Miastenia grave com resposta eletrográfica de síndrome miastênica: relato de um caso

Werneck, Lineu Cesar; Bittencourt, Paulo Cesar Trevisol; Nóvak, Edison Matos

doi:10.1590/S0004-282X1985000200012

Resumos

Relato de caso de paciente de 61 anos de idade, com história progressiva de astenia, episódios de disfagia, disfonia e diplopia que flutuavam conforme a atividade, que respondeu ao uso de anticolinesterásicos e corticoesteróides. A investigação eletrográfica foi típica de síndrome miastênica (Eaton-Lambert). Investigação radiológica e laboratorial repetida três vezes em período de 7 anos procurando neoplasia foi sempre negativa. Biópsia de músculo revelou atrofia de fibras do tipo II. Testes de estimulação repetitivos em três ocasiões diferentes no período de 7 anos, foram compatíveis a síndrome miastênica (incremento de até 418%) e em uma ocasião foi típico de miastenia grave (decremento abaixo de 20%). São discutidos os achados eletrofisiológicos em relação aos da literatura.

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 corticoesteroids. The repetitive estimulation 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 estimulation tests repeated three times, was typical of myasthenia gravis in one ocasion 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.

Miastenia grave com resposta eletrográfica de síndrome miastênica. Relato de um caso

Myasthenia gravis with electro graphic findings of myasthenic syndrome. A case report

Lineu Cesar Werneck^I; Paulo Cesar Trevisol Bittencourt^II; Edison Matos Nóvak^I

^IProfessor Assistente de Neurologia. Departamento de Clínica Médica, Universidade Federal do Paraná

^IIResidente de Neurologia. Departamento de Clínica Médica, Universidade Federal do Paraná

RESUMO

Relato de caso de paciente de 61 anos de idade, com história progressiva de astenia, episódios de disfagia, disfonia e diplopia que flutuavam conforme a atividade, que respondeu ao uso de anticolinesterásicos e corticoesteróides. A investigação eletrográfica foi típica de síndrome miastênica (Eaton-Lambert). Investigação radiológica e laboratorial repetida três vezes em período de 7 anos procurando neoplasia foi sempre negativa. Biópsia de músculo revelou atrofia de fibras do tipo II. Testes de estimulação repetitivos em três ocasiões diferentes no período de 7 anos, foram compatíveis a síndrome miastênica (incremento de até 418%) e em uma ocasião foi típico de miastenia grave (decremento abaixo de 20%). São discutidos os achados eletrofisiológicos em relação aos da literatura.

SUMMARY

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 corticoesteroids. The repetitive estimulation 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 estimulation tests repeated three times, was typical of myasthenia gravis in one ocasion 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.

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Trabalho realizado na Especialidade de Neurologia, Departamento de Clínica Médica, Universidade Federal do Paraná.

Departamento de Clínica Médica, Especialidade de Neurologia, Hospital de Clínicas da Universidade Federal do Paraná - Rua General Carneiro, 180 - 80000, Curitiba, PR - Brasil.

1. BOIARDI, A.; BUSSONE, G. & NEFRI, S. - Alternating myasthenia and myastheniform syndrome in the same subject. J. Neurol. 220:57, 1979.
2. BROWN, J.W.; NELSON, J.R. & JERMANN, C.Jr. - Sjogren's syndrome with myopathic and myasthenic features. Bull. Los Angeles neurol. Soc. 33:9, 1968.
3. DAHLS, D.S. & SATO, S. - Unusual myasthenic state in a teen-age boy. Neurology (Minneapolis) 24:897, 1974.
4. DELL'OSSO, L.F.; AYYAR, D.R.; DARÓFF, R.B. & ABEL, L.A. - Edrophonium test in Eaton-Lambert syndrome: quantitative oculography. Neurology 33:1157, 1983.
5. DRACHMANN, D.B. - Myasthenia gravis. N. Engl. J. Med. 298:136, 1978.
6. EATON, L.M. & LAMBERT, E.H. - Electromyographic and electric stimulation of nerves in diseases of motor unit. Observations on myasthenic syndrome associated with malignant tumors. J. amer. med. Assoc. 163:1117, 1957.
7. ELMRVIST, D. & LAMBERT, E.H. - Detailed analysis of neuromuscular transmission in a patient with myasthenic syndrome sometimes associated with bronchogenic carcinoma. Mayo Clin. Proc. 43:689, 1968.
8. ENGEL, A.G.; LAMBERT, E.H. & HOWARD, F.M. - Immune complexes (IgG and C3) at the motor end-plate in myasthenia gravis. Ultrastructural and light microscopic localization and electrophysiologic correlations. Mayo Clinic. Proc. 52:267, 1977.
9. ENGEL, A.G.; LINDSTROM, J.M.; LAMBERT, E.H. & LENNON, V.A. - Ultrastructural localization of the acetylcholine receptor in myasthenia gravis and its experimental autoimune model. Neurology (Minneapolis) 27:307, 1977.
10. ENGEL, A.G. & SANTA, T. - Histometric analysis of the ultrastructure of the neuro-muscular junction in myasthenia gravis and in myasthenic syndrome. Ann. N.Y. Acad. Sci. 183:35, 1971.
11. FAMBROUGH, D.M.; DRACHMAN, D.B.; STARJAMURTI, S. - Neuromuscular junction in myasthenia gravis: decreased acetylcholine receptors. Science 182:293, 1973.
12. FETTBL, M.R.; SHIN, H.S.; PENN, A.S.; LOVELACE, R.E. & ROWLAND, L.P. - Combined Eaton-Lambert Syndrome and myasthenia gravis. Neurology 28:398, 1978.
13. GUTMAN, L.; CROSBY, T.W.; TAKAMORI, M. & MARTIN, J.D. - The Eaton-Lambert syndrome and autoimmune disorders. Amer. J. Med. 53:354, 1972.
14. GUTMAN, L. & PRATT, I. - Pathophysiologic aspects of human botulism. Arch. Neurol. (Chicago) 33:175, 1976.
15. LAMBERT, E.H.; ROOKE, E.D. - Myasthenic state and lung cancer. In L. BRAIN & F.H. MORRIS Jr.: The Remote Effects of Cancer on the Nervous System. Grune & Stratton, New York, 1965.
16. LANG, B.; NEWSON-DAVIS, J.; WRAY, D. & VINCENT, A. - Autoimmune aetiology for myasthenic (Eaton-Lambert) syndrome. Lancet 2:224, 1981.
17. MAYER, R.F. & WILLIAMS, I.R. - Incrementing responses in myasthenia gravis. Arch. Neurol. (Chicago) 31:24, 1974.
18. MORI, M. & TAKAMORI, M. - A case of hyperthyroidism with two different types of neuromuscular transmission block. Clin. Neurol. 15:834, 1975.
19. MORI, M. & TAKAMORI, M. - Hyperthyroidism and myasthenia gravis with features of Eaton-Lambert syndrome. Neurology (Minneapolis) 26:882, 1976.
20. NAKANO, T.; SHINDO, M.; OGUCHI, K.; YANAGISAWA, N. & TSURAGOSHI, H. - Chronic polymiosites with electrophysiological characteristis of myasthenia gravis and myasthenic syndrome. A case report. Clin. Neurol. 20:100, 1980.
21. NORRIS, F.H. Jr. - Neuromuscular transmission in thyroid disease. Ann. int. Med. 64:81, 1966.
22. OH, S.J. - The Eaton-Lambert syndrome in ocular myasthenia gravis. Arch. Neurol. 13:183, 1974.
23. OH, S.J.; ESLAMI, N.; NISHIHIRA, T.; SARALA, P.K.; KUBA, T.; ELMORE, R.S.; SUNWOO, I.N. & RO, Y.I. - Electrophysiological and clinical correlation in myasthenia gravis. Ann. Neurol. 12:348, 1982.
24. SCHWARTZ, M. & STALBERG, E. - Myasthenia gravis with features of the myasthenic syndrome. Neurology (Minneapolis) 25:80, 1975.
25. SIMPSON, J.A. - Myasthenia gravis and myasthenic syndromes. In J.C. Walton: Disorders of Voluntary Muscles. Churchill Livingstone, London, 1981.
26. SINGER, P.; SMITH, L.; ZIELGER, D.K. & FESTOFF, B.W. - Posttetanic potentation in a patient with myasthenia gravis. Neurology 31:1345, 1981.
27. STALBERG, E. - Clinical electrophysiology in myasthenia gravis. J. Neurol. Neurosurg. Psychiat 43:622, 1980.
28. SWIFT, T.R. - Disorders of neuromuscular transmission other than myasthenia gravis. Muscle & Nerve 4:334, 1981.
29. TAKAMORI, M. & GUTMAN, L. - Intermittent defect of acetylcholine release in myasthenia gravis. Neurology (Minneapolis) 21:47, 1971.
30. TAKAMORI, M.; GUTMAN, L..; CORSET, T.W. & MARTIN, J.D. - Myasthenic syndromes in hypothyroidism. Electrophysiological study of neuromuscular transmission and muscle contraction in two patients. Arch. Neurol. (Chicago) 26:326, 1972.
31. WERNECK, L.C. - O valor da biópsia muscular em neurologia. Análise de 290 exames a fresco e pela histoquímica. Rev. bras. Clin. Terap. 10 (Edição especial), 1981.
32. WERNECK, L..C. - A lesão muscular na miastenia grave. Estudo de 17 casos com histoquimica muscular. Arq. Neuro-Psiquiat. (São Paulo) 40:67, 1982.

Datas de Publicação

Publicação nesta coleção
13 Ago 2012
Data do Fascículo
Jun 1985

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

[1] 1. BOIARDI, A.; BUSSONE, G. & NEFRI, S. - Alternating myasthenia and myastheniform syndrome in the same subject. J. Neurol. 220:57, 1979.

[2] 2. BROWN, J.W.; NELSON, J.R. & JERMANN, C.Jr. - Sjogren's syndrome with myopathic and myasthenic features. Bull. Los Angeles neurol. Soc. 33:9, 1968.

[3] 3. DAHLS, D.S. & SATO, S. - Unusual myasthenic state in a teen-age boy. Neurology (Minneapolis) 24:897, 1974.

[4] 4. DELL'OSSO, L.F.; AYYAR, D.R.; DARÓFF, R.B. & ABEL, L.A. - Edrophonium test in Eaton-Lambert syndrome: quantitative oculography. Neurology 33:1157, 1983.

[5] 5. DRACHMANN, D.B. - Myasthenia gravis. N. Engl. J. Med. 298:136, 1978.

[6] 6. EATON, L.M. & LAMBERT, E.H. - Electromyographic and electric stimulation of nerves in diseases of motor unit. Observations on myasthenic syndrome associated with malignant tumors. J. amer. med. Assoc. 163:1117, 1957.

[7] 7. ELMRVIST, D. & LAMBERT, E.H. - Detailed analysis of neuromuscular transmission in a patient with myasthenic syndrome sometimes associated with bronchogenic carcinoma. Mayo Clin. Proc. 43:689, 1968.

[8] 8. ENGEL, A.G.; LAMBERT, E.H. & HOWARD, F.M. - Immune complexes (IgG and C3) at the motor end-plate in myasthenia gravis. Ultrastructural and light microscopic localization and electrophysiologic correlations. Mayo Clinic. Proc. 52:267, 1977.

[9] 9. ENGEL, A.G.; LINDSTROM, J.M.; LAMBERT, E.H. & LENNON, V.A. - Ultrastructural localization of the acetylcholine receptor in myasthenia gravis and its experimental autoimune model. Neurology (Minneapolis) 27:307, 1977.

[10] 10. ENGEL, A.G. & SANTA, T. - Histometric analysis of the ultrastructure of the neuro-muscular junction in myasthenia gravis and in myasthenic syndrome. Ann. N.Y. Acad. Sci. 183:35, 1971.

[11] 11. FAMBROUGH, D.M.; DRACHMAN, D.B.; STARJAMURTI, S. - Neuromuscular junction in myasthenia gravis: decreased acetylcholine receptors. Science 182:293, 1973.

[12] 12. FETTBL, M.R.; SHIN, H.S.; PENN, A.S.; LOVELACE, R.E. & ROWLAND, L.P. - Combined Eaton-Lambert Syndrome and myasthenia gravis. Neurology 28:398, 1978.

[13] 13. GUTMAN, L.; CROSBY, T.W.; TAKAMORI, M. & MARTIN, J.D. - The Eaton-Lambert syndrome and autoimmune disorders. Amer. J. Med. 53:354, 1972.

[14] 14. GUTMAN, L. & PRATT, I. - Pathophysiologic aspects of human botulism. Arch. Neurol. (Chicago) 33:175, 1976.

[15] 15. LAMBERT, E.H.; ROOKE, E.D. - Myasthenic state and lung cancer. In L. BRAIN & F.H. MORRIS Jr.: The Remote Effects of Cancer on the Nervous System. Grune & Stratton, New York, 1965.

[16] 16. LANG, B.; NEWSON-DAVIS, J.; WRAY, D. & VINCENT, A. - Autoimmune aetiology for myasthenic (Eaton-Lambert) syndrome. Lancet 2:224, 1981.

[17] 17. MAYER, R.F. & WILLIAMS, I.R. - Incrementing responses in myasthenia gravis. Arch. Neurol. (Chicago) 31:24, 1974.

[18] 18. MORI, M. & TAKAMORI, M. - A case of hyperthyroidism with two different types of neuromuscular transmission block. Clin. Neurol. 15:834, 1975.

[19] 19. MORI, M. & TAKAMORI, M. - Hyperthyroidism and myasthenia gravis with features of Eaton-Lambert syndrome. Neurology (Minneapolis) 26:882, 1976.

[20] 20. NAKANO, T.; SHINDO, M.; OGUCHI, K.; YANAGISAWA, N. & TSURAGOSHI, H. - Chronic polymiosites with electrophysiological characteristis of myasthenia gravis and myasthenic syndrome. A case report. Clin. Neurol. 20:100, 1980.

[21] 21. NORRIS, F.H. Jr. - Neuromuscular transmission in thyroid disease. Ann. int. Med. 64:81, 1966.

[22] 22. OH, S.J. - The Eaton-Lambert syndrome in ocular myasthenia gravis. Arch. Neurol. 13:183, 1974.

[23] 23. OH, S.J.; ESLAMI, N.; NISHIHIRA, T.; SARALA, P.K.; KUBA, T.; ELMORE, R.S.; SUNWOO, I.N. & RO, Y.I. - Electrophysiological and clinical correlation in myasthenia gravis. Ann. Neurol. 12:348, 1982.

[24] 24. SCHWARTZ, M. & STALBERG, E. - Myasthenia gravis with features of the myasthenic syndrome. Neurology (Minneapolis) 25:80, 1975.

[25] 25. SIMPSON, J.A. - Myasthenia gravis and myasthenic syndromes. In J.C. Walton: Disorders of Voluntary Muscles. Churchill Livingstone, London, 1981.

[26] 26. SINGER, P.; SMITH, L.; ZIELGER, D.K. & FESTOFF, B.W. - Posttetanic potentation in a patient with myasthenia gravis. Neurology 31:1345, 1981.

[27] 27. STALBERG, E. - Clinical electrophysiology in myasthenia gravis. J. Neurol. Neurosurg. Psychiat 43:622, 1980.

[28] 28. SWIFT, T.R. - Disorders of neuromuscular transmission other than myasthenia gravis. Muscle & Nerve 4:334, 1981.

[29] 29. TAKAMORI, M. & GUTMAN, L. - Intermittent defect of acetylcholine release in myasthenia gravis. Neurology (Minneapolis) 21:47, 1971.

[30] 30. TAKAMORI, M.; GUTMAN, L..; CORSET, T.W. & MARTIN, J.D. - Myasthenic syndromes in hypothyroidism. Electrophysiological study of neuromuscular transmission and muscle contraction in two patients. Arch. Neurol. (Chicago) 26:326, 1972.

[31] 31. WERNECK, L.C. - O valor da biópsia muscular em neurologia. Análise de 290 exames a fresco e pela histoquímica. Rev. bras. Clin. Terap. 10 (Edição especial), 1981.

[32] 32. WERNECK, L..C. - A lesão muscular na miastenia grave. Estudo de 17 casos com histoquimica muscular. Arq. Neuro-Psiquiat. (São Paulo) 40:67, 1982.

Brasil

Brasil

Miastenia grave com resposta eletrográfica de síndrome miastênica: relato de um caso

Myasthenia gravis with electro graphic findings of myasthenic syndrome: a case report

Resumos

Datas de Publicação