Associação rara: polimiosite, miastenia grave e timoma

Boekel, V. Van; Godoy, J. M.; Menezes, J. M. T.

doi:10.1590/S0004-282X1990000400018

Resumos

Fundamentados no fato de que, tanto na literatura nacional quanto na estrangeira, inexiste referência da associação de polimiosite, miastenia grave e timoma em adulto jovem do sexo masculino, os autores se propõem a descrever um caso da citada síndrome. O paciente aqui apresentado teve sua doença iniciada aos 22 anos de idade, atingindo sua expressão clínica máxima 4 anos miais tarde. No estudo em apreço, são enfatizadas as dificuldades no diagnóstico diferencial entre polimiosite e miastenia grave, doenças que, a principio, têm suas manifestações clinicas características, de modo geral bem individualizadas. Na presente observação chamamos a atenção para a possível existência de aspectos clínicos sugestivos de miastenia em enfermos com polimiosite e, por outro lado, alterações histopatológicas típicas de polimiosite em músculos de miastênicos, inviabilizando muitas vezes o diagnóstico clínico de certeza.

Based on the inexistence of references in Brazilian and International literature of the association polymyositis, myasthenia gravis and thymoma in a young male adult, the authors propose to describe one case presenting the associated syndrome. The disease began when the patient was 22 years old, being the climax of its clinical expression four years later. It is emphasyzed the difficulty on the differential diagnosis between polymyositis and myasthenia gravis, once these diseases frequently have their clinical manifestations well individualized. In this study we call attention to the existence of clinical aspects of myasthenia gravis in patients presenting polymyositis and, on the other hand, histopathological alterations typical of polymyositis in myasthenic muscles, what makes the clinical diagnosis uncertain many times.

V. Van Boekel^I; J. M. Godoy^II; J. M. T. Menezes^III

^IMédica do Serviço de Clínica Médica e da UTI do HI - Unidade de Terapia Intensiva do Hospital de Ipanema (HI, INAMPS-RJ)

^IIProfessar Adjunto de Neurologia da Universidade do Estado do Rio de Janeiro (TJERJ) - Unidade de Terapia Intensiva do Hospital de Ipanema (HI, INAMPS-RJ)

^IIIMédica do Serviço de Clínica Médica do HI - Unidade de Terapia Intensiva do Hospital de Ipanema (HI, INAMPS-RJ)

RESUMO

Fundamentados no fato de que, tanto na literatura nacional quanto na estrangeira, inexiste referência da associação de polimiosite, miastenia grave e timoma em adulto jovem do sexo masculino, os autores se propõem a descrever um caso da citada síndrome. O paciente aqui apresentado teve sua doença iniciada aos 22 anos de idade, atingindo sua expressão clínica máxima 4 anos miais tarde. No estudo em apreço, são enfatizadas as dificuldades no diagnóstico diferencial entre polimiosite e miastenia grave, doenças que, a principio, têm suas manifestações clinicas características, de modo geral bem individualizadas. Na presente observação chamamos a atenção para a possível existência de aspectos clínicos sugestivos de miastenia em enfermos com polimiosite e, por outro lado, alterações histopatológicas típicas de polimiosite em músculos de miastênicos, inviabilizando muitas vezes o diagnóstico clínico de certeza.

SUMMARY

Based on the inexistence of references in Brazilian and International literature of the association polymyositis, myasthenia gravis and thymoma in a young male adult, the authors propose to describe one case presenting the associated syndrome. The disease began when the patient was 22 years old, being the climax of its clinical expression four years later. It is emphasyzed the difficulty on the differential diagnosis between polymyositis and myasthenia gravis, once these diseases frequently have their clinical manifestations well individualized. In this study we call attention to the existence of clinical aspects of myasthenia gravis in patients presenting polymyositis and, on the other hand, histopathological alterations typical of polymyositis in myasthenic muscles, what makes the clinical diagnosis uncertain many times.

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Dra. Valentima van Boekel - Rua Ozorio Duque Estrada 74/90BB - - Rio de Janeiro RJ -Brasil.

1. Ashizawa T, Elias SB, Appel SH - The interaction of myasthenic immunoglobulins and cholinergic agonists on acetylcholine receptors (AChrs) of rat rayotubes. Neurology 30:388, 1980.
2. Askmark H, Osterman PO, Roxin LE, Venge P - Radioimmunoassay of serum myoglobin in neuromuscular diseases. J Neurol Neurosurg Psychiat 44:68, 1981.
3. Barraquer-Bordas MML, Serra JP, Salisachs-Rowe P - Polymyosite chronique nodulaire fcoale avec syndrome myasthénique. Rev Neurol 113:69, 1965.
4. Behan WMH, Behan PO, DICK HA - HLA-B8 in polymyositis. N Engl J Med 298:1260, 1978.
5. Bohan A, Feter JB - Polymyositis and dermatomyositis (first of two parts). N Engl J Med 292:344, 1975.
6. Engel WK - Myasthenia gravis, corticosteroids, anticholinesterases. Ann N Y Acad Sci 274:623, 1976.
7. Engel WK, Festoff BW, Patten BM, Swerdlow ML., Newball HH, Thompson MD - Myasthenia gravis. Ann Intern Med 81:225, 1974.
8. Engel WK, Foidart-Dessalle M - Elevations of hemopexin levels in neuromuscular disease, Arch Neurol 35:577, 1978.
9. Engel WK, Lichter AS, Dalakas MC - Splenic and total-body irradiation treatment of myasthenia gravis. Ann N Y Acad Sci 377:744, 1981.
10. Johns TR, Crowley WJ, Miller JQ, Campa JF - The syndrome of myasthenia and polymyositis with comments on therapy. Ann N Y Acad Sci 183:64, 1971.
11. Klein JJ, Gottlieb AJ, Mones RJ, Appel SH, Osserman KE - Thymoma and polymyositis. Onset of myasthenia gravis after thymectomy: report of two cases. Arch Intern Med 113:192, 1964.
12. Kornfeld P, Siegal S, Weiner LB, Osserman KE - Studies in myasthenia gravis: immunologic response in thymectomized and nonthymectomized patients. Ann Intern Med 63:416, 1965.
13. Lederman RJ, Salanga VD. Wilbourn AJ, Hanson MR, Dudley AW - Focal inflammatory myopathy. Muscle & Nerve 7:142. 1984.
14. Lisak RP, Zweiman B - Mitogen and muscle extract induced in vitro proliferative responses in myasthenia gravis, dermatomyositis, and polymyositis. J Neurol Neurosurg Psychiat 38:521, 1975.
15. Lisak RP, Zweiman B - Serum immunoglobulin levels in myasthenia gravis, polymyositis, and dermatomyositis. J Neurol Neurosurg Psychiat 39:34, 1976.
16. Marchiori PE, Hirata MTA, Scaff M, Oliveira RM, Cossermeli W, Levy JA, Assis JL - Dermatopolimiosite: avaliação de 63 pacientes. Arq Neuro-Psiquiat (São Paulo) 45:137, 1987.
17. Messina C, Bonianno N, Baldare S, Vita G - Muscle uptake of muscular disorders: a 99m technetium pyrophosphate in patients neuronquantitative study. J. Neurol Sci 53:1, 1982.
18. Oosterhuis HJGH, Bethlem J, Feltkamp TEW - Muscle pathology, thymoma, and immunological abnormalities in patients with myasthenia gravis. J Neurol Neurosurg Psychiat 31:460, 1968.
19. Papatestas AE, Apert LI, Osserman KB, Osserman RS, Kant AE - Studies in myasthenia gravis: effects of thymectomy results on 185 patients with nonthymomatous and thymomatous myasthenia gravis, 1941-1969. Am J Med 50:465, 1971.
20. Paterson JH, Churchill-Davidson HC - Discussion on myasthenia. Proc Roy Soc Med 49:789, 1956.
21. Pestronk A, Drachman DB - Polymyositis: reduction of acetylcholine receptors in skeletal muscle. Muscle & Nerve 8:233, 1985.
22. Schwab RS, Perlo VP - Syndromes simulating myasthenia gravis. Ann N Y Acad Sci 135:350, 1966.
23. Souadjian JV, Enriquez P, Silverstein MN, Pépin JM - The spectrum of diseases associated with thymoma, coincidence or syndrome? Arch Intern Med 134:374, 1974.
24. Strauss AJL, Snell KC, Duntley BJ, Soban EJ, Stewart HL - Spontaneous thymoma, polymyositis, and serum autoantibodies to striated muscle in the rodent, subgenus Praomys (Mastomys) natalensis: similarities to thymoma with myasthenia gravis in man. Lancet 1:1126, 1968.
25. Weiller PJ, Durand JM, Prince-Zucchelli MA, Cross D, Pouget J, Pelissier JF, Mongin M - L'association polymyosite, myasthénie, thymome: un eas et revue de la literature. Ann Med Interne 135:299, 1984.

Associação rara: polimiosite, miastenia grave e timoma

An unusual association: polymyositis, myasthenia gravis, and thjmoma

Datas de Publicação

Publicação nesta coleção
19 Maio 2011
Data do Fascículo
Dez 1990

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

[1] 1. Ashizawa T, Elias SB, Appel SH - The interaction of myasthenic immunoglobulins and cholinergic agonists on acetylcholine receptors (AChrs) of rat rayotubes. Neurology 30:388, 1980.

[2] 2. Askmark H, Osterman PO, Roxin LE, Venge P - Radioimmunoassay of serum myoglobin in neuromuscular diseases. J Neurol Neurosurg Psychiat 44:68, 1981.

[3] 3. Barraquer-Bordas MML, Serra JP, Salisachs-Rowe P - Polymyosite chronique nodulaire fcoale avec syndrome myasthénique. Rev Neurol 113:69, 1965.

[4] 4. Behan WMH, Behan PO, DICK HA - HLA-B8 in polymyositis. N Engl J Med 298:1260, 1978.

[5] 5. Bohan A, Feter JB - Polymyositis and dermatomyositis (first of two parts). N Engl J Med 292:344, 1975.

[6] 6. Engel WK - Myasthenia gravis, corticosteroids, anticholinesterases. Ann N Y Acad Sci 274:623, 1976.

[7] 7. Engel WK, Festoff BW, Patten BM, Swerdlow ML., Newball HH, Thompson MD - Myasthenia gravis. Ann Intern Med 81:225, 1974.

[8] 8. Engel WK, Foidart-Dessalle M - Elevations of hemopexin levels in neuromuscular disease, Arch Neurol 35:577, 1978.

[9] 9. Engel WK, Lichter AS, Dalakas MC - Splenic and total-body irradiation treatment of myasthenia gravis. Ann N Y Acad Sci 377:744, 1981.

[10] 10. Johns TR, Crowley WJ, Miller JQ, Campa JF - The syndrome of myasthenia and polymyositis with comments on therapy. Ann N Y Acad Sci 183:64, 1971.

[11] 11. Klein JJ, Gottlieb AJ, Mones RJ, Appel SH, Osserman KE - Thymoma and polymyositis. Onset of myasthenia gravis after thymectomy: report of two cases. Arch Intern Med 113:192, 1964.

[12] 12. Kornfeld P, Siegal S, Weiner LB, Osserman KE - Studies in myasthenia gravis: immunologic response in thymectomized and nonthymectomized patients. Ann Intern Med 63:416, 1965.

[13] 13. Lederman RJ, Salanga VD. Wilbourn AJ, Hanson MR, Dudley AW - Focal inflammatory myopathy. Muscle & Nerve 7:142. 1984.

[14] 14. Lisak RP, Zweiman B - Mitogen and muscle extract induced in vitro proliferative responses in myasthenia gravis, dermatomyositis, and polymyositis. J Neurol Neurosurg Psychiat 38:521, 1975.

[15] 15. Lisak RP, Zweiman B - Serum immunoglobulin levels in myasthenia gravis, polymyositis, and dermatomyositis. J Neurol Neurosurg Psychiat 39:34, 1976.

[16] 16. Marchiori PE, Hirata MTA, Scaff M, Oliveira RM, Cossermeli W, Levy JA, Assis JL - Dermatopolimiosite: avaliação de 63 pacientes. Arq Neuro-Psiquiat (São Paulo) 45:137, 1987.

[17] 17. Messina C, Bonianno N, Baldare S, Vita G - Muscle uptake of muscular disorders: a 99m technetium pyrophosphate in patients neuronquantitative study. J. Neurol Sci 53:1, 1982.

[18] 18. Oosterhuis HJGH, Bethlem J, Feltkamp TEW - Muscle pathology, thymoma, and immunological abnormalities in patients with myasthenia gravis. J Neurol Neurosurg Psychiat 31:460, 1968.

[19] 19. Papatestas AE, Apert LI, Osserman KB, Osserman RS, Kant AE - Studies in myasthenia gravis: effects of thymectomy results on 185 patients with nonthymomatous and thymomatous myasthenia gravis, 1941-1969. Am J Med 50:465, 1971.

[20] 20. Paterson JH, Churchill-Davidson HC - Discussion on myasthenia. Proc Roy Soc Med 49:789, 1956.

[21] 21. Pestronk A, Drachman DB - Polymyositis: reduction of acetylcholine receptors in skeletal muscle. Muscle & Nerve 8:233, 1985.

[22] 22. Schwab RS, Perlo VP - Syndromes simulating myasthenia gravis. Ann N Y Acad Sci 135:350, 1966.

[23] 23. Souadjian JV, Enriquez P, Silverstein MN, Pépin JM - The spectrum of diseases associated with thymoma, coincidence or syndrome? Arch Intern Med 134:374, 1974.

[24] 24. Strauss AJL, Snell KC, Duntley BJ, Soban EJ, Stewart HL - Spontaneous thymoma, polymyositis, and serum autoantibodies to striated muscle in the rodent, subgenus Praomys (Mastomys) natalensis: similarities to thymoma with myasthenia gravis in man. Lancet 1:1126, 1968.

[25] 25. Weiller PJ, Durand JM, Prince-Zucchelli MA, Cross D, Pouget J, Pelissier JF, Mongin M - L'association polymyosite, myasthénie, thymome: un eas et revue de la literature. Ann Med Interne 135:299, 1984.