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Primary Biliary Cirrhosis and Myasthenia Gravis are both autoimmune conditions, however, there are only rare case reports of their association. This is a case report of acetylcholine receptor antibody positive generalized myasthenia gravis in a female patient with antimitochondrial antibody positive, liver biopsy-confirmed primary biliary cirrhosis.

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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+MM0 x WIT groups (86.62 x 157.57, p<0.001) and PR+MM1 x WIT groups (73.93 x 157.57, p<0.001). Linear regression showed correlation between Hp levels and QMGSS (r=0.759, p<0.001). CONCLUSION: Our results suggest that Hp may be useful in clinical practice as a disease severity marker in MG.

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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, p<0.001) and PR+MM1xWIT groups (73.93x157.57, p<0.001). Linear regression showed correlation between Hp levels and QMGSS (r=0.759, p<0.001). CONCLUSION: Our results suggest that Hp may be useful in clinical practice as a disease severity marker in MG.

OBJECTIVO: 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. RESULTADOS: Observou-se diferença significativa entre os grupos CSR+MM0xWIT (86,62x157,57, p<0,001) e entre PR+MM1xWIT (73,93x157,57, p<0,001). A regressão linear mostrou correlação positiva entre os valores de Hp e os escores QMGSS (r=0,759, p<0,001). CONCLUSÃO: O estudo sugere que valores altos de Hp se correlacionaram a maior gravidade da MG.

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Myasthenia gravis is an autoimmune disease characterized by weakness and fatigue of the skeletal muscles of the face and extremities. Pregnancy is associated with 19-41% of exacerbations. We present a 19-year-old woman who was seen at the neurology clinic 10 days after her last cesarean section with diplopia and trouble swallowing liquids and solids for the previous 3 months. On examination she had slurred speech, bilateral palpebral ptosis, and mild droop of her shoulders. An arterial blood sample showed a PCO2 of 50 mmHg and a PO2 of 70 mmHg. The tensilon test was positive, Intravenous methylprednisolone and immunoglobulin and oral pyridostigmine effectively relieved her myasthenic signs and symptoms. Five assays for acetylcholine or anti-striational antibodies in her serum were negative. A test for muscle-specific tyrosine kinase antibody was positive. In 10-20% of patients with myasthenia gravis no antibodies to acetylcholine receptors are detectable. Forty percent of this group has an antibody to muscle-specific tyrosine kinase.

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Neuromuscular transmission, muscle electrical activity and muscle mechanical properties have been studied in mice after the intraperitoneal transference of sera from myasthenic patients. Measurements of mepp's amplitude appear to be a suitable method for myasthenia gravis diagnosis since a high percentage of the sera from these patients caused a decrease in mepp's amplitude. The increase of the premenstrum weakness in myasthenic patients did not appear to be associated with greater fall of mepp's amplitude, since serum obtained in the premenstruum and far from it produced similar results. Myasthenic serum did not modify the electrical properties of innervated muscle sarcolemma. The resting membrane potential and the action potential parameters remained unchanged. However, tetrodotoxin resistant-action potentials of denervated muscles was reduced by myasthenic serum, possibly in association with receptor endocytosis process induced by the immunoglobulin. Muscle mechanical properties did not show alterations, even in the presence of positive titer of anti-striated muscle antibody.

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La medición de la amplitud de los potenciales de placa miniatura (ppm) resultó ser un método útil para el diagnóstico de la miastenia grave, ya que un alto porcentaje de suero de estos pacientes provocó una disminución de la amplitud de los pp,. El aumento de la debilidad premenstrual en las pacientes miasténicas parece no estar asociado con una mayor caída de la amplitud de los ppm, ya que el suero obtenido en el período premenstural y lejos de este, indujeron resultados similares. El suero miasténico no modificó la actividad eléctrica del músculo inervado; el potencial de membrana y los parámetros del potencial de acción no mostraton variaciones. Sin embargo, cuando la transferencia pasiva se realizó en animales previamente desnervados, se redujo el número de potenciales de acción resistentes a la tetrodotoxina. Este efecto podría estar vinculado a la endocitosis de los receptores inducida por los anticuerpos. Las propiedades mecánicas del músculo no mostraron modificaciones en nuestro modelo experimental, aún en el caso en que el suero transferido contenía títulos positivos de anticuerpos anti-músculo estriado (AU)

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La medición de la amplitud de los potenciales de placa miniatura (ppm) resultó ser un método útil para el diagnóstico de la miastenia grave, ya que un alto porcentaje de suero de estos pacientes provocó una disminución de la amplitud de los pp,. El aumento de la debilidad premenstrual en las pacientes miasténicas parece no estar asociado con una mayor caída de la amplitud de los ppm, ya que el suero obtenido en el período premenstural y lejos de este, indujeron resultados similares. El suero miasténico no modificó la actividad eléctrica del músculo inervado; el potencial de membrana y los parámetros del potencial de acción no mostraton variaciones. Sin embargo, cuando la transferencia pasiva se realizó en animales previamente desnervados, se redujo el número de potenciales de acción resistentes a la tetrodotoxina. Este efecto podría estar vinculado a la endocitosis de los receptores inducida por los anticuerpos. Las propiedades mecánicas del músculo no mostraron modificaciones en nuestro modelo experimental, aún en el caso en que el suero transferido contenía títulos positivos de anticuerpos anti-músculo estriado

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A significant decline of CD3 cell detected by rosettes and a significant increased of B cell populations were observed. The total CD3+, helper CD4+ and suppressor CD8+ T-cell subsets showed no significant variation em relation to sex, age thymectomy and corticotherapy by monoclonal antibodies.

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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 elevaçäo significante na populaçäo de linfócito B e reduçäo nos linfócitos T totais CD3 + por rosáceas. Näo foram observadas modificaçöes nas subpopulaçöes celulares com timectomia e corticosteróides

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Twelve patients between 15 months and 13 years of age with clinical and pharmacological features of myasthenia gravis were studied. Repetitive nerve stimulation did not offer valuable information; the patients demonstrated either inspecific muscular decremental response or had normal behavior. Two clear groups of patients were identified after measurements of acetylcholine receptor (AChR) antibodies and MEPP amplitude recorded in the diaphragm of mice injected with sera from those patients. The first group included patients with positive AChR antibodies titers and decreased MEPP amplitude. The second one had negative AChR antibodies titers and MEPPs with normal amplitude. These data strongly suggest immunologic and non-immunologic mechanisms for the former and later respectively.

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Se analizaron 21 historias clinicas recuperadas, de los 28 pacientes con miastenia gravis tratados con timectomia en el Hospital San Juan de Dios durante el periodo comprendido entre enero de 1980 y diciembre de 1987. En esta serie, 17 de los pacientes fueron mujeres y 4 hombres cuyas edades oscilaban entre 14 y 53 anos. La timectomia se practico entre 1 y 12 anos despues de iniciada la enfermedad obteniendose mejoria o remision del cuadro clinico en la mayor parte de los casos. En todos los casos el diagnostico de la enfermedad fue hecho por el neurologo. Con base en las respuestas del paciente a las pruebas de fatigabilidad del ejercicio, la prueba de edrofonio (tension) y datos electromiograficos. En todos los casos se hicieron estudios inmunologicos complementarios y otros examenes de laboratorio, requeridos para aclarar la condicion individual del paciente. De 8 informes radiologicos preoperatorios de torax solo uno informa masa en el mediastino anterior, mientras que en 14 TAC solo se informa un timo normal. 14 de los 21 pacientes recibieron tratamiento preoperatorio con meostigmine solo o asociado con prednisolona. Durante el postoperatorio se presentaron 6 complicaciones del acto quirurgico y solo uno dependiente del cuadro miastenio. Se describen los hallazgos patologicos del timo, su evolucion postoperatoria y el seguimiento de la enfermedad, ademas se hace una revision de la literatura..

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