Miastenia gravis y el timo: pasado, presente y futuro
Palabras clave:
miastenia gravis, timo, timectomía, cirugía torácica asistida por videoResumen
La miastenia gravis es una enfermedad neuromuscular autoinmune caracterizada por debilidad y fatiga muscular. Ha sido reconocida desde hace más de tres siglos, pero sólo hasta el siglo XX se desarrollaron intervenciones terapéuticas encaminadas a aliviar los síntomas y alterar el curso clínico de la enfermedad. En la fisiopatología, se destaca el papel del timo como órgano central que contiene todos los elementos necesarios para iniciar y montar una respuesta inmune contra antígenos propios, particularmente, el receptor de acetilcolina en la placa motora. Debido a esto, la timectomía extendida aparece como una alternativa terapéutica que logra la remisión completa de la enfermedad hasta en 75% de los pacientes y la mejoría hasta de 99%.
Sin embargo, el abordaje abierto convencional (esternotomía) es bastante agresivo, por lo que, en los últimos años, la cirugía de tórax ha desarrollado técnicas quirúrgicas mediante toracoscopia asistida por video que permiten realizar el mismo procedimiento con menos complicaciones y mejores resultados cosméticos.
Debido al beneficio hasta ahora mostrado, se debe ofrecer el tratamiento quirúrgico a todos los pacientes con miastenia gravis generalizada y a una buena proporción de los que padecen síntomas oculares.
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Referencias bibliográficas
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2. CONTI-FINE BM., MILANI M., KAMINSKI HJ. Myasthenia gravis: past, present, and future. J Clin Invest. 2006;116:2843-54.
3. FLACHENECKER P. Epidemiology of neuroimmunological diseases. J Neurol. 2006;253(Suppl.5):V2-8. Erratum in: J Neurol. 2008;255:308.
4. KALB B., MATELL G., PIRSKANEN R., LAMBE M. Epidemiology of myasthenia gravis: a population-based study in Stockholm, Sweden. Neuroepidemiology. 2002;21:221-5.
5. OÖPIK M., PUKSA L., LÜÜS SM., KAASIK AE., JAKOBSEN J. Clinical and laboratory-reconfirmed myasthenia gravis: a population-based study. Eur J Neurol. 2008;15:246-52.
6. BATEMAN KJ., SCHINKEL M., LITTLE F., LIEBENBERG L., VINCENT A., HECKMANN JM. Incidence of seropositive myasthenia gravis in Cape Town and South Africa. S Afr Med J. 2007;97:959-62.
7. MANTEGAZZA R., BEGHI E., PAREYSON D., ANTOZZI C., PELUCHETTI D., SGHIRLANZONI A., et al. A multicentre follow-up study of 1152 patients with myasthenia gravis in Italy. J Neurol. 1990;237:339-44.
8. ZHANG X., YANG M., XU J., ZHANG M., LANG B., WANG W., et al. Clinical and serological study of myasthenia gravis in HuBei Province, China. J Neurol Neurosurg Psychiatry. 2007;78:386-90.
9. SÁNCHEZ JL., URIBE CS., FRANCO A., JIMÉNEZ ME., ARCOS-BURGOS M., PALACIO LG. Prevalencia de la miastenia gravis en Antioquia, Colombia. Rev Neurol. 2002;34:1010-2.
10. NIKS EH., KUKS JB., VERSCHUUREN JJ. Epidemiology of myasthenia gravis with anti-muscle specific kinase antibodies in The Netherlands. J Neurol Neurosurg Psychiatry. 2007;78:417-8.
11. OWE JF., DALTVEIT AK., GILHUS NE. Causes of death among patients with myasthenia gravis in Norway between 1951 and 2001. J Neurol Neurosurg Psychiatry. 2006;77:203-7.
12. HILL M., BEN-SHLOMO Y. Neurological care and risk of hospital mortality for patients with myasthenia gravis in England. J Neurol Neurosurg Psychiatry. 2008;79:421-5.
13. VINCENT A. Immunology of disorders of neuromuscular transmission. Acta Neurol Scand. 2006;183(Suppl.):1-7.
14. RAGHEB S., LISAK RP. The thymus and myasthenia gravis. Chest Surg Clin N Am. 2001;11:311-27.
15. CASTELLANO JE., BENITO MA., RAMÍREZ R. Receptores nicotínicos neuronales. Rev Fac Med. 2001;49:155-61.
16. RAICA M., CIMPEAN AM., RIBATTI D. Myasthenia gravis and the thymus gland. A historical review. Clin Exp Med. 2008;8:61-4.
17. YOUNGER DS., RAKSADAWAN N. Medical therapies in myasthenia gravis. Chest Surg Clin N Am. 2001;11:329-36.
18. LEVINSON AI., SONG D., GAULTON G., ZHENG Y. The intrathymic pathogenesis of myasthenia gravis. Clin Dev Immunol. 2004;11:215-20.
19. MELMS A., LUTHER C., STOECKLE C., PÖSCHEL S., SCHROTH P., VARGA M., et al. Thymus and myasthenia gravis: antigen processing in the human thymus and the consequences for the generation of autoreactive T cells. Acta Neurol Scand. 2006;183(Suppl.):12-3.
20. HOHLFELD R, WEKERLE H. Reflections on the “intrathymic pathogenesis” of myasthenia gravis. J Neuroimmunol. 2008;201- 202:21-7.
21. YOUNGER DS., RAKSADAWAN N. Medical therapies in myasthenia gravis. Chest Surg Clin N Am. 2001;11:329-36.
22. BIRD SJ. Diagnosis of myasthenia gravis. UpToDate 2008;16.2.
23. JARETZKI A 3RD., BAROHN RJ., ERNSTOFF RM., KAMINSKI HJ., KEESEY JC., PENN AS., et al. Myasthenia gravis: recommendations for clinical research standards. Task Force of the Medical Scientific Advisory Board of the Myasthenia Gravis Foundation of America. Neurology. 2000;55:16-23.
24. MEHNDIRATTA MM., KUNTZER T., PANDEY S. Anticholinesterase treatment for myasthenia gravis (Protocol). Cochrane Database Syst Rev. 2008;(1):CD006986.
25. SCHNEIDER-GOLD C., GAJDOS P., TOYKA KV., HOHLFELD RR. Corticosteroids for myasthenia gravis. Cochrane Database Syst Rev. 2005;(2):CD002828.
26. HART IK., SATHASIVAM S., SHARSHAR T. Immunosuppressive agents for myasthenia gravis. Cochrane Database Syst Rev. 2007;(4):CD005224.
27. GAJDOS P., CHEVRET S., TOYKA K. Plasma exchange for myasthenia gravis. Cochrane Database Syst Rev. 2002;(4):CD002275.
28. GAJDOS P., CHEVRET S., TOYKA K. Intravenous immunoglobulin for myasthenia gravis. Cochrane Database Syst Rev. 2008;(1):CD002277.
29. GROB D., BRUNNER N., NAMBA T., PAGALA M. Lifetime course of myasthenia gravis. Muscle Nerve. 2008;37:141-9. 30. MASAOKA A. Extended trans-sternal thymectomy for myasthenia gravis. Chest Surg Clin N Am. 2001;11:369-87.
31. BLALOCK A., MASON MF., MORGAN HJ., RIVEN SS. Myasthenia gravis and tumors of the thymic region. Report of a case in which the tumor was removed. Ann Surg. 1939;110:544-61.
32. BLALOCK A. Thymectomy in the treatment of myasthenia gravis: report of 20 cases. J Thorac Surg. 1944;13:316-39.
33. HARVEY AM., LILIENTHAL JL., TALBOT SA. Observations on the nature of myasthenia gravis. The effect of thymectomy on neuromuscular transmission. J Clin Invest. 1942;21:579-88.
34. KEYNES G. The results of thymectomy in myasthenia gravis. BMJ. 1949;2:611-6. 35. KIRSCHNER PA. Studies in myasthenia gravis. Transcervical total thymectomy. JAMA. 1969;209:906-10.
36. MASAOKA A., NAGAOKA Y., KOTAKE Y. Distribution of thymic tissue at the anterior mediastinum. Current procedures in thymectomy. J Thorac Cardiovasc Surg. 1975;70:747-54.
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Publicado
2009-12-20
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Tapias-Vargas, L.; Tapias-Vargas, L. F.; Tapias, L. Miastenia Gravis Y El Timo: Pasado, Presente Y Futuro. Rev Colomb Cir 2009, 24, 269-282.
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