Clinical Screening Test for Ocular Myasthenia Gravis.

    Science.gov (United States)

    Apinyawasisuk, Supanut; Zhou, Xinkai; Tian, Jack J; Garcia, Giancarlo A; Karanjia, Rustum; Sadun, Alfredo A

    2017-09-01

    Forced eyelid closure test (FECT) is a clinical screening test developed from the original Cogan lid twitch (CLT) sign to assist in the diagnosis of ocular myasthenia gravis (OMG), We evaluated the sensitivity and specificity of FECT compared with CLT and benchmarked to standard diagnostic tests. This study was a retrospective chart review of 48 patients using electronic medical records of those that presented with ptosis and/or diplopia at Doheny Eye Institute, University of California, Los Angeles between February 2015 and April 2016. Patients without FECT testing were excluded. FECT and CLT results, and final diagnosis were recorded. To perform FECT, the patient was asked to squeeze his or her eyelids shut for 5-10 seconds then open quickly and fixate in primary position. The excessive upward overshoot of eyelids movement indicated a positive FECT. The test was performed by a neuro-ophthalmologist before establishing the diagnosis. Patients who had equivocal test results and/or inconclusive final diagnosis were excluded. Of the 48 patients studied, 18 patients (37.5%) had positive FECT; 15 of whom had a final diagnosis of OMG (83.3%). Of the 30 patients with negative FECT, 1 had OMG (3.3%). Of the 48 patients, 35 patients also had a documented CLT result (72.9%). CLT was positive in 11 of these 35 patients (31.4%), and 9 of these 11 had OMG (81.8%). Of the 24 patients with negative CLT, 2 of them had OMG (8.3%). Sensitivity and specificity of FECT were 94% and 91% (joint 95% confidence region: sensitivity × specificity = [0.70, 1] × [0.75, 1]). The relative true-positive fraction (rTPF) between FECT and CLT was 1.15; the relative false-positive fraction was 1.31. FECT is a simple clinical screening test with good sensitivity and specificity for OMG.

  12. Characterization of CD4 and CD8 T Cell Responses in MuSK Myasthenia Gravis

    Science.gov (United States)

    Yi, JS; Guidon, A; Sparks, S; Osborne, R; Juel, VC; Massey, JM; Sanders, DB; Weinhold, KJ; Guptill, JT

    2014-01-01

    Muscle specific tyrosine kinase myasthenia gravis (MuSK MG) is a form of autoimmune MG that predominantly affects women and has unique clinical features, including prominent bulbar weakness, muscle atrophy, and excellent response to therapeutic plasma exchange. Patients with MuSK MG have predominantly IgG4 autoantibodies directed against MuSK on the postsynaptic muscle membrane. Lymphocyte functionality has not been reported in this condition. The goal of this study was to characterize T-cell responses in patients with MuSK MG. Intracellular production of IFN-gamma, TNF-alpha, IL-2, IL-17, and IL-21 by CD4+ and CD8+ T-cells was measured by polychromatic flow cytometry in peripheral blood samples from 11 Musk MG patients and 10 healthy controls. Only one MuSK MG patient was not receiving immunosuppressive therapy. Regulatory T-cells (Treg) were also included in our analysis to determine if changes in T cell function were due to altered Treg frequencies. CD8+ T-cells from MuSK MG patients had higher frequencies of polyfunctional responses than controls, and CD4+ T-cells had higher IL-2, TNF-alpha, and IL-17. MuSK MG patients had a higher percentage of CD4+ T-cells producing combinations of IFN-gamma/IL-2/TNF-gamma, TNF-alpha/IL-2, and IFN-gamma/TNF-alpha. Interestingly, Treg numbers and CD39 expression were not different from control values. MuSK MG patients had increased frequencies of Th1 and Th17 cytokines and were primed for polyfunctional proinflammatory responses that cannot be explained by a defect in Treg function or number. PMID:24378287

  13. Evaluation of the quality of guidelines for myasthenia gravis with the AGREE II instrument.

    Directory of Open Access Journals (Sweden)

    Zhenchang Zhang

    Full Text Available Clinical practice guidelines (CPGs are systematically developed statements to assist practitioners in making decisions about appropriate healthcare in specific clinical circumstances. The methodological quality of CPGs for myasthenia gravis (MG are unclear.To critically evaluate the methodological quality of CPGs for MG using AGREE II instrument.A systematical search strategy on PubMed, EMBASE, DynaMed, the National Guideline Clearinghouse (NGC and the Chinese Biomedical Literature database (CBM was performed on September 20th 2013. All guidelines related to MG were evaluated with AGREE II. The software used for analysis was SPSS 17.0.A total of 15 CPGs for MG met the inclusion criteria (12 CPGs in English, 3 CPGs in Chinese. The overall agreement among reviews was moderate or high (ICC >0.70. The mean scores (mean ± SD for al six domains were presented as follows: scope and purpose (60.93% ± 16.62%, stakeholder involvement (40.93% ± 20.04%, rigor of development (37.22% ± 30.46%, clarity of presentation (64.26% ± 16.36%, applicability (28.19% ± 20.56% and editorial independence (27.78% ± 28.28%. Compared with non-evidence-based CPGs, evidence-based CPGs had statistically significant higher quality scores for all AGREE II domains (P0.05. The quality scores of CPGs developed by NGC/AAN were higher than the quality scores of CPGs developed by other organizations for all domains. The difference was statistically significant for all domains with the exception of clarity of presentation (P = 0.07.The qualities of CPGs on MG were generally acceptable with several flaws. The AGREE II instrument should be adopted by guideline developers, particularly in China.

  14. Myasthenia gravis: descriptive analysis of life-threatening events in a recent nationwide registry.

    Science.gov (United States)

    Ramos-Fransi, A; Rojas-García, R; Segovia, S; Márquez-Infante, C; Pardo, J; Coll-Cantí, J; Jericó, I; Illa, I

    2015-07-01

    Myasthenia gravis (MG) may become life-threatening if patients have respiratory insufficiency or dysphagia. This study aimed to determine the incidence, demographic characteristics, risk factors, response to treatment and outcome of these life-threatening events (LTEs) in a recent, population-based sample of MG patients. A retrospective analysis of MG patients who presented with an LTE between 2000 and 2013 was performed. Participants were identified from a neuromuscular diseases registry in Spain that includes 648 patients with MG (NMD-ES). Sixty-two (9.56%) patients had an LTE. Thirty-two were classified as class V according to the MG Foundation of America, and 30 as class IVB. Fifty per cent were previously diagnosed with MG and median duration of the disease before the LTE was 24 months (3-406). The most common related factor was infection (n = 18). All patients received intravenous human immunoglobulin; 11 had a second infusion and six had plasma exchange. Median time to feeding tube removal was 13 days (1-434). Median time to weaning from ventilation was 12 days (3-176), and it was significantly shorter in late onset MG (≥50 years) (P = 0.019). LTEs improved <2 weeks in 55.8% but did not improve until after 1 month in 20% of patients. Four patients died. No other factors influenced mortality or duration of LTEs. The percentage of LTEs in MG patients was low, particularly amongst those previously diagnosed and treated for the disease. The significant percentage of treatment-resistant LTEs indicates that more effective treatment approaches are needed for this vulnerable sub-population. © 2015 EAN.

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

    Directory of Open Access Journals (Sweden)

    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.

  16. 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...

  17. 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.

  18. 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.

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

    Directory of Open Access Journals (Sweden)

    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.

  20. 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.

  1. Caspase-1 inhibitor regulates humoral responses in experimental autoimmune myasthenia gravis via IL-6- dependent inhibiton of STAT3.

    Science.gov (United States)

    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.

  2. Conversando con Oriol Bohigas

    OpenAIRE

    Redondo Domínguez, Ernesto; Moya Sala, Joaquim

    2015-01-01

    [EN] Interview with Oriol Bohigas [ES] Entrevista con Oriol Bohigas Redondo Domínguez, E.; Moya Sala, J. (2015). Conversando con… Oriol Bohigas. EGA. Revista de Expresión Gráfica Arquitectónica. 20(26):22-35. doi:10.4995/ega.2015.4061 22 35 20 26

  3. DR Con o:

    African Journals Online (AJOL)

    which could fall under the Ugandan influence. The con-. flict in the ..... The Congolese people and international community within SADC, the AU ..... ments and make peace among themselves. However, one ... friends overnight.There is a great ...

  4. fertilizada con diferentes abonos

    Directory of Open Access Journals (Sweden)

    Jorge Alberto Elizondo-Salazar

    2007-01-01

    Full Text Available Producción y calidad de la biomasa de morera (Morus alba fertilizada con diferentes abonos. Se llevó a cabo un experimento en la Estación Experimental “Alfredo Volio Mata” de la Universidad de Costa Rica con el fi n de evaluar la aplicación de 150 kg de N/ha/año proveniente de dos abonos orgánicos: lombriabono y compostaje; y de un fertilizante químico, sobre la producción y calidad de la biomasa de morera. El periodo experimental comprendió un ciclo de 12 meses, iniciando en julio del 2003 y fi nalizando en julio del 2004. Se utilizó una plantación de morera de 12 años de establecida con una densidad de siembra de 27.777 plantas/ ha. Se empleó un diseño de bloques completos al azar con cuatro tratamientos: dos abonos orgánicos, nitrato de amonio (33,5% N y un control. Las plantas se podaron a 0,6 m sobre el nivel del suelo al inicio del ensayo. Durante el periodo experimental, las plantas fueron podadas consecutivamente cada 90 días. Las hojas y los tallos fueron separados y analizados para determinar el contenido de materia seca y proteína cruda. La producción de materia seca fue 23% superior y el contenido de proteína cruda fue signifi cativamente mayor con el nitrógeno químico, mientras que el contenido de materia seca fue menor. No se encontraron diferencias signifi cativas entre el tratamiento control y los tratamientos orgánicos.

  5. 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.

  6. 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

  7. Anticuerpos anti 21 hidroxilasa séricos en pacientes con anticuerpos antifracción microsomal: Síndrome poliendocrino autoinmune Seric 21- hydroxilase antibodies in patients with anti-microsomal fraction antibodies: Autoimmune polyendocrine syndrome

    Directory of Open Access Journals (Sweden)

    Silvia Botta

    2007-04-01

    Full Text Available El síndrome poliendocrino autoinmune (SPA es la asociación de enfermedades endocrinas autoinmunes con otros desórdenes autoinmunes no endocrinos. Los tipos 1, 2 y 4 presentan adrenalitis autoinmune, esto indica la presencia de autoanticuerpos, y su marcador serológico específico es el anti 21 hidroxilasa (a21-OH. El SPA tipo 2 es la asociación de adrenalitis, enfermedad tiroidea y/o diabetes mellitus inducidas por autoanticuerpos. Como componentes menores, pueden estar asociados entre otros, vitiligo, alopecia y miastenia. Nuestros objetivos fueron: establecer la prevalencia de a21-OH séricos en pacientes con anticuerpos anti fracción microsomal (AFM positivos, enfermedad tiroidea autoinmune y/o afecciones endocrinas y no endocrinas autoinmunes; diagnosticar formas incompletas de SPA y estudiar individuos con probable riesgo de progresión a un SPA completo. Estudiamos 72 pacientes AFM positivos y 60 sujetos tomados como grupo control, AFM negativos. Hallamos a21-OH elevados en dos pacientes: A= 47 U/ml, hipotiroidismo autoinmune y miastenia; y B= 8.75 U/ml, hipotiroidismo autoinmune y vitiligo; ambos con ausencia de insuficiencia adrenal. La prevalencia de a21-OH encontrada fue del 2.8%. Las pacientes A y B corresponden a un SPA tipo 2 incompleto y latente en relación al componente adrenal. Considerando a los a21-OH marcadores de enfermedad autoinmune latente, el eventual riesgo de evolución hacia la afección clínica sugiere la necesidad de estrechos controles clínicos y bioquímicos periódicos.Autoimmune polyendocrine syndrome (APS is the association of autoimmune endocrine diseases, with other autoimmune nonendocrine disorders. APS types 1, 2 and 4 include autoimmune adrenalitis; this suggests the presence of autoantibodies. A specific serological marker for these is the anti 21- hydroxilase autoantibody (a21-OH. APS type 2 is the association of autoimmune adrenalitis, to autoimmune thyroid disease and/or diabetes mellitus, all

  8. pacientes con falla cardiaca

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    Diana Marcela Achury Saldaña

    2007-01-01

    Full Text Available Objetivo: determinar la adherencia al tratamiento de pacientes con falla cardiaca hospitalizados, al aplicar un plan educativo quefomenta el autocuidado.Método: estudio cuasiexperimental (entrevistas enfermera-paciente realizado entre diciembre de 2004 y mayo de 2006, con unamuestra de 50 pacientes seleccionados por conveniencia. Se diseñó un instrumento para evaluar los comportamientos de los pacientes,con base en algunos resultados de la adherencia y sus respectivos indicadores de la taxonomía NOC (Nursing out comes classification. Laadherencia al tratamiento fue medida en dos momentos: el primero durante la hospitalización, seguido de la aplicación del plan educativoantes del alta, que proporcionaba información en el manejo de su enfermedad desde una dimensión física, psicológica y social quepromueve el autocuidado; y el segundo un mes después del alta en su domicilio.Resultados: diferencias estadísticamente significativas (P=0,0001 que demuestran cómo mediante la capacitación al paciente enel manejo de su tratamiento farmacológico y no farmacológico, el establecimiento de una sana relación entre el profesional de enfermeríay el paciente, y la participación de la familia, se logra una total adherencia al tratamiento.Conclusiones: para lograr una adherencia total del paciente con falla cardiaca al tratamiento es necesario un proceso educativo y unseguimiento continuo y personalizado que motive permanentemente al paciente y se le reconozca el papel protagónico en su cuidado y manejo de la enfermedad.

  9. A Case of Myasthenia Gravis Presenting Solely With Bulbar Palsy Not Associated With Easy Fatigability.

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    Pinank R. Mer

    2015-12-01

    Full Text Available Introduction: Myesthenia Gravis (MG is a neuromuscular disorder characterized by weakness and fatigability of skeletal muscles. The underlying defect is a decrease in number of available acetylcholine receptors (AChRs at neuromuscular junction due to an antibody-mediated autoimmune attack. Case Report: A 26 year old male patient from Dhar (M.P., a tribal district, presented to civil hospital Ahmedabad (CHA with complaints of unable to drink water since 5 months. Patient complained of water coming out from nose. Gradually he developed inability to eat also, with no evidence of vomiting.2 months later patient developed slurring of speech which was followed by total inability to speak. Along with that he developed diplopia on binocular vision. No such symptoms in siblings were there. On examination general examination was normal. On CNS examination he was conscious oriented with normal planter and deep tendon reflexes. On cranial nerve examination 3, 7, 9, 10th nerve palsies seen in the form of absence of medial & upward movement of left eye (3rd, inability to close eyes completely (3rd, reduced power and tone of buccinators muscle (7th, gag reflex absent (9th, presence of dysphagia (10th. Rest of the CNS findings were normal. He was given ryle’s tube feeding and investigated.CBC, LFT, RFT, urine routine-micro were normal.CSF examination showed mild increase of protein.MRI brain was normal. Vitamin B12, TSH, Calcium, HbA1c, ESR, RA factor were normal.ANA was (+ with nuclear speckled pattern. Fundus examination was normal with abnormal ocular movement as described.VEP study was normal.EMG NCV showed reduced amplitude of bilateral facial nerve, involvement of right oculomotor nerve suggesting bulbar myasthenia >generalized myasthenia. Then we did AChR antibody test which came highly positive. He was started T.Pyridostigmine 60mg TDS and T. Prednisolone 5mg/kg and discharged. On follow up we found marked improvement of symptoms. Conclusion: The

  10. Social disadvantages associated with myasthenia gravis and its treatment: a multicentre cross-sectional study.

    Science.gov (United States)

    Nagane, Yuriko; Murai, Hiroyuki; Imai, Tomihiro; Yamamoto, Daisuke; Tsuda, Emiko; Minami, Naoya; Suzuki, Yasushi; Kanai, Tetsuya; Uzawa, Akiyuki; Kawaguchi, Naoki; Masuda, Masayuki; Konno, Shingo; Suzuki, Hidekazu; Aoki, Masashi; Utsugisawa, Kimiaki

    2017-02-23

    To clarify the social disadvantages associated with myasthenia gravis (MG) and examine associations with its disease and treatment. Cross-sectional study. We evaluated 917 consecutive cases of established MG seen at 13 neurological centres in Japan over a short duration. All patients completed a questionnaire on social disadvantages resulting from MG and its treatment and a 15-item MG-specific quality of life scale at study entry. Clinical severity at the worst condition was graded according to the MG Foundation of America classification, and that at the current condition was determined according to the quantitative MG score and MG composite. Maximum dose and duration of dose ≥20 mg/day of oral prednisolone during the disease course were obtained from the patients' medical records. Achievement of the treatment target (minimal manifestation status with prednisolone at ≤5 mg/day) was determined at 1, 2 and 4 years after starting treatment and at study entry. We found that 27.2% of the patients had experienced unemployment, 4.1% had been unwillingly transferred and 35.9% had experienced a decrease in income, 47.1% of whom reported that the decrease was ≥50% of their previous total income. In addition, 49.0% of the patients reported feeling reduced social positivity. Factors promoting social disadvantages were severity of illness, dose and duration of prednisolone, long-term treatment, and a depressive state and change in appearance after treatment with oral steroids. Early achievement of the treatment target was a major inhibiting factor. Patients with MG often experience unemployment, unwilling job transfers and a decrease in income. In addition, many patients report feeling reduced social positivity. To inhibit the social disadvantages associated with MG and its treatment, greater focus needs to be placed on helping patients with MG resume a normal lifestyle as soon as possible by achieving the treatment target. Published by the BMJ Publishing Group

  11. MRI manifestations of thymus in myasthenia gravis (MG) patients in various age groups

    International Nuclear Information System (INIS)

    Wang Ying; Peng Xi; Li Zhizhao; Jiang Kuiming; Song Ting; Dong Tianfa; Xiao Youcheng

    2003-01-01

    Objective: To study MRI findings of the thymus in patients with myasthenia gravis (MG) in different age groups and to analyze the relationship between the morphological changes of thymus and the MG. Methods: In total 90 patients with MG (male: female=43:47) received MR scan and were divided into four groups (group A, B, C and D) by age. Fourteen patients out of 90 received additional enhanced scan. Group A included 33 patients aged under 10 years (m:f=18:15); 27 patients aged 11-25 years were in group B (m:f=12:15); group C had 17 patients aged 26-50 years (m:f=6:11); and in group D there were 13 patients whose ages were over 51 years (m:f=7:6). And 30 Non-MG patients aged 8-75 years were selected as control group, in which the thickness, the fat collection, and glandulous atrophy of thymus was studied on CT. Results: 1) The thymus was unremarkable in 44 cases out of 90 (48.88%). 2) Enlarged thymus was shown in 42 cases out of 90 (46.66%), in which non-nodular enlargement was revealed in 34 cases and nodular enlargement in 8 cases. There were 27 cases with abnormality of thymus out of 33 (81.81%) in group A, 12 cases out of 27 (44.44%) in group B and 3 cases in group C, but no abnormality was found in group D. 3) Only 4/90 patients (4.44%) had thymic mass that respectively seen in one case of group B, two of group C and one of group D. No evidence of the involvement of the adjacent structure was found on MRI in the cases of thymic mass. No thymus enlargement was revealed in control group. Fat collection in thymus was seen in both study groups and control group. Conclusion: Intimate relationship between the abnormality of the thymus gland and MG exists in children and teenagers. While in the middle-aged patients or the seniors, further studies should be made to find out whether there is a correlation

  12. Shenqi Fuzheng Injection Alleviates the Transient Worsening Caused by Steroids Pulse Therapy in Treating Myasthenia Gravis

    Science.gov (United States)

    Qi, Guo-Yan; Liu, Peng

    2013-01-01

    Purpose. To evaluate the treatment effect and side effect of Shenqi Fuzheng Injection (SFI) on alleviating transient worsening of myasthenia gravis (MG) symptoms caused by high-dose steroids pulse therapy. Methods. Sixty-six consecutive patients with MG were randomly divided into two groups: the treatment group treated with SFI and methylprednisolone pulse therapy (MPT) and the control group treated with MPT alone. The severity of MG before, during, and after MPT and the duration of transient worsening (TW) were evaluated and compared with the clinical absolute scoring (AS) and relative scoring (RS) system. Results. Twenty-nine patients experienced TW in each group. At TW, the AS was significantly increased (P < 0.000) in both groups compared with baseline data, with the AS increase in the treatment group (16.8 ± 2) significantly smaller (P < 0.05) than in the control group (24.9 ± 2.5). At the end of the treatment course, the AS for the treatment group was significantly decreased (7.5 ± 0.9) compared with at TW, although no significant difference compared with the control (9.7 ± 1.1). The TW lasted 1–6 days (mean 3.7) for the treatment group, significantly shorter (P < 0.05) than 2–12 days (mean 7.8) for the control. The RS for the treatment group at the end of treatment was 43.8%–100% (mean 76.8% ± 2.6%), significantly better than the control group: 33.3%–100% (mean 67.2 ± 3.6%). Slight side effects (18.75%) included maldigestion and rash in the treatment group. Conclusion. SFI has a better treatment effect and few side effects and can alleviate the severity and shorten the duration of the transient worsening of MG during steroids pulse therapy. PMID:24348721

  13. Frequency of myasthenic crisis in relation to thymectomy in generalized myasthenia gravis: A 17-year experience

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    Shalmani Hamidreza

    2004-09-01

    Full Text Available Abstract Background Myasthenic crisis is the most serious life-threatening event in generalized myasthenia gravis (MG patients. The objective of this study was to assess the long-term impact of thymectomy on rate and severity of these attacks in Iranian patients. Methods We reviewed the clinical records from 272 myasthenic patients diagnosed and treated in our neurology clinic during 1985 to 2002. Fifty-three patients were excluded because of unconfirmed diagnosis, ocular form of MG, contraindication to surgery, concomitant diseases and loss to follow-up. The Osserman classification was used to assess the initial severity of the disease. Frequency and severity of the attacks were compared between two groups with appropriate statistical tests according to the nature of variables. Multivariate logistic regression analysis was used to assess the predictors of myasthenic crisis in the group of patients without thymoma. Results 110 patients were in thymectomy group and the other 109 patients were on medical therapy. These two groups had no significant differences with respect to age at onset, gender, Osserman score in baseline and follow up period. 62 patients (28.3% of all 219 patients had reported 89 attacks of myasthenic crisis. 20 patients of 62 (32% were in thymectomy group and 42 (68% were in the other group. There was significant difference between the two groups in number of patients with crisis (P = 0.001; odds ratio = 2.8 with 95% CI of 1.5 to 5.2. In addition, these attacks were more severe in group of non-thymectomized patients as the duration of ICU admission was longer and they needed more ventilatory support during their attacks. Regression model showed thymectomy and lower age at onset as two predictors of decrement in myasthenic crisis rate in non-thymomatous MG patients. Conclusions It is suggested that frequency and severity of myasthenic attacks as important endpoints in evaluation of MG patients. Thymectomy seems to have a

  14. Detection and clinical significance of serum autoantibodies in patients with myasthenia gravis

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    Yun LIU

    2016-10-01

    Full Text Available Objective To investigate the expressions and clinical significance of serum anti - acetylcholine receptor antibodies (AChR-Ab, anti-Titin antibodies (Titin-Ab, and anti-Ryanodine receptor antibodies (RyR-Ab in patients with myasthenia gravis (MG. Methods Serum AChR-Ab, Titin-Ab and RyR-Ab were detected with enzyme-linked immunosorbent assay (ELISA in 182 MG patients, 105 patients of other neurological diseases (OND and 62 normal controls. Results Serum AChR-Ab, Titin-Ab and RyR-Ab were detected positive respectively in 68.13% (124/182, 64.29% (117/182 and 67.03% (122/182 of patients in MG group. The positive rates of those antibodies in MG group were significantly higher than those in OND group (P = 0.000, for all and control group (P = 0.000, for all. When 3 antibodies coexisted, the sensitivity in the diagnosis of MG was 41.21%, with 99.40% of specificity. There was no significant difference in the positive rate of serum AChR-Ab, Titin-Ab and RyR-Ab between early-onset MG subgroup and late-onset MG subgroup (P > 0.05, for all. The positive rates of AChR-Ab were significantly higher in MG patients with thymoma than in those without thymoma (P = 0.004. There was no significant difference in the positive rate of Titin-Ab and RyR-Ab between MG with thymoma subgroup and MG without thymoma subgroup (P > 0.05, for all. The prevalence of AChR-Ab in generalized MG (GMG atients (Ⅱa and Ⅱb was higher than those in ocular MG (OMG patients (typeⅠ; P = 0.005, 0.012. There was no significant difference in the positive rate of Titin-Ab and RyR-Ab between GMG subgroup and OMG subgroup (P > 0.05, for all. Conclusions Serum AChR-Ab, Titin-Ab and RyR-Ab can be used as ndicators of the diagnosis of MG. Patients with 3 coexisted positive antibodies are highly suspected as MG. Higher AChR-Ab level in serum of OMG patients indicates the possibility of progressing to GMG. DOI: 10.3969/j.issn.1672-6731.2016.10.007

  15. Cementos con cenizas volantes

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    Ossa M., Mauricio

    1984-03-01

    additions of 20 and 30% .

    Casi la generalidad de los estudios realizados sobre cementos con adición de cenizas volantes se refieren a sus características y comportamiento en pastas, morteros y hormigones, siempre en relación con aquéllos del cemento portland. Esta vez, se desarrolló un trabajo experimental orientado a relacionar entre sí los cementos con adiciones de cenizas volantes y de puzolana natural. Para ello se fabricaron a escala de laboratorio cementos de ambos tipos, empleando como materias primas comunes clinker y yeso y, como variables, diferentes porcentajes de las dos adiciones, que cumplieron previamente los requisitos normalizados en cuanto a sus actividades puzolánicas. La calidad de los cementos fabricados resultó adecuada y concordante con la del cemento portland-puzolánico obtenido a escala industrial con los mismos clinker, yeso y puzolana natural de este estudio. Posteriormente, se determinaron las características de los cementos experimentales y se confeccionaron morteros normales para la realización de ensayos físicos y mecánicos. Los resultados de ensayos indicaron que los cementos con adición de cenizas volantes (CCV requieren menos agua para consistencia normal, presentan tiempos de fraguado mayores y expansiones en autoclave menores que los cementos con adición de puzolana (CP. Los calores de hidratación a 7 y 28 días de edad fueron aproximadamente similares para ambos tipos de cemento. En morteros normales, los cementos CCV mostraron menor retracción de secado, mayor retentividad y mayor fluidez (para igual cantidad de agua que los cementos CP. En los ensayos de exudación se observó que ésta depende más de la finura que el tipo de adición. Finalmente, los ensayos mecánicos señalaron que las resistencias a compresión y flexotracción de los morteros con cementos CCV son menores a edades inferiores que 14 días (del orden de 5 a 10% a un día de edad, pero que a partir de entonces pasan a ser mayores que las de

  16. Correlation of single-breath count test and neck flexor muscle strength with spirometry in myasthenia gravis.

    Science.gov (United States)

    Elsheikh, Bakri; Arnold, W David; Gharibshahi, Shahram; Reynolds, Jerold; Freimer, Miriam; Kissel, John T

    2016-01-01

    Although formal spirometry is the gold standard for monitoring respiratory function in patients with myasthenia gravis (MG), such testing is often delayed or unavailable. There is a need for a simple bedside test that can accurately measure respiratory function. We conducted a prospective, cross-sectional, single-blind study in adults with acetylcholine receptor antibody positive MG. Participants performed the single breath count test (SBCT) and underwent manual muscle strength testing, and a respiratory therapist performed spirometry blinded to SBCT and strength results. Thirty-one patients, aged 57 ± 19 years participated. SBCT showed significant correlations with forced vital capacity (FVC), negative inspiratory force, and neck flexor strength (P strength (P = 0.02) but no correlation with shoulder abductor strength. These data suggest that the SBCT and neck flexor strength testing are valuable tools for bedside assessment of respiratory function in MG patients. © 2015 Wiley Periodicals, Inc.

  17. Myasthenia gravis and thymus: long-term follow-up screening of thymectomized and non-thymectomized patients

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    Paulo Jose Lorenzoni

    2013-07-01

    Full Text Available Thymoma screening is recommended at the onset of myasthenia gravis (MG or when patients with MG present with clinical deterioration or a progressive increase of anti-acetylcholine receptor antibody. However, it is unknown if it is necessary to repeat the screening of thymoma at fixed intervals, even in the absence of MG deterioration, when the initial screening is negative. We analyzed the recurrence rate and incidence of new thymoma in a series of patients with well-controlled MG. The sample consisted of 53 patients, aged 17 to 72 years, and the follow-up varied between 75 and 472 months. The chest computerized tomography detected thymus abnormalities in eight patients at the initial screening and no abnormalities in all patients at a second screening after five years. The findings of this study support the classical opinion that screening for thymoma should be recommended only if there is clinical deterioration due to the disease.

  18. competencia con China

    Directory of Open Access Journals (Sweden)

    Elena de la Paz Hernández Águila

    2007-01-01

    Full Text Available El artículo ofrece un diagnóstico del desempeño de la industria mexicana del calzado desde la década de los ochenta hasta la actualidad. Analiza la problemática que ha enfrentado esta rama industrial a partir del proceso de apertura comercial y de la competencia en su mercado interno con productos provenientes de países asiáticos, particularmente China. Problematiza al respecto los retos y las perspectivas que a mediano plazo enfrentará este sector empresarial y sobre las posibilidades de competir en el mercado globalizado.

  19. Construir con Madera

    OpenAIRE

    Olabe-Velasco, F. (Fermín); Val-Hernández, Y. (Yolanda); Varela-de-la-Cruz, P. (Perla); Cabrero-Ballarín, J.M. (José Manuel)

    2010-01-01

    Guía divulgativa ‘Construir con madera’, elaborada por la Cátedra Madera de la Universidad de Navarra y el Gobierno de Navarra. La publicación pretende explicar de forma sencilla los beneficios y posibilidades de este material en la construcción, tanto en lo que respecta a su resistencia, comportamiento frente al fuego, durabilidad, capacidad de aislamiento, propiedades acústicas, estética, respeto al medio ambiente y sostenibilidad como fuente de energía. A modo de ejemplo, en la ...

  20. 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.

  1. Juvenile myasthenia gravis in Norway: Clinical characteristics, treatment, and long-term outcome in a nationwide population-based cohort.

    Science.gov (United States)

    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.

  2. Immunization with Recombinantly Expressed LRP4 Induces Experimental Autoimmune Myasthenia Gravis in C57BL/6 Mice.

    Science.gov (United States)

    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.

  3. Entrevista con Giovanni Levi

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    Monica Oliveira

    2017-06-01

    Full Text Available En esta entrevista, Giovanni Levi - como un conocedor del tema de Familia - realiza una importante evaluación sobre el actual estado de las investigaciones realizadas en el Brasil y em el exterior. Con estilo franco, agudo y lucido critica las visiones tradicionales y sus ilusiones ypropone nuevos conceptos y métodos. La historia de la familia debería ceder espacio para el estudio de las redes relacionales o de los mundos relacionales. De la misma forma, la historia cuantitativa debería abrir espacio para el estudio de las cualidades. Ya con relación a la historia de las elites, tan estudiada y reproducida en una diversidad de trabajos, que deberíase mirar en otra perspectiva. Es decir, no mirar a las reglas sociales predeterminadas, sino a los desvíos y a las variaciones. Levi defiende que los historiadores deben trascender a los documentos que se encuentran fácilmente y que pueden fortalecer perspectivas deformadas y esequilibradas de la sociedad. Para él, los historiadores deben esforzarse por estudiar a aquellos grupos que dejaron pocos rastros documentales. En ese esfuerzo existiría una nueva mirada sobre la historia de la familia.

  4. Entrevista con Patricia Ariza

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    Esperanza Londoño La Rotta

    2017-01-01

    Full Text Available Pensamiento, Palabra y Obra entrevista a una artista, feminista y activista política, quien como mujer y artista ha permitido pensar el arte más allá de un simple espectáculo. Toda una vida dedicada al teatro y a darle voz, a través de sus obras, a víctimas del conflicto colombiano, defensora de derechos humanos; además de hacer evidente en su vida y a través de la plataforma “Artistas por la paz”, las múltiples relaciones que se pueden establecer entre el arte, la construcción de paz y la resolución de conflictos. Hablamos en su casa, en medio del calor de la bienvenida con Patricia Ariza, directora del festival alternativo de teatro, de Mujeres en Escena y de la Corporación Colombiana de Teatro, entre otras muchas actividades que voluntariamente su espíritu libertario ha asumido. Esta entrevista se realizó antes del 2 de octubre, pero con la revisión de los acuerdos que propició el plebiscito ganado por una ínfima minoría por el no, sigue siendo vigente este planteamiento.

  5. 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)

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

    Science.gov (United States)

    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.

  7. 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.

  8. 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.

  9. preescolares desnutridos con madres con obesidad y sin obesidad

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    Viridiana Vanessa Conzuelo-González

    2009-01-01

    Full Text Available El primer objetivo fue conocer cuántos menores de cinco años con diferentes grados de desnutrición tienen una madre con sobrepeso/obesidad/ en una comunidad indígena que vive en extrema pobreza y bajo condiciones de migración masculina internacional. El segundo fue comparar tres variables socionutricionales (ingreso familiar, educación de la madre y adecuación nutrimental de la dieta diaria entre estos hogares y los hogares con desnutrición infantil y madres sin obesidad. Se realizó un estudio transversal (2006-2007, en la comunidad mazahua de San Francisco Tepeolulco, Municipio de Temascalcingo; que incluyó a 85 hogares integrados por preescolares con desnutrición inscritos al programa Oportunidades. Se determinó el estado nutrición de los preescolares con indicadores antropométricos y se obtuvo el IMC de las madres de estos infantes. Se aplicó una encuesta socionutricional, incluida el recordatorio de 24 horas, y complementado con la observación participante (cualitativa. Se encontró que 83% de las madres mazahuas presentaron sobrepeso u obesidad. El estado de nutrición de los preescolares con madres con obesidad presentó un porcentaje mayor de desnutrición (76%. En la variable género, se encontró que 54% de los niños con madres con obesidad tenía baja talla. Al relacionar el nivel educativo de la madre, esta variable resultó ser estadísticamente significativa (p=0.015, donde el analfabetismo está más relacionado con la desnutrición infantil que tienen madres de bajo y/o peso normal. La elevada prevalencia de hogares conformados con preescolares con desnutrición y madres con obesidad, es un síntoma más de la pobreza en zonas indígenas en México, con bajo índice de desarrollo humano.

  10. autorregulado con estudiantes universitarios

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    Jairo Andrés Montes

    2005-01-01

    Full Text Available El propósito del presente estudio es describir la forma en la que se presentan los procesos de aprendizaje autorregulado con un grupo de estudiantes (22 estudiantes de tercer semestre de Psicología de la PUJ, Cali, en el evento de preparación para la presentación un examen. Asimismo se describen las correlaciones que ocurren entre las distintas fases de dicho proceso de autorregulación del aprendizaje. Para conseguir los objetivos propuestos se ha hecho uso de una observación de desempeño en tiempo real, es decir, de la observación durante una sesión de preparación de examen de los estudiantes, en la cual se emplearon protocolos verbales para dar cuenta de lo que «pasaba por su mente» mientras estudiaban. Una entrevista semi-estructurada y una prueba objetiva. Los resultados fueron analizados a la luz del modelo mixto de procesamiento de información y constructivismo abordado por Winne(1998. Como resultado se encontró una relación significativa entre los niveles de desempeño en el proceso de ARR y el resultado del examen. Igualmente se encontraron bajos niveles de regulación en una parte importante de la muestra y un desfase significativo entre conocimiento declarativo de ARR y desempeño en el mismo

  11. GRAVI-2 space experiment: investigating statoliths displacement and location effects on early stages of gravity perception pathways in lentil roots.

    Science.gov (United States)

    Bizet, François; Eche, Brigitte; Pereda Loth, Veronica; Badel, Eric; Legue, Valerie; Brunel, Nicole; Label, Philippe; Gérard, Joëlle

    2016-07-01

    The plants ability to orient their growth with respect to external stimuli such as gravity is a key factor for survival and acclimation to their environment. Belowground, plant roots modulate their growth towards gravity, allowing soil exploration and uptake of water and nutrients. In roots, gravity sensing cells called statocytes are located in the center of the root cap. Statocytes contain starch-filled plastids denser than the cytoplasm, which sedimentation along the direction of gravity is widely accepted as being involved into early stages of gravity perception (the starch-statolith hypothesis; Sack, 1991). Root gravitropism following statoliths displacement is based on auxin redistribution in the root apex, inducing differential growth between the root upward and downward sides. However at the cell scale, the chain of transduction starting from statoliths displacement and leading to auxin redistribution remains poorly documented. Signaling molecules such as calcium, reactive oxygen species, nitric oxide and inositol 1,4,5-triphosphate are serious candidates previously shown to be involved within minutes before modification of the expression of auxin-related genes (Morita, 2010; Sato et al., 2015). Here, we observe and quantify statoliths displacements and locations at various levels of gravity to investigate two hypothesis: (i) Are contacts between statoliths and the endoplasmic reticulum necessary to induce gravitropism? (ii) Are very low displacements of statoliths sufficient to initiate transduction pathways such as the calcium's one? These questionings have led to an experiment called GRAVI-2 which took place aboard the ISS in 2014. During the experiment, lentil roots were grown in the European modular cultivation system for several hours in microgravity and were then submitted to short high gravity stimulus (5 and 15 minutes at 2 g) before the return to Earth for analyses. Ongoing cytological measurements will reveal the effects of statoliths

  12. Ocular myasthenia gravis induced by human acetylcholine receptor ϵ subunit immunization in HLA DR3 transgenic mice.

    Science.gov (United States)

    Wu, Xiaorong; Tuzun, Erdem; Saini, Shamsher S; Wang, Jun; Li, Jing; Aguilera-Aguirre, Leopoldo; Huda, Ruksana; Christadoss, Premkumar

    2015-12-01

    Extraocular muscles (EOM) are preferentially involved in myasthenia gravis (MG) and acetylcholine receptor (AChR) antibody positive MG patients may occasionally present with isolated ocular symptoms. Although experimental autoimmune myasthenia gravis (EAMG) induced by whole AChR immunization closely mimics clinical and immunopathological aspects of MG, EOM are usually not affected. We have previously developed an EAMG model, which imitates EOM symptoms of MG by immunization of human leukocyte antigen (HLA) transgenic mice with α or γ-subunits of human AChR (H-AChR). To investigate the significance of the ϵ-subunit in ocular MG, we immunized HLA-DR3 and HLA-DQ8 transgenic mice with recombinant H-AChR ϵ-subunit expressed in Escherichia coli. HLA-DR3 transgenic mice showed significantly higher clinical ocular and generalized MG severity scores and lower grip strength values than HLA-DQ8 mice. H-AChR ϵ-subunit-immunized HLA-DR3 transgenic mice had higher serum anti-AChR antibody (IgG, IgG1, IgG2b, IgG2c and IgM) levels, neuromuscular junction IgG and complement deposit percentages than ϵ-subunit-immunized HLA-DQ8 transgenic mice. Control mice immunized with E. coli extract or complete Freund adjuvant (CFA) did not show clinical and immunopathological features of ocular and generalized EAMG. Lymph node cells of ϵ-subunit-immunized HLA-DR3 mice showed significantly higher proliferative responses than those of ϵ-subunit-immunized HLA-DQ8 mice, crude E. coli extract-immunized and CFA-immunized transgenic mice. Our results indicate that the human AChR ϵ-subunit is capable of inducing myasthenic muscle weakness. Diversity of the autoimmune responses displayed by mice expressing different HLA class II molecules suggests that the interplay between HLA class II alleles and AChR subunits might have a profound impact on the clinical course of MG. Copyright © 2015 European Federation of Immunological Societies. Published by Elsevier B.V. All rights reserved.

  13. Puentes con vigas pretensadas

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    Editorial, Equipo

    1965-07-01

    Full Text Available This paper describes one of the three bridges which Hidrocivil, S. A., has built in Catalonia (northern Spain, over the river Ripoll. The other two bridges are very similar to this one, both in construction and design, and show only minor adjustments to the local topography. The contracting firm proposed several alterations in the prefabrication and constructional procedure, in relation to the initial project, and these changes were accepted. The main feature of these projects is the use of prestressed beams, built at the workshop in sections, and joined together by means of sixty 7 mm cables in each beam. As the shear forces are more acute at the joints, the end of each section has a kind of diaphragm, to provide a large contact area, and hence greater surface to transmit the shear forces. The methods of construction are also of interest. Briefly, they involve building the bridge piles, and use these to support a provisional structure with transversal movement. This provisional structure, in turn, served as platform for two bridge cranes, which lifted the girders to their final location. After the first span was completed, the deck was concreted and the auxiliary structure pushed forward to the next span, to repeat the same operations. This arrangement saved the use of provisional framework.En este trabajo se describe uno de los tres puentes que Hidrocivil, S. A., ha construido.—previo concurso— en la región catalana; concretamente, el que salva el río Ripoll. Los otros dos no han sido objeto de descripción general por ser muy similares, en lo que a ejecución y concepción se refiere, con la única variante que presentan las características topográficas locales. La empresa propuso ciertas variantes— que fueron aceptadas— en la prefabricación y métodos de construcción. El interés de estas obras se centra en el empleo de vigas pretensadas, prefabricadas en taller por trozos, y solidarizados en el mismo mediante las operaciones

  14. Violencia con el anciano

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    Rita Campillo Motilva

    2002-08-01

    Full Text Available La violencia doméstica es tan antigua como la humanidad misma y se reconocen la violencia infantil, contra la mujer y al anciano, fundamentalmente; siendo este último grupo una población en ascenso por las mayores expectativas de vida de los últimos años. Como resultado de ello, el número de casos de abuso en el anciano se incrementará y el impacto de este abuso sobre la salud debe ser considerado de forma adecuada. La gama de maltratos es variadísima e incluye el abuso físico, emocional, financiero, sexual, por negligencia, negación a brindarle ayuda y otras formas más. Los ancianos con deterioro cognitivo son los más vulnerables. El médico en la atención primaria de salud es un pilar importante en la prevención y educación de este problema.Domestic violence is as old as humanity itself. Child, women and elderly abuse are mainly recognized. The elderly group is increasing due to the higher life expectancy experimented during the last years. As a result, the number of battered elderly will grow and the impact of this abuse on health should be adequately considered. The range of abuse is very wide and it includes physical, emotional, financial and sexual abuse, negligence, rejection to give assistance and others. The elderly with cognitive deterioration are the most vulnerable. The physician at the primary health care level is an important milestone in the prevention and education of this problem.

  15. Agrin-LRP4-MuSK signaling as a therapeutic target for myasthenia gravis and other neuromuscular disorders.

    Science.gov (United States)

    Ohno, Kinji; Ohkawara, Bisei; Ito, Mikako

    2017-10-01

    Signal transduction at the neuromuscular junction (NMJ) is compromised in a diverse array of diseases including myasthenia gravis, Lambert-Eaton myasthenic syndrome, Isaacs' syndrome, congenital myasthenic syndromes, Fukuyama-type congenital muscular dystrophy, amyotrophic lateral sclerosis, and sarcopenia. Except for sarcopenia, all are orphan diseases. In addition, the NMJ signal transduction is impaired by tetanus, botulinum, curare, α-bungarotoxin, conotoxins, organophosphate, sarin, VX, and soman to name a few. Areas covered: This review covers the agrin-LRP4-MuSK signaling pathway, which drives clustering of acetylcholine receptors (AChRs) and ensures efficient signal transduction at the NMJ. We also address diseases caused by autoantibodies against the NMJ molecules and by germline mutations in genes encoding the NMJ molecules. Expert opinion: Representative small compounds to treat the defective NMJ signal transduction are cholinesterase inhibitors, which exert their effects by increasing the amount of acetylcholine at the synaptic space. Another possible therapeutic strategy to enhance the NMJ signal transduction is to increase the number of AChRs, but no currently available drug has this functionality.

  16. Effect of ethnic origin and gender on the clinical manifestations of myasthenia gravis among the Jewish population in Israel.

    Science.gov (United States)

    Asmail, Ali; Kesler, Anat; Drory, Vivian E; Kolb, Hadar; Karni, Arnon

    2017-06-15

    Reports on patients with myasthenia gravis (MG) of different ethnic origins demonstrated differences in weakness distribution and serological results. We studied MG characteristics in a cohort of Ashkenazi (ASH) and non-Ashkenazi (NASH) Jewish origin according to their ethnic origins and gender. The frequency of age of MG onset was distributed in a bi-modal fashion in the female patients and increased gradually over time, with a peak around 70years of age in the male patients. Ocular MG was more frequent in males and ASH patients. Unlike previous reports, our male patients had a higher proportion of positive serum anti-acetyl choline receptor (AChR) than female patients, with no ethnic-based differences in the rates of anti-AChR or anti-muscle specific kinase. Comorbidity with another autoimmune disease was more frequent among female patients with late-onset MG and NASH patients (mainly Israel-born). Male MG patients tended to have more malignant comorbidities than female MG patients. These results demonstrate the effect of ethnicity on clinical aspects of MG within the Jewish population in Israel, and reveal novel effects of gender-associated comorbidities in patients with MG. Copyright © 2017. Published by Elsevier B.V.

  17. Brazilian cross-cultural translation and adaptation of the "Questionnaire of Life Quality Specific for Myasthenia Gravis - 15 items"

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    Aline Mansueto Mourao

    2013-12-01

    Full Text Available Objective To translate and to perform the cross-cultural adaptation of the “Questionnaire of Life Quality Specific for Myasthenia Gravis - 15 items” (MG-QOL15. Method The original English version of the questionnaire was translated into Portuguese. This version was revised and translated back into English. Later, both English versions were compared and the divergences were corrected in the Portuguese text. At a second stage, ten patients with MG followed at the Neuromuscular Diseases Clinic from the University Hospital, Universidade Federal de Minas Gerais answered the questionnaire. The authors analyzed the difficulties and misunderstandings in the application of the questionnaire. Results The questions 8, 13 and 15 were considered difficult to understand and were modified in the final Portuguese version. Most patients (70% had a total score above 25, and the statements 3, 8 and 9 showed the highest scores. Conclusion The Brazilian version of the questionnaire MG-QOL15 seems to be a promising tool for the assessment of Brazilian patients with MG.

  18. Clinical profile of patients with myasthenia gravis followed at the University Hospital, Federal University of Minas Gerais

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    Aline Mansueto Mourão

    2015-04-01

    Full Text Available Summary Objective: to determine the clinical profile of patients with myasthenia gravis (MG; followed at the Neuromuscular Diseases Clinic of the University Hospital, Federal University of Minas Gerais, Brazil, and to compare it with other Brazilian case series. Methods: sociodemographic and clinical data were collected from patients, and a systematic literature review performed, focusing on national studies on the clinical profile of MG patients. Results: sixty nine patients were enrolled in the study. Fifty five (91% subjects were female and the mean age (SD was 37.6 (±11.4 years. The mean disease duration was 14.1 years. Regarding treatment, prednisone was the most used strategy (64%, followed by the use of azathioprine (43%. There was no difference between thymectomized (42 and non-thymectomized (27 patients regarding disease severity and medication use. Conclusion: clinical and socio-demographic features of this MG sample from a University-based clinic resemble those reported in other Brazilian series and in the international literature.

  19. [Myasthenia gravis in adults of institutions pertaining to the Mexican public health system: an analysis of hospital discharges during 2010].

    Science.gov (United States)

    Tolosa-Tort, Paulina; Chiquete, Erwin; Domínguez-Moreno, Rogelio; Vega-Boada, Felipe; Reyes-Melo, Isael; Flores-Silva, Fernando; Sentíes-Madrid, Horacio; Estañol-Vidal, Bruno; García-Ramos, Guillermo; Herrera-Hernández, Miguel; Ruiz-Sandoval, José L; Cantú-Brito, Carlos

    2015-01-01

    Epidemiological studies on myasthenia gravis (MG) in Mexico is mainly derived from experiences in referral centers. To describe the epidemiological characteristics of hospital discharges during 2010 with the diagnosis of MG in adults hospitalized in the Mexican public health system. We consulted the database of hospital discharges during 2010 of the National Health Information System (Ministry of Health, IMSS, IMSS oportunidades, ISSSTE, PEMEX, and the Ministry of Defense). The MG records were identified by the code G70.0 of the International Classification of Diseases 10th revision. During 2010 there were 5,314,132 hospital discharges (4,254,312 adults). Among them, 587 (0.01%) were adults with MG (median age: 47 years, 60% women). Women with MG were significantly younger than men (median age: 37 vs. 54 years, respectively; p < 0.001). The median hospital stay was six days. The case fatality rate was 3.4%, without gender differences. Age was associated with the probability of death. We confirmed the bimodal age-gender distribution in MG. The in-hospital case fatality rate in Mexico is consistent with recent reports around the world.

  20. T-cell receptor V sub. alpha. and C sub. alpha. alleles associated with multiple sclerosis and myasthenia gravis

    Energy Technology Data Exchange (ETDEWEB)

    Oksenberg, J.R.; Cavalli-Sforza, L.L.; Steinman, L. (Stanford Univ., CA (USA)); Sherritt, M.; Bernard, C.C. (LaTrobe Univ., Victoria (Australia)); Begovich, A.B.; Erlich, H.A. (Cetus Corporation, Emeryville, CA (USA))

    1989-02-01

    Polymorphic markers in genes encoding the {alpha} chain of the human T-cell receptor (TcR) have been detected by Southern blot analysis in Pss I digests. Polymorphic bands were observed at 6.3 and 2.0 kilobases (kb) with frequencies of 0.30 and 0.44, respectively, in the general population. Using the polymerase chain reaction (PCR) method, the authors amplified selected sequences derived from the full-length TcR {alpha} cDNA probe. These PcR products were used as specific probes to demonstrate that the 6.3-kb polymorphic fragment hybridizes to the variable (V)-region probe and the 2.0-kb fragment hybridizes to the constant (C)-region probe. Segregation of the polymorphic bands was analyzed in family studies. To look for associations between these markers and autoimmune diseases, the authors have studied the restriction fragment length polymorphism distribution of the Pss I markers in patients with multiple sclerosis, myasthenia gravis, and Graves disease. Significant differences in the frequency of the polymorphic V{sub {alpha}} and C{sub {alpha}} markers were identified between patients and healthy individuals.

  1. Amyotrophic Lateral Sclerosis and Myasthenia Gravis Overlap Syndrome: A Review of Two Cases and the Associated Literature

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    Hongfei Tai

    2017-05-01

    Full Text Available ObjectiveTo describe the characteristics of patients with amyotrophic lateral sclerosis (ALS and myasthenia gravis (MG overlap syndrome and explore the relationship between the two diseases.MethodsWe conducted a search of medical records at Peking Union Medical University Hospital from 1983 to 2015 for coexistence of ALS and MG and searched the PubMed database for all literature describing ALS and MG overlap syndrome published through December 2016. We analyzed the clinical and neurophysiological characteristics of patients by groups according to strict diagnostic criteria.ResultsWe presented 2 patients in our database with combined ALS and MG, and together with 25 cases reported in the literature, the patients were divided into 4 groups: 12 patients with MG followed by ALS, 8 patients with ALS followed by MG, 5 ALS patients with false-positive anti-acetylcholine receptor, and the other 2 ALS patients with only myasthenia symptoms. Most patients had limb onset ALS, and myasthenia symptoms mainly affected ocular and bulbar muscles. Clinical and neurophysiological characteristics were summarized.ConclusionThese findings support the conclusion that immunological mechanisms and alterations in the neuromuscular junction are related to ALS pathogenesis.

  2. Thymus cells in myasthenia gravis selectively enhance production of anti-acetylcholine-receptor antibody by autologous blood lymphocytes

    International Nuclear Information System (INIS)

    Newsom-Davis, J.; Willcox, N.; Calder, L.

    1981-01-01

    We investigated the role of the thymus in 16 patients with myasthenia gravis without thymoma by studying the production of anti-acetylcholine-receptor antibody by thymic and blood lymphocytes cultured alone or together. In 10 responders (with the highest receptor-antibody titers in their plasma), cultured thymic cells spontaneously produced measurable receptor antibody. Receptor-antibody production by autologous blood lymphocytes was enhanced by the addition of responder's thymic cells, irradiated to abrogate antibody production and suppression (P<0.01). This enhancement was greater and more consistent than that by pokeweed mitogen; it depended on viable thymic cells, appeared to be selective for receptor antibody, and correlated with the ratio of thymic helper (OKT4-positive or OKT4+) to suppressor (OKT8+) T cells (P<0.01). These results suggest that myasthenic thymus contains cell-bound acetylcholine-receptor-like material or specific T cells (or both) that can aid receptor-antibody production. This may be relevant to the benefits of thymectomy in myasthenia and to the breakdown in self-tolerance in this and other autoimmune diseases

  3. Thymus cells in myasthenia gravis selectively enhance production of anti-acetylcholine-receptor antibody by autologous blood lymphocytes

    Energy Technology Data Exchange (ETDEWEB)

    Newsom-Davis, J.; Willcox, N.; Calder, L.

    1981-11-26

    We investigated the role of the thymus in 16 patients with myasthenia gravis without thymoma by studying the production of anti-acetylcholine-receptor antibody by thymic and blood lymphocytes cultured alone or together. In 10 responders (with the highest receptor-antibody titers in their plasma), cultured thymic cells spontaneously produced measurable receptor antibody. Receptor-antibody production by autologous blood lymphocytes was enhanced by the addition of responder's thymic cells, irradiated to abrogate antibody production and suppression (P<0.01). This enhancement was greater and more consistent than that by pokeweed mitogen; it depended on viable thymic cells, appeared to be selective for receptor antibody, and correlated with the ratio of thymic helper (OKT4-positive or OKT4+) to suppressor (OKT8+) T cells (P<0.01). These results suggest that myasthenic thymus contains cell-bound acetylcholine-receptor-like material or specific T cells (or both) that can aid receptor-antibody production. This may be relevant to the benefits of thymectomy in myasthenia and to the breakdown in self-tolerance in this and other autoimmune diseases.

  4. Antibodies to AChR, MuSK and VGKC in a patient with myasthenia gravis and Morvan's syndrome.

    Science.gov (United States)

    Díaz-Manera, Jordi; Rojas-García, Ricard; Gallardo, Eduard; Juárez, Cándido; Martínez-Domeño, Alejandro; Martínez-Ramírez, Sergi; Dalmau, Josep; Blesa, Rafael; Illa, Isabel

    2007-07-01

    A 46-year-old woman presented to a local hospital with acute respiratory failure and a 2-year progressive history of fatigue, personality changes, increased sweating, dysphagia with substantial weight loss, dysarthria, and intermittent ptosis and diplopia. Neurological examination showed facial weakness, lingual atrophy and bulbar palsy, which necessitated the use of a feeding tube and ventilatory support. Mild limb weakness with severe muscle atrophy and diffuse muscle twitches were observed. The patient had also developed visual hallucinations and persecutory delusions. Her personal and family medical histories were unremarkable. Sensory and motor nerve conduction studies, repetitive nerve stimulation, electromyogram, blood-cell counts, general chemistry and metabolic function tests, a CT scan, an [(18)F]fluorodeoxyglucose-PET scan, and tests for serum antibodies to acetylcholine receptors, muscle-specific tyrosine kinase, voltage-gated potassium channels, P/Q-type voltage-gated calcium channels, and paraneoplastic antigens, were carried out. Myasthenia gravis associated with antibodies to acetylcholine receptor and muscle-specific tyrosine kinase, and Morvan's syndrome associated with antibodies to voltage-gated potassium channels in the absence of thymoma. Combined treatment with prednisone, intravenous immunoglobulin, ciclosporin, and rituximab.

  5. Acne polimorfo: tratamiento con Implacen

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    Rubén Pérez Armas

    1995-06-01

    Full Text Available Se realiza el estudio de 40 pacientes con acné polimorfo, los que fueron atendidos en la Consulta de Dermatología del Hospital Provincial Clinicoquirúrgico Docente "Celia Sánchez Manduley", en el período comprendido de enero de 1988 a diciembre de 1989. Se revisa la literatura médica sobre los diversos métodos y medicamentos utilizados en la terapéutica de esta dermatosis. Se describe el esquema de tratamiento empleado con implacén en 30 pacientes; los 10 restantes se trataron con placebo; se compara dicho esquema con los tradicionales y se observan mejores resultados con nuestro estudio. Se destaca la ausencia de recaídas, así como el resultado del tratamiento de acuerdo con el sexo.A study was performed in 40 patients presenting with polymorphic acne who were attended in the Dermatology Department of "Celia Sánchez Manduley" Clinicosurgical and Teaching Hospital from January, 1988 to December, 1989. A review of the literature was made seeking for the different methods and drugs used for the treatment of this dermatosis. The treatment schedule with the use of implacen in 30 patients is described. Such therapeutic schedule was compared with traditional ones and better results were observed with the use of implacen. The fact that there were no relapses is highlighted, as well as the result of treatment according to sex.

  6. con mala calidad de vida

    Directory of Open Access Journals (Sweden)

    Agustín Martín-Rodríguez

    2007-01-01

    Full Text Available En este estudio ex post facto se ha analizado si los familiares de pacientes con mala calidad de vida presentan diferencias en las variables clínicas de personalidad y relaciones familiares en función de que el paciente haya estado o no ingresado en una Unidad de Cuidados Intensivos. Seleccionamos dos grupos: 29 familiares de pacientes traumatizados graves transcurridos cuatro años de su ingreso en una UCI de Traumatología y con mala calidad de vida (debido a secuelas físicas y/o psicológicas tras el ingreso, tales como traumatismos craneoencefálicos, politraumatismos y tetraplejias traumáticas y 32 familiares de pacientes con mala calidad de vida con cuatro años de evolución de su enfermedad física (hipertensión, diabetes, artritis reumatoide y síndrome de intestino irritable que no han estado ingresados en la UCI. Para alcanzar nuestro objetivo empleamos una Encuesta Psicosocial y los siguientes instrumentos: Cuestionario de Análisis Clínico, Escala de Clima Social en la Familia y Escala de Adaptación Psicosocial de la Enfermedad. Los resultados mostraron que los familiares de pacientes con mala calidad de vida que estuvieron ingresados en la UCI hace cuatro años, presentan diferencias significativas en las variables agitación y expresividad comparados con los familiares de pacientes con mala calidad de vida que no han estado ingresados en la UCI.

  7. Reliability and main findings of the FEES-Tensilon Test in patients with myasthenia gravis and dysphagia.

    Science.gov (United States)

    Im, Sun; Suntrup-Krueger, Sonja; Colbow, Sigrid; Sauer, Sonja; Claus, Inga; Meuth, Sven G; Dziewas, Rainer; Warnecke, Tobias

    2018-05-26

    Diagnosis of pharyngeal dysphagia caused by myasthenia gravis (MG) based on clinical examination alone is often challenging. Flexible endoscopic evaluation of swallowing (FEES) combined with Tensilon (edrophonium) application, referred to as the FEES-Tensilon Test, was developed to improve diagnostic accuracy and to detect the main symptoms of pharyngeal dysphagia in MG. Here we investigated inter- and intra-rater reliability of the FEES-Tensilon Test and analyzed the main endoscopic findings. Four experienced raters reviewed a total of 20 FEES-Tensilon-Test videos in randomized order. Residue severity was graded at 4 different pharyngeal spaces before and after Tensilon administration. All interpretations were performed twice per rater, 4 weeks apart (a total of 160 scorings). Intra-rater test-retest reliability and inter-rater reliability levels were calculated. The most frequent FEES findings in MG patients before Tensilon application were prominent residues of semi solids spread all over the hypopharynx in varying locations. The reliability level in the interpretation of the FEES-Tensilon test was excellent regardless of the raters' profession or years of experience with FEES. All 4 raters showed high inter- and intra- reliability levels in interpreting the FEES-Tensilon Test based on residue clearance (kappa=0.922, 0.981). Degree of residue normalization in the vallecular space after Tensilon application showed the highest inter- and intra-rater reliability level (kappa=0.863, 0.957) followed by the epiglottis (kappa=0.813, 0.946) and pyriform sinuses (kappa=0.836, 0.929). Interpretation of the FEES-Tensilon Test based on residue severity and degree of Tensilon clearance, especially in the vallecular space, is consistent and reliable. This article is protected by copyright. All rights reserved. This article is protected by copyright. All rights reserved.

  8. Clonal heterogeneity of thymic B cells from early-onset myasthenia gravis patients with antibodies against the acetylcholine receptor.

    Science.gov (United States)

    Vrolix, Kathleen; Fraussen, Judith; Losen, Mario; Stevens, Jo; Lazaridis, Konstantinos; Molenaar, Peter C; Somers, Veerle; Bracho, Maria Alma; Le Panse, Rozen; Stinissen, Piet; Berrih-Aknin, Sonia; Maessen, Jos G; Van Garsse, Leen; Buurman, Wim A; Tzartos, Socrates J; De Baets, Marc H; Martinez-Martinez, Pilar

    2014-08-01

    Myasthenia gravis (MG) with antibodies against the acetylcholine receptor (AChR-MG) is considered as a prototypic autoimmune disease. The thymus is important in the pathophysiology of the disease since thymus hyperplasia is a characteristic of early-onset AChR-MG and patients often improve after thymectomy. We hypothesized that thymic B cell and antibody repertoires of AChR-MG patients differ intrinsically from those of control individuals. Using immortalization with Epstein-Barr Virus and Toll-like receptor 9 activation, we isolated and characterized monoclonal B cell lines from 5 MG patients and 8 controls. Only 2 of 570 immortalized B cell clones from MG patients produced antibodies against the AChR (both clones were from the same patient), suggesting that AChR-specific B cells are not enriched in the thymus. Surprisingly, many B cell lines from both AChR-MG and control thymus samples displayed reactivity against striated muscle proteins. Striational antibodies were produced by 15% of B cell clones from AChR-MG versus 6% in control thymus. The IgVH gene sequence analysis showed remarkable similarities, concerning VH family gene distribution, mutation frequency and CDR3 composition, between B cells of AChR-MG patients and controls. MG patients showed clear evidence of clonal B cell expansion in contrast to controls. In this latter aspect, MG resembles multiple sclerosis and clinically isolated syndrome, but differs from systemic lupus erythematosus. Our results support an antigen driven immune response in the MG thymus, but the paucity of AChR-specific B cells, in combination with the observed polyclonal expansions suggest a more diverse immune response than expected. Copyright © 2013 Elsevier Ltd. All rights reserved.

  9. Diving of great shearwaters (Puffinus gravis in cold and warm water regions of the South Atlantic Ocean.

    Directory of Open Access Journals (Sweden)

    Robert A Ronconi

    Full Text Available BACKGROUND: Among the most widespread seabirds in the world, shearwaters of the genus Puffinus are also some of the deepest diving members of the Procellariiformes. Maximum diving depths are known for several Puffinus species, but dive depths or diving behaviour have never been recorded for great shearwaters (P. gravis, the largest member of this genus. This study reports the first high sampling rate (2 s of depth and diving behaviour for Puffinus shearwaters. METHODOLOGY/PRINCIPAL FINDINGS: Time-depth recorders (TDRs were deployed on two female great shearwaters nesting on Inaccessible Island in the South Atlantic Ocean, recording 10 consecutive days of diving activity. Remote sensing imagery and movement patterns of 8 males tracked by satellite telemetry over the same period were used to identify probable foraging areas used by TDR-equipped females. The deepest and longest dive was to 18.9 m and lasted 40 s, but most (>50% dives were <2 m deep. Diving was most frequent near dawn and dusk, with <0.5% of dives occurring at night. The two individuals foraged in contrasting oceanographic conditions, one in cold (8 to 10°C water of the Sub-Antarctic Front, likely 1000 km south of the breeding colony, and the other in warmer (10 to 16°C water of the Sub-tropical Frontal Zone, at the same latitude as the colony, possibly on the Patagonian Shelf, 4000 km away. The cold water bird spent fewer days commuting, conducted four times as many dives as the warm water bird, dived deeper on average, and had a greater proportion of bottom time during dives. CONCLUSIONS/SIGNIFICANCE: General patterns of diving activity were consistent with those of other shearwaters foraging in cold and warm water habitats. Great shearwaters are likely adapted to forage in a wide range of oceanographic conditions, foraging mostly with shallow dives but capable of deep diving.

  10. 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:…

  11. Tratamiento con implantes Leader-Nano en paciente con oligodoncia

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    Salvador Javier Santos Medina

    2015-03-01

    Full Text Available Los implantes dentales de titanio han revolucionado el mundo de la rehabilitación desde su surgimiento. De manera particular, el empleo de implantes de carga inmediata acorta el tiempo quirúrgico y protésico, con el consiguiente bienestar estético. Se presenta el caso de una paciente femenina de 32 años de edad, con antecedentes de oligodoncia de ambos incisivos laterales superiores y portadora de prótesis parcial acrílica. Fue atendida por el equipo multidisciplinario de implantes en la Clínica Estomatológica Docente “3 de Octubre” y se le realizó tratamiento de rehabilitación integral con implantes Leader-Nano y prótesis fija con corona acrílica sobre dichos implantes. La implantología fue satisfactoria en la paciente; la mejoría estética y funcional, así como la satisfacción de la paciente, fueron los principales logros obtenidos

  12. 7-Ketocholesterol and 7-hydroxycholesterol in pork meat and its gravy thermally treated without additives and in the presence of onion and garlic.

    Science.gov (United States)

    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.

  13. Dietary Patterns High in Red Meat, Potato, Gravy, and Butter Are Associated with Poor Cognitive Functioning but Not with Rate of Cognitive Decline in Very Old Adults1234

    Science.gov (United States)

    Davies, Karen; Adamson, Ashley; Kirkwood, Thomas; Hill, Tom R; Siervo, Mario; Mathers, John C; Jagger, Carol

    2016-01-01

    Background: Healthy dietary patterns (DPs) have been linked to better cognition and reduced risk of dementia in older adults, but their role in cognitive functioning and decline in the very old (aged ≥85 y) is unknown. Objective: We investigated the association between previously established DPs from the Newcastle 85+ Study and global and attention-specific cognition over 5 y. Methods: We followed up with 302 men and 489 women (1921 birth cohort from Northeast United Kingdom) for change in global cognition [measured by the Standardized Mini-Mental State Examination (SMMSE)] over 5 y and attention (assessed by the cognitive drug research attention battery) over 3 y. We used 2-step clustering to derive DPs and mixed models to determine the relation between DPs and cognition in the presence of the dementia susceptibility gene. Results: Previously, we characterized 3 DPs that differed in intake of red meat, potato, gravy, and butter and varied with key health measures. When compared with participants in DP1 (high red meat) and DP3 (high butter), participants in DP2 (low meat) had higher SMMSE scores at baseline (P gravy (DP1), or butter (DP3) were associated with poor cognition but not with the rate of cognitive decline in very old adults. PMID:26740685

  14. 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.

  15. Dietary Patterns High in Red Meat, Potato, Gravy, and Butter Are Associated with Poor Cognitive Functioning but Not with Rate of Cognitive Decline in Very Old Adults.

    Science.gov (United States)

    Granic, Antoneta; Davies, Karen; Adamson, Ashley; Kirkwood, Thomas; Hill, Tom R; Siervo, Mario; Mathers, John C; Jagger, Carol

    2016-02-01

    Healthy dietary patterns (DPs) have been linked to better cognition and reduced risk of dementia in older adults, but their role in cognitive functioning and decline in the very old (aged ≥85 y) is unknown. We investigated the association between previously established DPs from the Newcastle 85+ Study and global and attention-specific cognition over 5 y. We followed up with 302 men and 489 women (1921 birth cohort from Northeast United Kingdom) for change in global cognition [measured by the Standardized Mini-Mental State Examination (SMMSE)] over 5 y and attention (assessed by the cognitive drug research attention battery) over 3 y. We used 2-step clustering to derive DPs and mixed models to determine the relation between DPs and cognition in the presence of the dementia susceptibility gene. Previously, we characterized 3 DPs that differed in intake of red meat, potato, gravy, and butter and varied with key health measures. When compared with participants in DP1 (high red meat) and DP3 (high butter), participants in DP2 (low meat) had higher SMMSE scores at baseline (P gravy (DP1), or butter (DP3) were associated with poor cognition but not with the rate of cognitive decline in very old adults.

  16. Standardization of the experimental autoimmune myasthenia gravis (EAMG) model by immunization of rats with Torpedo californica acetylcholine receptors — Recommendations for methods and experimental designs

    Science.gov (United States)

    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

  17. 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)

  18. Evaluation of repetitive stimulation test (RST in 30 patients with Myasthenia Gravis, who were previously confirmed by clinical sign and tensilon test 1996-99

    Directory of Open Access Journals (Sweden)

    "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

  19. con dietas suplementadas con Cromo-L-metionina

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    Ram\\u00F3n Garc\\u00EDa-Castillo

    2006-01-01

    Full Text Available Un total de 48 cerdos (Sus scrofa domesticus; 24 machos castrados y 24 hembras cruzados (Yorkshire, Hampshire, Duroc y Landrace de 3,5 a 4,0 meses de edad y 60,0 ± 5,0 kg PV en finalización. Se alimentaron con dietas isoproteícas (14,5 % PC e isoenergéticas (3.400 kcal EM/kg de MS, adicionadas con Cr-L-metionina (MiCroPlex® (0, 200, 400 y 600 ppb. El experimento tuvo una duración de 45 días y se realizó de agosto a noviembre del 2002 en las instalaciones de la Universidad Autónoma Agraria Antonio Narro, localizada en Saltillo, Coahuila, México. Al tener los animales aproximadamente 95 kg PV, se tomó muestra de 15 ml de sangre por cada animal para determinar la concentración de glucosa, ácido úrico, creatinina, urea, proteinas totales y colesterol. Se aplicó un diseño completamente al azar con arreglo factorial 2 x 4; dos para el factor sexo y cuatro para nivel de cromo. Los metabolitos en suero no fueron afectados (P>0,05 por el factor sexo. La glucosa en suero disminuyó (P<0,05 y el colesterol incrementó (P<0,05 con cromo en la dieta. Se concluye que el Cr incrementa el metabolismo de glucosa y disminuye el de colesterol, con lo cual puede haber energía disponible para síntesis de proteína la cual es necesaria para el crecimiento de los animales

  20. Recubrimiento de acero con polidopamina

    OpenAIRE

    Carrasco Rodríguez, Javier

    2013-01-01

    Se ha obtenido recubrimientos de polidopamina en acero mecánicamente resistentes y con tiempos de obtención relativamente pequeños a través de la polimerización de la dopamina bajo diferentes condiciones.

  1. Santiago, una ciudad con temor

    Directory of Open Access Journals (Sweden)

    Enrique Oviedo S.

    1999-04-01

    Full Text Available El objetivo general de este artículo es evaluar los efectos de la inseguridad ciudadana en el uso del espacio público. Dicha evaluación exige analizar dos relaciones que se establecen en el ámbito de la violencia: la relación entre victimización y percepción de inseguridad; y la que se establece entre actitudes sociales y resolución pacífica de conflictos nacionales. Para ello, se analizaron las variables victimización, percepción de inseguridad, uso del espacio físico, actitudes hacia el sistema institucional político y social y hacia la resolución de conflictos nacionales, y las posibles relaciones entre ellas. Los datos para realizar el estudio se obtuvieron por medio de una encuesta que se llevó a cabo con 1 200 personas de 18 y 70 años de edad residentes en la ciudad de Santiago. Los resultados indican que Santiago es una ciudad de habitantes con temor y que el aumento de la percepción de inseguridad de sus habitantes contrasta con el hecho de que las tasas de victimización se hayan mantenido, más o menos, constantes en los años que precedieron a la encuesta. El temor se relaciona con el abandono del espacio público físico y sociopolítico, así como con el refugio en los espacios y la vida privados. La actitud de resolver los conflictos por medios no pacíficos es frecuente y se asocia en mayor medida con la inseguridad, la actitud negativa hacia la democracia y la falta de expectativas sobre el futuro del país. Los resultados de este estudio respaldan la idea de que para superar el temor la gente tiende a adaptarse a la realidad adoptando una postura conformista, homogeneizando las creencias y los comportamientos, y sobreestimando la fuerza como medio para resolver las diferencias.

  2. Santiago, una ciudad con temor

    Directory of Open Access Journals (Sweden)

    Oviedo S. Enrique

    1999-01-01

    Full Text Available El objetivo general de este artículo es evaluar los efectos de la inseguridad ciudadana en el uso del espacio público. Dicha evaluación exige analizar dos relaciones que se establecen en el ámbito de la violencia: la relación entre victimización y percepción de inseguridad; y la que se establece entre actitudes sociales y resolución pacífica de conflictos nacionales. Para ello, se analizaron las variables victimización, percepción de inseguridad, uso del espacio físico, actitudes hacia el sistema institucional político y social y hacia la resolución de conflictos nacionales, y las posibles relaciones entre ellas. Los datos para realizar el estudio se obtuvieron por medio de una encuesta que se llevó a cabo con 1 200 personas de 18 y 70 años de edad residentes en la ciudad de Santiago. Los resultados indican que Santiago es una ciudad de habitantes con temor y que el aumento de la percepción de inseguridad de sus habitantes contrasta con el hecho de que las tasas de victimización se hayan mantenido, más o menos, constantes en los años que precedieron a la encuesta. El temor se relaciona con el abandono del espacio público físico y sociopolítico, así como con el refugio en los espacios y la vida privados. La actitud de resolver los conflictos por medios no pacíficos es frecuente y se asocia en mayor medida con la inseguridad, la actitud negativa hacia la democracia y la falta de expectativas sobre el futuro del país. Los resultados de este estudio respaldan la idea de que para superar el temor la gente tiende a adaptarse a la realidad adoptando una postura conformista, homogeneizando las creencias y los comportamientos, y sobreestimando la fuerza como medio para resolver las diferencias.

  3. Heterocyclic amine content in beef cooked by different methods to varying degrees of doneness and gravy made from meat drippings.

    Science.gov (United States)

    Sinha, R; Rothman, N; Salmon, C P; Knize, M G; Brown, E D; Swanson, C A; Rhodes, D; Rossi, S; Felton, J S; Levander, O A

    1998-04-01

    Meats cooked at high temperatures sometimes contain heterocyclic amines (HCAs) that are known mutagens and animal carcinogens, but their carcinogenic potential in humans has not been established. To investigate the association between HCAs and cancer, sources of exposure to these compounds need to be determined. Beef is the most frequently consumed meat in the United States and for this study we determined HCA values in beef samples cooked in ways to represent US cooking practices, the results of which can be used in epidemiological studies to estimate HCA exposure from dietary questionnaires. We measured five HCAs [2-amino-3-methylimidazo[4,5-f]quinoline (IQ), 2-amino-3,4-dimethylimidazo[4,5-f]quinoline (MeIQ), 2-amino-3,8-dimethylimidazo[4,5-f]quinoxaline (MeIQx), 2-amino-3,4,8-trimethylimidazo[4,5-f]quinoxaline (DiMeIQx) and 2-amino-1-methyl-6-phenylimidazo[4,5-b]pyridine (PhIP)] in different types of cooked beef using solid-phase extraction and HPLC. Steak and hamburger patties were pan-fried, oven-broiled, and grilled/barbecued to four levels of doneness (rare, medium, well done or very well done), while beef roasts were oven cooked to three levels of doneness (rare, medium or well done). The measured values of the specific HCAs varied with the cut of beef, cooking method, and doneness level. In general, MeIQx content increased with doneness under each cooking condition for steak and hamburger patties, up to 8.2 ng/g. PhIP was the predominant HCA produced in steak (1.9 to 30 ng/g), but was formed only in very well done fried or grilled hamburger. DiMeIQx was found in trace levels in pan-fried steaks only, while IQ and MeIQ were not detectable in any of the samples. Roast beef did not contain any of the HCAs, but the gravy made from the drippings from well done roasts had 2 ng/g of PhIP and 7 ng/g of MeIQx. Epidemiological studies need to consider the type of meat, cooking method and degree of doneness/surface browning in survey questions to adequately assess

  4. The benefits and tolerance of exercise in myasthenia gravis (MGEX): study protocol for a randomised controlled trial.

    Science.gov (United States)

    Birnbaum, Simone; Hogrel, Jean-Yves; Porcher, Raphael; Portero, Pierre; Clair, Bernard; Eymard, Bruno; Demeret, Sophie; Bassez, Guillaume; Gargiulo, Marcela; Louët, Estelle; Berrih-Aknin, Sonia; Jobic, Asmaa; Aegerter, Philippe; Thoumie, Philippe; Sharshar, Tarek

    2018-01-18

    Research exploring the effects of physical exercise in auto-immune myasthenia gravis (MG) is scarce. The few existing studies present methodological shortcomings limiting the conclusions and generalisability of results. It is hypothesised that exercise could have positive physical, psychological as well as immunomodulatory effects and may be a beneficial addition to current pharmacological management of this chronic disease. The aim of this study is to evaluate the benefits on perceived quality of life (QOL) and physical fitness of a home-based physical exercise program compared to usual care, for patients with stabilised, generalised auto-immune MG. MGEX is a multi-centre, interventional, randomised, single-blind, two-arm parallel group, controlled trial. Forty-two patients will be recruited, aged 18-70 years. Following a three-month observation period, patients will be randomised into a control or experimental group. The experimental group will undertake a 40-min home-based physical exercise program using a rowing machine, three times a week for three months, as an add-on to usual care. The control group will receive usual care with no additional treatment. All patients will be followed up for a further three months. The primary outcome is the mean change in MGQOL-15-F score between three and six months (i.e. pre-intervention and immediately post-intervention periods). The MGQOL-15-F is an MG-specific patient-reported QOL questionnaire. Secondary outcomes include the evaluation of deficits and functional limitations via MG-specific clinical scores (Myasthenia Muscle Score and MG-Activities of Daily Living scale), muscle force and fatigue, respiratory function, free-living physical activity as well as evaluations of anxiety, depression, self-esteem and overall QOL with the WHO-QOL BREF questionnaire. Exercise workload will be assessed as well as multiple safety measures (ECG, biological markers, medication type and dosage and any disease exacerbation or crisis

  5. Comparative study for surgical management of thymectomy for non-thymomatous myasthenia gravis from the French national database EPITHOR.

    Science.gov (United States)

    Orsini, Bastien; Santelmo, Nicola; Pages, Pierre Benoit; Baste, Jean Marc; Dahan, Marcel; Bernard, Alain; Thomas, Pascal Alexandre

    2016-09-01

    Thymectomy may be part of the therapeutic strategy in patients with myasthenia gravis (MG) without thymoma. Median sternotomy is still considered as the gold standard, but during the last 15 years, several groups have demonstrated the non-inferiority of cervicotomy with upper sternotomy and minimally invasive techniques. To date, there is no consensus on surgical procedure choice. The aim of our study was to compare the morbidity and mortality of three techniques [cervicotomy with upper sternotomy versus sternotomy versus video-assisted thoracic surgery (VATS)/robotic-assisted thoracic surgery (RATS)] from the national database EPITHOR and to analyse French epidemiology. From the national thoracic surgery database EPITHOR, we have extracted all the details regarding thymectomies performed for non-thymomatous MG. We have divided thymectomy into three groups: A-sternotomy; B-cervicotomy with upper sternotomy; C-VATS/RATS. We investigated the postoperative morbidity and mortality without analysis of the long-term evolution of the disease not available on EPITHOR. From 2005 to 2013, 278 patients were included: 131 (47%) in Group A, 31 (11%) in Group B and 116 (42%) in Group C. The sex ratio F/M was 2.3. The mean age was, respectively, 42 ± 17, 42 ± 16, 35 ± 14 years old (P < 0.01). The number of patients without comorbidities was 63 (48%), 25 (81%) and 78 (65%), respectively (P < 0.01). The operative time was 94 ± 37, 79 ± 42 and 112 ± 59 min, respectively (P < 0.01). The number of patients who presented at least one postoperative complication was 12 (14%), 0 and 3 (9%) (P= 0.03), respectively. The postoperative lengths of stay were 7.7 ± 4.5, 5 ± 1.7 and 4.5 ± 2 days, respectively (P < 0.01). There was no death. In our study, we were unable to prove the superiority of minimally invasive techniques due to the important differences between the groups. However, this study shows us major changes in French surgical procedures during the last decade with an

  6. IgG4 autoantibodies against muscle-specific kinase undergo Fab-arm exchange in myasthenia gravis patients.

    Science.gov (United States)

    Koneczny, Inga; Stevens, Jo A A; De Rosa, Anna; Huda, Saif; Huijbers, Maartje G; Saxena, Abhishek; Maestri, Michelangelo; Lazaridis, Konstantinos; Zisimopoulou, Paraskevi; Tzartos, Socrates; Verschuuren, Jan; van der Maarel, Silvère M; van Damme, Philip; De Baets, Marc H; Molenaar, Peter C; Vincent, Angela; Ricciardi, Roberta; Martinez-Martinez, Pilar; Losen, Mario

    2017-02-01

    Autoimmunity mediated by IgG4 subclass autoantibodies is an expanding field of research. Due to their structural characteristics a key feature of IgG4 antibodies is the ability to exchange Fab-arms with other, unrelated, IgG4 molecules, making the IgG4 molecule potentially monovalent for the specific antigen. However, whether those disease-associated antigen-specific IgG4 are mono- or divalent for their antigens is unknown. Myasthenia gravis (MG) with antibodies to muscle specific kinase (MuSK-MG) is a well-recognized disease in which the predominant pathogenic IgG4 antibody binds to extracellular epitopes on MuSK at the neuromuscular junction; this inhibits a pathway that clusters the acetylcholine (neurotransmitter) receptors and leads to failure of neuromuscular transmission. In vitro Fab-arm exchange-inducing conditions were applied to MuSK antibodies in sera, purified IgG4 and IgG1-3 sub-fractions. Solid-phase cross-linking assays were established to determine the extent of pre-existing and inducible Fab-arm exchange. Functional effects of the resulting populations of IgG4 antibodies were determined by measuring inhibition of agrin-induced AChR clustering in C2C12 cells. To confirm the results, κ/κ, λ/λ and hybrid κ/λ IgG4s were isolated and tested for MuSK antibodies. At least fifty percent of patients had IgG4, but not IgG1-3, MuSK antibodies that could undergo Fab-arm exchange in vitro under reducing conditions. Also MuSK antibodies were found in vivo that were divalent (monospecific for MuSK). Fab-arm exchange with normal human IgG4 did not prevent the inhibitory effect of serum derived MuSK antibodies on AChR clustering in C2C12 mouse myotubes. The results suggest that a considerable proportion of MuSK IgG4 could already be Fab-arm exchanged in vivo. This was confirmed by isolating endogenous IgG4 MuSK antibodies containing both κ and λ light chains, i.e. hybrid IgG4 molecules. These new findings demonstrate that Fab-arm exchanged antibodies

  7. May positron emission tomography reveal ectopic or active thymus in preoperative evaluation of non-thymomatous myasthenia gravis?

    Science.gov (United States)

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

    2014-09-05

    In myasthenia gravis (MG) both native and ectopic thymic tissue containing germinal centers should show greater metabolism compared to adjacent tissues. We evaluated whether preoperative standardized uptake value (SUV) of 18fluoro-deoxy-glucose on Positron Emission Tomography (PET) might be increased and correlated with the presence of native or ectopic germinal centers. From 2005 to 2012 we performed extended thymectomy in 68 patients with non-thymomatous MG. All patients underwent PET-scan preoperatively and one-year postoperatively. SUVs were assessed in thymic and perithymic regions. Then it was matched with same-age, non-MG and non-neoplastic control group and finally correlated with presence of germinal centers in native thymus or in the ectopic tissue found in the surgical specimens. Mean SUV was significantly increased in MG patients compared to control group. Thymic SUV was significantly higher in presence of thymic germinal centers [3.5 (2.4-5.0) Vs 2.1 (1.4-2.5), p = 0.021] while perithymic SUV was significantly higher in presence of ectopic germinal centers [3.1 (2.7-3.5) Vs 1.3 (0.9-1.7), p = 0.001]. SUV was significantly correlated with MG score (rho = 0.289, p = 0.017) and marginally with antibodies anti-acetylcholine receptors (rho = 0.129, p = 0.05). At Kaplan Meier analysis, ectopic thymic tissue (p = 0.045) and ectopic germinal centers (p = 0.036) were significant predictors of complete stable remission, but preoperative dichotomized thymic (3.5 or more Vs less) (p = 0.083) and perithymic (2.1 or more Vs less) (p = 0.052) SUVs did not. Thymic and perithymic SUVs were significantly higher in patients with MG than non-MG and non-neoplastic patients. Thymic SUV was significantly correlated with the presence of germinal centers. Perithymic SUV resulted significantly correlated with the discovery of ectopic active thymic tissue. Neither thymic nor perithymic high SUVs predicted remission.

  8. pacientes con insuficiencia renal terminal

    Directory of Open Access Journals (Sweden)

    Karen Herrera Herrera

    2011-01-01

    Full Text Available La presente investigación fundamenta en la clínica psicoanalítica el estudio de dos casos de tres personas diagnosticadas con IRT que reciben tratamiento de hemodiálisis, en razón a que dadas las características y el aumento de los reportes que se presentan, ya esto se considera un problema de salud pública. El objetivo principal es describir las características dinámicas del proceso de duelo en pacientes con IRT en un centro de terapia renal de la ciudad de Cartagena. El procedimiento metodológico empleó un diseño de tipo cualitativo; la investigación se desarrolló con un diseño clínico mediante el estudio de casos, y fundamentada en la hermenéutica psicoanalítica. Todo esto respaldado en la historia clínica, la entrevista semiestructurada individual y familiar, los test proyectivos, test del dibujo de la figura humana Machover y TAT de Murray, para la debida integración de los análisis. Se concluye que predominan funciones fallidas de los progenitores y que son individuos provenientes de familias psicosomáticas, que utilizan la enfermedad para obtener un beneficio secundario.

  9. con bajo peso al nacer

    Directory of Open Access Journals (Sweden)

    Adriana Mora Antó

    2005-01-01

    Full Text Available Esta investigación dio cuenta de la relación entre el estilo de funcionamiento familiar, los patrones de crianza y las edades de desarrollo evolutivo en niños, nacidos con bajo peso. El estudio descriptivo correlacional se realizó con 41 niños y sus madres, aplicándose cuestionarios sobre funcionamiento familiar, prácticas de crianza y desarrollo infantil. Los resultados señalaron la existencia de un funcionamiento familiar caracterizado por una cohesión amalgamada y una adaptabilidad caótica, una disciplina complaciente, falta de control y de límites claros en la díada madre-hijo. Se trataba de familias monoparentales, donde la temprana edad de concepción, el madresolterismo y el apoyo de la familia extensa eran constantes. Las edades evolutivas registradas indicaron un desarrollo inferior a la edad cronológica, en la mayor parte de los casos; sin embargo, éstas tendieron a ser superiores al compararlas con la edades reales de los infantes. No se encontró una correlación estadísticamente significativa entre la edad de desarrollo y los diferentes factores del funcionamiento familiar para algunos de los rangos de edad considerados; sin embargo, no se lo descartó por completo, especialmente en lo referente al optimismo familiar

  10. An assessment of radioimmunoassay procedures for determination of anti-acetylcholine receptor antibodies in the sera of patients with myasthenia gravis

    International Nuclear Information System (INIS)

    Carter, B.; Harrison, R.; Lunt, G.G.; Morris, H.; Savage-Marengo, T.; Stephenson, F.A.

    1981-01-01

    A reproducible radioimmunoassay procedure for the determination of anti-acetylcholine receptor antibodies in the sera of patients with myasthenia gravis is described and examined in detail. The assay combines features of a number of methods previously outlined and allows repeat determinations of antibody titre in a given myasthenic serum sample with coefficient of variation 6%. The mean +- standard deviation for normal human serum anti-acetylcholine receptor antibodies was found by this procedure to be 0.024 +- 0.033 nmol/l α-bungarotoxin binding sites whereas the range for myasthenic patients was 0-139.14 nmol/l with a mean value of 7.55 nmol/l α-bungarotoxin binding sites. (author)

  11. [Myasthenia gravis, Graves-Basedow disease and other autoimmune diseases in patient with diabetes type 1 - APS-3 case report, therapeutic complications].

    Science.gov (United States)

    Klenczar, Karolina; Deja, Grażyna; Kalina-Faska, Barbara; Jarosz-Chobot, Przemysława

    2017-01-01

    Diabetes type 1(T1D) is the most frequent form of diabetes in children and young people, which essence is autoimmune destruction of pancreatic B cells islet. Co-occurrence of other autoimmune diseases is observed in children with T1D, the most often are: Hashimoto disease or coeliac disease. We report the case of the patient, who presents coincidence of T1D with other rare autoimmune diseases such as: Graves - Basedow disease, myasthenia gravis, vitiligo and IgA deficiency. All mentioned diseases significantly complicated both endocrine and diabetic treatment of our patient and they negatively contributed her quality of life. The clinical picture of the case allows to recognize one of the autoimmune polyendocrine syndromes: APS-3 and is associated with still high risk of developing another autoimmune disease. © Polish Society for Pediatric Endocrinology and Diabetology.

  12. Effect of intravenous immunoglobulin in Guilain-Barre syndrome, myasthenia gravis and chronic idiopathic demyelinative polyneuropathy, A survey in Imam Khomeini Hospital

    Directory of Open Access Journals (Sweden)

    Qaffarpoor M

    1999-09-01

    Full Text Available With retrospective evaluation of 44 patients suffering from Guilan-Barre Syndrome (GBS, Chronic Idiopathic Demtyelinative Polyradiculoneuropathy (CIDP and Myasthenia Gravis (MG treated with intravenous immunoglobulin, we found following results: 1 Initial symptoms of improvement on forth or fifth days. 2 Maximum recovery for CIDP and MG were after 16-24 and 3-11 days, respectively. 3 No major complication, but mild side effects in 32% of patients. 4 In patients with GBS one grade improvement achieved after 8-30 days. 5 Intravenous immunoglobulin (IVIG plus plasmapheresis had no advantages over IVIG alone. 6 No reasonable conclusion about relapsing rate and duration of response due to follow up restrictions.

  13. Myasthenia Gravis With Thymoma, Manifesting as AChR-Ab-Positive, Distinct Bulbar Palsy Accompanied by Dysgeusia: A Case Series and Review of Literature

    Directory of Open Access Journals (Sweden)

    Kai Zhu

    2018-04-01

    Full Text Available In this review, we summarized three cases of myasthenia gravis (MG with taste disorder and describe their clinical features in detail. Three MG patients presented with significant bulbar palsy symptoms, high AChR-Ab titers, and negative MuSK-Ab, were diagnosed with thymoma. Furthermore, we observed that dysgeusia could manifest earlier than the occurrence of typical MG symptoms, even predict a MG relapse or a myasthenic crisis in the course of MG. We believe that dysgeusia is a non-motor symptom of MG, which especially exists in MG patients with thymoma and serious bulbar palsy. Therefore, being alert to this symptom may facilitate the early diagnosis of MG and judge the progress of the disease.

  14. Guidelines for pre-clinical assessment of the acetylcholine receptor-specific passive transfer myasthenia gravis model - recommendations for methods and experimental designs

    Science.gov (United States)

    Kusner, Linda L.; Losen, Mario; Vincent, Angela; Lindstrom, Jon; Tzartos, Socrates; Lazaridis, Konstantinos; Martinez-Martinez, Pilar

    2015-01-01

    Antibodies against the muscle acetylcholine receptor (AChR) are the most common cause of myasthenia gravis (MG). Passive transfer of AChR antibodies from MG patients into animals reproduces key features of human disease, including antigenic modulation of the AChR, complement-mediated damage of the neuromuscular junction, and muscle weakness. Similarly, AChR antibodies generated by active immunization in experimental autoimmune MG models can subsequently be passively transferred to other animals and induce weakness. The passive transfer model is useful to test therapeutic strategies aimed at the effector mechanism of the autoantibodies. Here we summarize published and unpublished experience using the AChR passive transfer MG model in mice, rats and rhesus monkeys, and give recommendations for the design of preclinical studies in order to facilitate translation of positive and negative results to improve MG therapies. PMID:25743217

  15. CD1d(hi)CD5+ B cells expanded by GM-CSF in vivo suppress experimental autoimmune myasthenia gravis.

    Science.gov (United States)

    Sheng, Jian Rong; Quan, Songhua; Soliven, Betty

    2014-09-15

    IL-10-competent subset within CD1d(hi)CD5(+) B cells, also known as B10 cells, has been shown to regulate autoimmune diseases. Whether B10 cells can prevent or suppress the development of experimental autoimmune myasthenia gravis (EAMG) has not been studied. In this study, we investigated whether low-dose GM-CSF, which suppresses EAMG, can expand B10 cells in vivo, and whether adoptive transfer of CD1d(hi)CD5(+) B cells would prevent or suppress EAMG. We found that treatment of EAMG mice with low-dose GM-CSF increased the proportion of CD1d(hi)CD5(+) B cells and B10 cells. In vitro coculture studies revealed that CD1d(hi)CD5(+) B cells altered T cell cytokine profile but did not directly inhibit T cell proliferation. In contrast, CD1d(hi)CD5(+) B cells inhibited B cell proliferation and its autoantibody production in an IL-10-dependent manner. Adoptive transfer of CD1d(hi)CD5(+) B cells to mice could prevent disease, as well as suppress EAMG after disease onset. This was associated with downregulation of mature dendritic cell markers and expansion of regulatory T cells resulting in the suppression of acetylcholine receptor-specific T cell and B cell responses. Thus, our data have provided significant insight into the mechanisms underlying the tolerogenic effects of B10 cells in EAMG. These observations suggest that in vivo or in vitro expansion of CD1d(hi)CD5(+) B cells or B10 cells may represent an effective strategy in the treatment of human myasthenia gravis. Copyright © 2014 by The American Association of Immunologists, Inc.

  16. A Novel Approach to Reinstating Tolerance in Experimental Autoimmune Myasthenia Gravis Using a Targeted Fusion Protein, mCTA1–T146

    Directory of Open Access Journals (Sweden)

    Alessandra Consonni

    2017-09-01

    Full Text Available Reinstating tissue-specific tolerance has attracted much attention as a means to treat autoimmune diseases. However, despite promising results in rodent models of autoimmune diseases, no established tolerogenic therapy is clinically available yet. In the experimental autoimmune myasthenia gravis (EAMG model several protocols have been reported that induce tolerance against the prime disease-associated antigen, the acetylcholine receptor (AChR at the neuromuscular junction. Using the whole AChR, the extracellular part or peptides derived from the receptor, investigators have reported variable success with their treatments, though, usually relatively large amounts of antigen has been required. Hence, there is a need for better formulations and strategies to improve on the efficacy of the tolerance-inducing therapies. Here, we report on a novel targeted fusion protein carrying the immunodominant peptide from AChR, mCTA1–T146, which given intranasally in repeated microgram doses strongly suppressed induction as well as ongoing EAMG disease in mice. The results corroborate our previous findings, using the same fusion protein approach, in the collagen-induced arthritis model showing dramatic suppressive effects on Th1 and Th17 autoaggressive CD4 T cells and upregulated regulatory T cell activities with enhanced IL10 production. A suppressive gene signature with upregulated expression of mRNA for TGFβ, IL10, IL27, and Foxp3 was clearly detectable in lymph node and spleen following intranasal treatment with mCTA1–T146. Amelioration of EAMG disease was accompanied by reduced loss of muscle AChR and lower levels of anti-AChR serum antibodies. We believe this targeted highly effective fusion protein mCTA1–T146 is a promising candidate for clinical evaluation in myasthenia gravis patients.

  17. Conversando con...Momoyo Kaijima

    OpenAIRE

    Gómez Alonso, Carlos; Álvarez Isidro, Eva; Torres Barchino, Ana

    2017-01-01

    [ES] Momoyo Kaijima es profesora en la Facultad de Arte y Diseño de la Universidad de Tsukuba en la Prefectura de Ibaraki y profesora visitante en la ETH de Zürich, en Royal Academy of Fine Arts, en Rice School of Architecture y en Harvard GSD. A lo largo de los años, Atelier Bow Wow ha colaborado con Krešimir Rogina, arquitecto de Zagreb y socio de la firma internacional Penezic&Rogina, en la realización del Grožnjan International Summer School of Architecture, siendo Rogina el nexo indispen...

  18. Food irradiation - pros and cons

    International Nuclear Information System (INIS)

    1983-01-01

    The use of ionising radiation for food preservation is a much-disputed topic, both among experts and among consumers. Pros and cons of this issue were discussed in detail at the consumers' forum. Professor Dr. Johannes Friedrich Diehl, Director of the Institute for Biochemistry of the Food Research Centre, Karlsruhe, is a well-known supporter of the new method of food preservation; he sees advantages in the radiopreservation of food because, for example, losses due to inedibility are reduced, the danger of salmonellosis is decreased, just as the use of chemicals. He thinks this method to be without danger to health, shown by many years of experience. Opponents to food irradiation like Prof. Dr. Konrad Pfeilsticker, Professor for food science and food chemistry at the Bonn University deem the method to be unnecessary and raise the problem of qualitative changes caused in the food. In the course of the discussions, the pros and cons seemed to balance each other out. (orig./AJ) [de

  19. De paseo con los Bourbaki

    Directory of Open Access Journals (Sweden)

    Miquel Escudero

    2012-04-01

    Full Text Available Setenta y siete años después de la fundación del grupo Bourbaki, procedemos a una reflexión que puede ser útil para los estudiantes que acaso no sepan de su existencia, ni tan siquiera los de matemáticas. Sería interesante conocer que a este grupo se le debe el signo del vacío como conjunto. Con todo lo discutible que sea el método Bourbaki en su reinterpretación de la matemática, no cabe duda de su importante repercusión hasta el punto de que ha marcado una época. Hay un antes y un después tras su irrupción, ningún matemático de primera fila de la segunda mitad del siglo XX fue ajeno a su influjo, para encabezarlo o para reprobarlo. Comenzaron como una juvenil extravagancia, pero repleta de conocimientos y con decidida voluntad de aprehender el rigor de forma exhaustiva.

  20. Tratamiento del paciente con artrosis

    Directory of Open Access Journals (Sweden)

    Francisco Vargas Negrín

    2014-01-01

    Full Text Available El manejo terapéutico del paciente con artrosis tiene como objetivo disminuir la sintomatología dolorosa e inflamatoria, mejorar la capacidad funcional del paciente y la aplicación de intervenciones terapéuticas eficaces y lo más seguras posibles. Un enfoque centrado en el paciente implica su participación activa en el diseño del plan terapéutico y en la toma de decisiones informadas oportunas en todas las etapas de la enfermedad. La educación terapéutica, la actividad física y el ejercicio terapéutico junto con el control de peso, en caso de sobrepeso u obesidad, constituyen el núcleo central del tratamiento. Los autocuidados individuales y por los familiares son fundamentales en el control del día a día del paciente. El uso de terapias físicas, ayudas técnicas (bastón, etc. y de fármacos tipo analgésicos simples, opioides y antiinflamatorios tiene evidencias demostradas en el control del dolor, mejora la funcionalidad y la calidad de vida del paciente y una clara recomendación de uso en el tratamiento de la artrosis. La cirugía conservadora y la de reemplazo articular se indican en los casos en los que no se logran los objetivos terapéuticos en casos concretos.