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

Rey, Roberto D.; Sanz, Olga P.; Fernandez, José M.; Rey, Raul C.; Panizza, Marcela; Lucilli, Norberto; Astudillo, Miguel A.; Diaz, Gloria; Villegas, Alejandro H.; Sica, Roberto E. P.

doi:10.1590/S0004-282X1990000300002

Resúmenes

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.

Between 1974 and 1987 we have examined 50 patients with the diagnosis of myasthenia gravis. Female preponderance (2,5: 1) was found. Also, it was observed that most of the patients were aged between 20 and 49 years. Beside the clinical examination, the following tests were performed: (1) edrofonium test, (2) supramaximal repetitive nerve stimulation, (3) serum acetylcholine antibodies titers and (4) intraperitoneal passive transference of patient's sera to mice and recording of meepp's amplitude in the phrenic-diaphragm preparation in vitro. These four tests gave positive values for myasthenia in 90 to 100% of the cases. Thymus radiological examination was carried out by pneumomediastinography, which proved to correlate with the hsitological picture of the gland, and computed tomography, which disclosed some discrepances with the histology. Treatment was based on anticholinesterase drugs, corticosteroids and thymectomy, being the corticosteroids the most valuable therapeutical tool. Nine patients treated with steroids disclosed transitory worsening of their signs and symptoms at very early stages after onset of corticosteroid therapy, 6 of them had a disfavorable course in their follow-up. This observation seem to have value in the early prognosis of the disease.

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

Diagnosis and treatment of myasthenia gravis: study of an inpatient population

Roberto D. Rey^I; Olga P. Sanz^I; José M. Fernandez^II; Raul C. Rey^I; Marcela Panizza^I; Norberto Lucilli^II; Miguel A. Astudillo^II; Gloria Diaz^III; Alejandro H. Villegas^II; Roberto E. P. Sica^I

^IDivision Neurologia - Hospital J.M. Ramos Mejia

^IIDivision «B» Cirugia - Hospital J.M. Ramos Mejia

^IIIDivision Diagnóstico por Imagenes - Hospital J.M. Ramos Mejia

RESUMEN

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.

SUMMARY

Between 1974 and 1987 we have examined 50 patients with the diagnosis of myasthenia gravis. Female preponderance (2,5: 1) was found. Also, it was observed that most of the patients were aged between 20 and 49 years. Beside the clinical examination, the following tests were performed: (1) edrofonium test, (2) supramaximal repetitive nerve stimulation, (3) serum acetylcholine antibodies titers and (4) intraperitoneal passive transference of patient's sera to mice and recording of meepp's amplitude in the phrenic-diaphragm preparation in vitro. These four tests gave positive values for myasthenia in 90 to 100% of the cases. Thymus radiological examination was carried out by pneumomediastinography, which proved to correlate with the hsitological picture of the gland, and computed tomography, which disclosed some discrepances with the histology. Treatment was based on anticholinesterase drugs, corticosteroids and thymectomy, being the corticosteroids the most valuable therapeutical tool. Nine patients treated with steroids disclosed transitory worsening of their signs and symptoms at very early stages after onset of corticosteroid therapy, 6 of them had a disfavorable course in their follow-up. This observation seem to have value in the early prognosis of the disease.

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Dr. Roberto D. Rey - Division Neurologia, Hospital Ramos Mejia - Urquiza 609 - 1221 Buenos Aires - Argentina.

1. Buckingham JM, Howard FM, Bernatz PhE, Payne S, Harrison EG, O'Brien PC, Weiland LH - The value of thymectomy in myasthenia gravis. Ann Surg 184:453, 1976.
2. Deschaux P, Rouabhia M - The thymus: key organ between endocrinologic and immunologic systems. In Jancovic BD, Marcovik BM, Spector NH (eds): Neuroimmune Interactions (Proc Second Internat Workshop Neuroimmunemodulation). Ann NY Acad Sci, New York, 1987, pg 49.
3. Dias-Tosta E, Morato-Fernandes RN - Miastenia grave: tratamento com timectomia, corticoide e plasmaierese. Arq Neuro-Psiquiat (São Paulo) 47:39, 1989.
4. Drachman DB, Adams RN, Stanley EF, Pestronik A - Mechanism of acetylcholine receptor loss in myastenia gravis. J Neurol Neurosurg Psychiat 43:601, 1980.
5. Engel AG - Myasthenia gravis and myasthenic syndromes. Ann Neurol 16:519, 1984.
6. Fambrough DM, Drachman DB, Satyamurti S - Neuromuscular junction in myasthenia gravis: decreased acetylcholine receptors. Science 182:293, 1973.
7. Garcia Erro MI, Saccolitti M, Losavio A, Rey RD, Arberas C, Taratuto A, Muchnik S, Sica REP. XVIII Congreso Argentino de Neurologia. Mendoza, 1988.
8. Garcia Erro MI, Sica REP, Losavio A, Muchnik S, Arroyo H - Tools to differentiate immunologic and non-immunologic myasthenia gravis in infancy. Neuropediatrics 19:92, 1988.
9. Geenen V, Legros JJ, Franchimont P, Defresne MP, Boniver J, Ivell R, Richter D - Synthesis of vasopressin and oxytocin in human thymic epithelium. In Jankovic BD, Markovic BM, Spector NH (eds): Neuroimmune Interactions (Proc Second Internat Workshop Neuroimmunemodulation). Ann NY Acad Sei, New York, 1987, pg 56.
10. Hadden JW - Neuroendocrine modulation of the thymus-dependent immune system: agonist and mechanism. In Jankovic BD, Markovic BM, Spector NH (eds): Neuroimmune Interactions (Proc Second Internat Workshop Neuroimmunemodulation). Ann NY Acad Sci, New York, 1987, pg 39.
11. Lindstrom JM, Seybold ME, Lennon VA, Whittingham S, Duane DD - Antibody to acetylcholine receptor in myasthenia gravis: prevalence, clinical correlates and diagnostic values. Neurology 26:1054, 1976.
12. Losavio A, Panizza M, Sica REP, Muchnik S - Transmisión neuromuscular y electrofisiología clínica en miastenia grave generalizada y ocular. XXIX Reunion S.A.I.C. Mar del Plata, 1984.
13. Mann JD, Johns TR, Campa JF - Long-term administration of corticosteroids in myasthenia gravis. Neurology 26:729, 1976.
14. Mossman S, Vincent A, Newsom-Davis J - Myasthenia gravis without acetylcholine-receptor antibody: a distinct disease entity. Lancet 1:658, 1986.
15. Oosterhuis HJGH, Newsom-Davis J, Wokke JHJ, Molenaar PC, Weerden TVM, Oen BS, Jennekens FGI, Veldman H, Vincent A, Wray DW, Prior C, Murray NMF - The slow channel syndrome: two new cases. Brain 110:1061, 1987.
16. Osserman KE, Genkins G - Studies in myasthenia gravis: review of a twenty-year experience in over 1200 patients. Mt Sinai JM 38:497, 1971.
17. Roncoroni AJ, Herrera MR, Rosemberg M - Timectomia en la miastenia gravis. Medicina (Buenos Aires) 38:9, 1978.
18. Rosemberg M, Herrera MR, Jauregui - «Recidiva tímica en la miastenia gravis>>. Am J Med 74:78, 1983.
19. Swift TR - Disorders of neuromuscular transmission other than myasthenia gravis. Muscle & Nerve 4:334, 1981.
20. Vincent A, Cull-Candy SG, Newsom-Davis J, Trautman A, Molenaar PC, Polak RL - Congenital myasthenia: end plate acetylcholine receptors and electrophysiology in five cases. Muscle & Nerve 4:306, 1981.
21. Vincent A, Newsom-Davis J - Anti-acetylcholine receptor antibodies. J Neurol Neurosurg Psychiat 43:590, 1980.

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[1] 1. Buckingham JM, Howard FM, Bernatz PhE, Payne S, Harrison EG, O'Brien PC, Weiland LH - The value of thymectomy in myasthenia gravis. Ann Surg 184:453, 1976.

[2] 2. Deschaux P, Rouabhia M - The thymus: key organ between endocrinologic and immunologic systems. In Jancovic BD, Marcovik BM, Spector NH (eds): Neuroimmune Interactions (Proc Second Internat Workshop Neuroimmunemodulation). Ann NY Acad Sci, New York, 1987, pg 49.

[3] 3. Dias-Tosta E, Morato-Fernandes RN - Miastenia grave: tratamento com timectomia, corticoide e plasmaierese. Arq Neuro-Psiquiat (São Paulo) 47:39, 1989.

[4] 4. Drachman DB, Adams RN, Stanley EF, Pestronik A - Mechanism of acetylcholine receptor loss in myastenia gravis. J Neurol Neurosurg Psychiat 43:601, 1980.

[5] 5. Engel AG - Myasthenia gravis and myasthenic syndromes. Ann Neurol 16:519, 1984.

[6] 6. Fambrough DM, Drachman DB, Satyamurti S - Neuromuscular junction in myasthenia gravis: decreased acetylcholine receptors. Science 182:293, 1973.

[7] 7. Garcia Erro MI, Saccolitti M, Losavio A, Rey RD, Arberas C, Taratuto A, Muchnik S, Sica REP. XVIII Congreso Argentino de Neurologia. Mendoza, 1988.

[8] 8. Garcia Erro MI, Sica REP, Losavio A, Muchnik S, Arroyo H - Tools to differentiate immunologic and non-immunologic myasthenia gravis in infancy. Neuropediatrics 19:92, 1988.

[9] 9. Geenen V, Legros JJ, Franchimont P, Defresne MP, Boniver J, Ivell R, Richter D - Synthesis of vasopressin and oxytocin in human thymic epithelium. In Jankovic BD, Markovic BM, Spector NH (eds): Neuroimmune Interactions (Proc Second Internat Workshop Neuroimmunemodulation). Ann NY Acad Sei, New York, 1987, pg 56.

[10] 10. Hadden JW - Neuroendocrine modulation of the thymus-dependent immune system: agonist and mechanism. In Jankovic BD, Markovic BM, Spector NH (eds): Neuroimmune Interactions (Proc Second Internat Workshop Neuroimmunemodulation). Ann NY Acad Sci, New York, 1987, pg 39.

[11] 11. Lindstrom JM, Seybold ME, Lennon VA, Whittingham S, Duane DD - Antibody to acetylcholine receptor in myasthenia gravis: prevalence, clinical correlates and diagnostic values. Neurology 26:1054, 1976.

[12] 12. Losavio A, Panizza M, Sica REP, Muchnik S - Transmisión neuromuscular y electrofisiología clínica en miastenia grave generalizada y ocular. XXIX Reunion S.A.I.C. Mar del Plata, 1984.

[13] 13. Mann JD, Johns TR, Campa JF - Long-term administration of corticosteroids in myasthenia gravis. Neurology 26:729, 1976.

[14] 14. Mossman S, Vincent A, Newsom-Davis J - Myasthenia gravis without acetylcholine-receptor antibody: a distinct disease entity. Lancet 1:658, 1986.

[15] 15. Oosterhuis HJGH, Newsom-Davis J, Wokke JHJ, Molenaar PC, Weerden TVM, Oen BS, Jennekens FGI, Veldman H, Vincent A, Wray DW, Prior C, Murray NMF - The slow channel syndrome: two new cases. Brain 110:1061, 1987.

[16] 16. Osserman KE, Genkins G - Studies in myasthenia gravis: review of a twenty-year experience in over 1200 patients. Mt Sinai JM 38:497, 1971.

[17] 17. Roncoroni AJ, Herrera MR, Rosemberg M - Timectomia en la miastenia gravis. Medicina (Buenos Aires) 38:9, 1978.

[18] 18. Rosemberg M, Herrera MR, Jauregui - «Recidiva tímica en la miastenia gravis>>. Am J Med 74:78, 1983.

[19] 19. Swift TR - Disorders of neuromuscular transmission other than myasthenia gravis. Muscle & Nerve 4:334, 1981.

[20] 20. Vincent A, Cull-Candy SG, Newsom-Davis J, Trautman A, Molenaar PC, Polak RL - Congenital myasthenia: end plate acetylcholine receptors and electrophysiology in five cases. Muscle & Nerve 4:306, 1981.

[21] 21. Vincent A, Newsom-Davis J - Anti-acetylcholine receptor antibodies. J Neurol Neurosurg Psychiat 43:590, 1980.

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

Diagnosis and treatment of myasthenia gravis: study of an inpatient population

Resúmenes

Fechas de Publicación