Plasmapheresis as preparatory method for thymectomy in myasthenia gravis

Seggia, José Carlos Brant; Abreu, Paulo; Takatani, M.

doi:10.1590/S0004-282X1995000300007

Abstracts

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.

myasthenia gravis; thymectomy; plasmapheresis

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 análise e manuseio destes pacientes. Obtivemos melhora clínica, da insuficiência respiratória, da força muscular e reduzimos também o custo da internação quando comparada aos pacientes em que a cirurgia foi realizada sem a plasmaferese e em alguns casos em que realizamos preparação com corticosteróides. Acreditamos que a plasmaferese deva ser considerada como método coadjuvante no tratamento cirúrgico desta grave afecção.

miastenia gravis; timectomia; plasmaferese

José Carlos Brant Seggia^I; Paulo Abreu^II; M. Takatani^III

^IHead of Clinic of the Neurologic Clinic

^IIStaff member of the Nephrology Clinic

^IIIResident at the Neurologic Clinic

SUMMARY

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.

Key words:myasthenia gravis, thymectomy, plasmapheresis.

RESUMO

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 análise e manuseio destes pacientes. Obtivemos melhora clínica, da insuficiência respiratória, da força muscular e reduzimos também o custo da internação quando comparada aos pacientes em que a cirurgia foi realizada sem a plasmaferese e em alguns casos em que realizamos preparação com corticosteróides. Acreditamos que a plasmaferese deva ser considerada como método coadjuvante no tratamento cirúrgico desta grave afecção.

Palavras-chave: miastenia gravis, timectomia, plasmaferese.

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REFERENCES

1. Appel SH, Elias SB, Chauvin P. The role of acetylcholine receptor antibodies in myasthenia gravis. Fed Proc 1979, 38: 2381-2385.

2. Barkas T, Mauron A, Roth B, Gabriel M, Tzartos S, Juillerat M, Alliod C, Ballivet M. Localization of the main immunogenic region and toxin binding site of the nicotinic acetylcholine receptor. Ann NY Acad Sci 1987, 505: 743-746.

3. Besinger UA, Toyka KV, Homberg M. Myasthenia gravis : long term correlation of binding and bungarotoxin-blocking antibodies against acetylcholine receptors with changes in disease severity. Neurology 1983, 33:1316-1321.

4. Campbell H, Bramwell E. Myatenia gravis . Brain 1900, 23: 277-336.

5. Chang CC, Lee CY. Electrophysiological study of neuromuscular blocking action of cobra neurotoxin. Br J Pharmacol Chemother 1966, 28: 172-181.

6. Chang CE, Lee CY. Isolation of neurotoxins from the venom of Bungarus multicinctus and their mode of neuromuscular blocking action. Arch Int Pharmacodyn Ther 1962, 144:241-257.

7. Cohen MS, Younger D. Aspects of the natural history of myasthenia gravis. Ann NY Acad Sci 1981, 377: 670-677.

8. Compston DAS, Vicent A, Newson-Davis J. Clinical, pathological, HLA antigen and immununological evidence for disease heterogeneity in myathenia gravis. Brain 1980,103: 579-601.

9. Dalakas MC, Rose JW, Paul J. Increased circulation of T lymphocytes bearing surface thymosin-a 1 in patients with myasthenia gravis : effects of thymectomy. Neurology 1983, 34: 144-149.

10. Dau PC, Lindstrom J, Cassei CK. Plasmapheresis and immunosuppressive therapy in myasthenia gravis . N Eng J Med 1977, 297: 1134-1140.

11. Engel AG, Sahashi K, Fumagalli G. The immunopathology of acquired myasthenia gravis. Ann NY Acad Sci 1981, 377: 158-174.

12. Fambrough DM, Drachman DB, Satyamurti S. Neuromuscular junction in myasthenia gravis: decreased acetylcholine receptors. Science 1973, 182:293-295.

13. Garlepp MJ, Kay PH, Dawkins RL. The diagnostic significance of autoantibodies to acetylcholine receptor. J Neuroimmunol 1982, 3:337-360.

14. Greer M, Schotland M. Myasthenia gravis in the newborn. Pediatrics 1960, 26: 101-108.

15. Ito Y, Miledi R, Vicent A, Newson-Davis J. Acetylcholine receptor s and endplate electrophysiology in myasthenia gravis . Brain 1978, 101: 345-368.

16. Seggia JCB, Abreu P. Plasmaferese em neurologia: análise crítica da indicação e protocolos. Arq Neuropsiquiatr 1992, 50: 324-328.

17. Keesey J, Buffkin D, Kebs D. Plasma exchange alone as therapy for myasthenia gravis .Ann NY Acad Sci

1981, 377: 729-743.

18. Lefvert AK. The human acetylcholine receptor antibody : studies of kinetic proprieties and the reaction with antiidiotypic antibodies. Ann NY Acad Sci 1981, 377: 125-142.

19. Newson-Davis J, Wilson SG, Vicent A. Long term effects of repeated plasma exchange in myasthenia gravis . Lancet 1979, 1: 464:-468.

20. Olarte MR, Schoenfeldt RS, Penn AS.. Effect of plasmapheresis in mysthenia gravis, 1978-1980. Ann NY Acad Sci 1981, 377:725-728.

21. Oppenheim H. Zur myastheniche Paralyse. Berlin: Karger, 1901.

22. Osserman KF, Genkins G. Studies in myasthenia gravis : review of a twenty year experience in over 1200 patients. Mt Sinai J Med 1961, 38: 197.

23. Simpson JA.. Myasthenia gravis: a new hypothesis . Scott Med J 1960, 4:419-436.

24. Tindall RS A. Humoral immunity in myasthenia gravis: biochemical characterization of acquired antireceptor antibodies and clinical correlations. Ann Neurol 1981, 10: 437-447.

25. Tzartos SJ, Seybold ME, Lindstrom JM. Specificities of antibodies to acetylcholine receptors in sera from myasthenia gravis patients measured by monoclonal antibodies. Proc Nat Acad Sci, USA

1982, 79:188-192.

26. Vicent A, Newson-Davis J. Acetylcholine receptor antibody characteristics in myasthenia gravis: I. Patients with generalized myasthenia or disease restricted to ocular muscles. Clin Exp Immunol 1982, 49: 257-265.

27. Vicent A, Newson-Davis J. Anti-acethylcholine receptors antibodies . J Neurol Neurosurg Psychiatry 1980, 43: 590-600.

Aceite: 7-fevereiro-1995.

Hospital dos Servidores do Estado, Rio de Janeiro, Brasil:

Dr. José Carlos Brant Seggia - Avenida Almirante Ary Parreiras 659 - 24230-321 Niterói RJ - Brasil.

1. Appel SH, Elias SB, Chauvin P. The role of acetylcholine receptor antibodies in myasthenia gravis. Fed Proc 1979, 38: 2381-2385.
2. Barkas T, Mauron A, Roth B, Gabriel M, Tzartos S, Juillerat M, Alliod C, Ballivet M. Localization of the main immunogenic region and toxin binding site of the nicotinic acetylcholine receptor. Ann NY Acad Sci 1987, 505: 743-746.
3. Besinger UA, Toyka KV, Homberg M. Myasthenia gravis : long term correlation of binding and bungarotoxin-blocking antibodies against acetylcholine receptors with changes in disease severity. Neurology 1983, 33:1316-1321.
4. Campbell H, Bramwell E. Myatenia gravis . Brain 1900, 23: 277-336.
5. Chang CC, Lee CY. Electrophysiological study of neuromuscular blocking action of cobra neurotoxin. Br J Pharmacol Chemother 1966, 28: 172-181.
6. Chang CE, Lee CY. Isolation of neurotoxins from the venom of Bungarus multicinctus and their mode of neuromuscular blocking action. Arch Int Pharmacodyn Ther 1962, 144:241-257.
7. Cohen MS, Younger D. Aspects of the natural history of myasthenia gravis. Ann NY Acad Sci 1981, 377: 670-677.
8. Compston DAS, Vicent A, Newson-Davis J. Clinical, pathological, HLA antigen and immununological evidence for disease heterogeneity in myathenia gravis. Brain 1980,103: 579-601.
9. Dalakas MC, Rose JW, Paul J. Increased circulation of T lymphocytes bearing surface thymosin-a 1 in patients with myasthenia gravis : effects of thymectomy. Neurology 1983, 34: 144-149.
10. Dau PC, Lindstrom J, Cassei CK. Plasmapheresis and immunosuppressive therapy in myasthenia gravis . N Eng J Med 1977, 297: 1134-1140.
11. Engel AG, Sahashi K, Fumagalli G. The immunopathology of acquired myasthenia gravis. Ann NY Acad Sci 1981, 377: 158-174.
12. Fambrough DM, Drachman DB, Satyamurti S. Neuromuscular junction in myasthenia gravis: decreased acetylcholine receptors. Science 1973, 182:293-295.
13. Garlepp MJ, Kay PH, Dawkins RL. The diagnostic significance of autoantibodies to acetylcholine receptor. J Neuroimmunol 1982, 3:337-360.
14. Greer M, Schotland M. Myasthenia gravis in the newborn. Pediatrics 1960, 26: 101-108.
15. Ito Y, Miledi R, Vicent A, Newson-Davis J. Acetylcholine receptor s and endplate electrophysiology in myasthenia gravis . Brain 1978, 101: 345-368.
16. Seggia JCB, Abreu P. Plasmaferese em neurologia: análise crítica da indicação e protocolos. Arq Neuropsiquiatr 1992, 50: 324-328.
18. Lefvert AK. The human acetylcholine receptor antibody : studies of kinetic proprieties and the reaction with antiidiotypic antibodies. Ann NY Acad Sci 1981, 377: 125-142.
19. Newson-Davis J, Wilson SG, Vicent A. Long term effects of repeated plasma exchange in myasthenia gravis . Lancet 1979, 1: 464:-468.
20. Olarte MR, Schoenfeldt RS, Penn AS.. Effect of plasmapheresis in mysthenia gravis, 1978-1980. Ann NY Acad Sci 1981, 377:725-728.
21. Oppenheim H. Zur myastheniche Paralyse. Berlin: Karger, 1901.
22. Osserman KF, Genkins G. Studies in myasthenia gravis : review of a twenty year experience in over 1200 patients. Mt Sinai J Med 1961, 38: 197.
23. Simpson JA.. Myasthenia gravis: a new hypothesis . Scott Med J 1960, 4:419-436.
24. Tindall RS A. Humoral immunity in myasthenia gravis: biochemical characterization of acquired antireceptor antibodies and clinical correlations. Ann Neurol 1981, 10: 437-447.
26. Vicent A, Newson-Davis J. Acetylcholine receptor antibody characteristics in myasthenia gravis: I. Patients with generalized myasthenia or disease restricted to ocular muscles. Clin Exp Immunol 1982, 49: 257-265.
27. Vicent A, Newson-Davis J. Anti-acethylcholine receptors antibodies . J Neurol Neurosurg Psychiatry 1980, 43: 590-600.

Plasmapheresis as preparatory method for thymectomy in myasthenia gravis

Plasmaferese como método preparatório de timectomia em miastenia grave

Publication Dates

Publication in this collection
09 Dec 2010
Date of issue
Sept 1995

This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.

[1] 1. Appel SH, Elias SB, Chauvin P. The role of acetylcholine receptor antibodies in myasthenia gravis. Fed Proc 1979, 38: 2381-2385.

[2] 2. Barkas T, Mauron A, Roth B, Gabriel M, Tzartos S, Juillerat M, Alliod C, Ballivet M. Localization of the main immunogenic region and toxin binding site of the nicotinic acetylcholine receptor. Ann NY Acad Sci 1987, 505: 743-746.

[3] 3. Besinger UA, Toyka KV, Homberg M. Myasthenia gravis : long term correlation of binding and bungarotoxin-blocking antibodies against acetylcholine receptors with changes in disease severity. Neurology 1983, 33:1316-1321.

[4] 4. Campbell H, Bramwell E. Myatenia gravis . Brain 1900, 23: 277-336.

[5] 5. Chang CC, Lee CY. Electrophysiological study of neuromuscular blocking action of cobra neurotoxin. Br J Pharmacol Chemother 1966, 28: 172-181.

[6] 6. Chang CE, Lee CY. Isolation of neurotoxins from the venom of Bungarus multicinctus and their mode of neuromuscular blocking action. Arch Int Pharmacodyn Ther 1962, 144:241-257.

[7] 7. Cohen MS, Younger D. Aspects of the natural history of myasthenia gravis. Ann NY Acad Sci 1981, 377: 670-677.

[8] 8. Compston DAS, Vicent A, Newson-Davis J. Clinical, pathological, HLA antigen and immununological evidence for disease heterogeneity in myathenia gravis. Brain 1980,103: 579-601.

[9] 9. Dalakas MC, Rose JW, Paul J. Increased circulation of T lymphocytes bearing surface thymosin-a 1 in patients with myasthenia gravis : effects of thymectomy. Neurology 1983, 34: 144-149.

[10] 10. Dau PC, Lindstrom J, Cassei CK. Plasmapheresis and immunosuppressive therapy in myasthenia gravis . N Eng J Med 1977, 297: 1134-1140.

[11] 11. Engel AG, Sahashi K, Fumagalli G. The immunopathology of acquired myasthenia gravis. Ann NY Acad Sci 1981, 377: 158-174.

[12] 12. Fambrough DM, Drachman DB, Satyamurti S. Neuromuscular junction in myasthenia gravis: decreased acetylcholine receptors. Science 1973, 182:293-295.

[13] 13. Garlepp MJ, Kay PH, Dawkins RL. The diagnostic significance of autoantibodies to acetylcholine receptor. J Neuroimmunol 1982, 3:337-360.

[14] 14. Greer M, Schotland M. Myasthenia gravis in the newborn. Pediatrics 1960, 26: 101-108.

[15] 15. Ito Y, Miledi R, Vicent A, Newson-Davis J. Acetylcholine receptor s and endplate electrophysiology in myasthenia gravis . Brain 1978, 101: 345-368.

[16] 16. Seggia JCB, Abreu P. Plasmaferese em neurologia: análise crítica da indicação e protocolos. Arq Neuropsiquiatr 1992, 50: 324-328.

[18] 18. Lefvert AK. The human acetylcholine receptor antibody : studies of kinetic proprieties and the reaction with antiidiotypic antibodies. Ann NY Acad Sci 1981, 377: 125-142.

[19] 19. Newson-Davis J, Wilson SG, Vicent A. Long term effects of repeated plasma exchange in myasthenia gravis . Lancet 1979, 1: 464:-468.

[20] 20. Olarte MR, Schoenfeldt RS, Penn AS.. Effect of plasmapheresis in mysthenia gravis, 1978-1980. Ann NY Acad Sci 1981, 377:725-728.

[21] 21. Oppenheim H. Zur myastheniche Paralyse. Berlin: Karger, 1901.

[22] 22. Osserman KF, Genkins G. Studies in myasthenia gravis : review of a twenty year experience in over 1200 patients. Mt Sinai J Med 1961, 38: 197.

[23] 23. Simpson JA.. Myasthenia gravis: a new hypothesis . Scott Med J 1960, 4:419-436.

[24] 24. Tindall RS A. Humoral immunity in myasthenia gravis: biochemical characterization of acquired antireceptor antibodies and clinical correlations. Ann Neurol 1981, 10: 437-447.

[26] 26. Vicent A, Newson-Davis J. Acetylcholine receptor antibody characteristics in myasthenia gravis: I. Patients with generalized myasthenia or disease restricted to ocular muscles. Clin Exp Immunol 1982, 49: 257-265.

[27] 27. Vicent A, Newson-Davis J. Anti-acethylcholine receptors antibodies . J Neurol Neurosurg Psychiatry 1980, 43: 590-600.