Miopatias associadas a agregados tubulares

Carvalho, Mary Souza; Lusvarghi, Edgard S.; Levy, Ângela Lobo F.; Salum, Paulo N. Borsoi; Rodrigues, Consuelo J.; Levy, José Antonio

doi:10.1590/S0004-282X1993000300013

Resumos

Os autores relatam a caso de paciente de 58 anos de idade do sexo masculino, com quadro de características miastênicas tanto clínica como eletromiograficamente, no qual a biópsia muscular com histoquímica e microscopia eletrônica permitiu fazer o diagnostico de miopatia associada a agregados tubulares. É chamada a atenção para o fato de que as alterações anátomo-patológicas encontradas podem estar presentes em um grupo heterogêneo de pacientes com grande variedade de sintomas, não havendo portanto motivo para considerar-se a existência de uma miopatia com agregados tubulares, já que os achados anátomo-patológicos são inespecíficos e não configuram moléstia específica.

miopatia; agregado tubular; histoquímica; ultra-estrutura

The authors report the case of a 58-year-old male patient with clinical and electromyographic features of myasthenia. Muscle biopsy with histochemistry and electronic microscopy made it possible to diagnose a myopathy associated with tubular aggregates. Attention is called to the fact that the anatomical pathologic alterations which were found may be present in a heterogenous group of patients showing a great variety of symptoms. Thus, there is no reason to consider the existence of a myopathy associated with tubular aggregates, since the anatomical and pathologic findings are inespecific and do not characterize any specific disease.

myopathy; tubular aggregates; histochemistry; ultrastructure

Mary Souza Carvalho^I; Edgard S. Lusvarghi^I; Ângela Lobo F. Levy^II; Paulo N. Borsoi Salum^I; Consuelo J. Rodrigues^III; José Antonio Levy^IV

^IMédico, CN/FMUSP

^IIBiomédica CN/FMUSP

^IIIPatologista, Disciplina de Técnica Cirúrgica, FMUSP

^IVProfessor Associado CN/FMUSP

RESUMO

Os autores relatam a caso de paciente de 58 anos de idade do sexo masculino, com quadro de características miastênicas tanto clínica como eletromiograficamente, no qual a biópsia muscular com histoquímica e microscopia eletrônica permitiu fazer o diagnostico de miopatia associada a agregados tubulares. É chamada a atenção para o fato de que as alterações anátomo-patológicas encontradas podem estar presentes em um grupo heterogêneo de pacientes com grande variedade de sintomas, não havendo portanto motivo para considerar-se a existência de uma miopatia com agregados tubulares, já que os achados anátomo-patológicos são inespecíficos e não configuram moléstia específica.

Palavras-chave: miopatia, agregado tubular, histoquímica, ultra-estrutura.

SUMMARY

The authors report the case of a 58-year-old male patient with clinical and electromyographic features of myasthenia. Muscle biopsy with histochemistry and electronic microscopy made it possible to diagnose a myopathy associated with tubular aggregates. Attention is called to the fact that the anatomical pathologic alterations which were found may be present in a heterogenous group of patients showing a great variety of symptoms. Thus, there is no reason to consider the existence of a myopathy associated with tubular aggregates, since the anatomical and pathologic findings are inespecific and do not characterize any specific disease.

Key words: myopathy, tubular aggregates, histochemistry, ultrastructure.

Full text available only in PDF format.

Texto completo disponível apenas em PDF.

Aceite: 31-janeiro-1993.

Dr. José Antonio Levy Departamento de Neurologia, Faculdade de Medicina USP - Av. Dr. Arnaldo 455 - 01246-000 São Paulo SP - Brasil.

Trabalho da Clínica Neurológica (CN) da Faculdade de Medicina (FM) da Universidade de São Paulo (USP) (Prof. Dr. Milberto Scaff) e do Laboratório de Patologia Molecular da FMUSP (Prof. Dr. Antonio Sesso)

1. Assis JL, Marchiori PE. Miastenia grave: fisiopatologia. In Assis JL. Miastenia grave. São Paulo: Sarvier, 1990, p 206.
2. Banker BQ. The congenital myopathies. In Engel A, Banker B. Myology. New York: McGraw-Hill 1986, p 1554-1556.
3. Bergman RB, Johns RJ. Ultrastructural alteration in muscles from patients with myasthenia gravis and Eaton-Lambert syndrome. Ann NY Acad Sci 1987, 183:88-122.
4. Bradley W. Ultrastructural changes in adynamia epsodica hereditaria and normokalemic familial periodic paralysis. Brain 1969, 93:379-390.
5. Brumback RA, Staton RG, Susag ME. Exercise induced pain, stiffness and tubular aggregation in skeletal muscle. J Neurol Neurosurg Psychiatry 1981, 44:250-254.
6. Duchen WH, The local effects of tetanus toxin on the electron microscopic structure of skeletal muscle fiber of the mouse. J Neurol Sci 1973, 19:169-177.
7. Engel AG, Lamber EH, Mulder DM, Torres CF, Sbashi K, Bertonini TE, Whitaker JN. Investigation of three oases of newly recognized familial congenital myasthenic syndrome. Ann Neurol 1971, 6:146-147.
8. Fardeau M, Tomé FM, Simon P. Muscle and nerve changes induced by perhexilene in man and mice. Muscle Nerve 1979, 2:24-36.
9. Groot JG, Arts WF. Familial myopathy tubular aggregates. J Neurol 1987, 227:35-39.
10. Mastaglia FL. Pathological changes in skeletal muscle in acromegaly. Acta Neuropathol 1973, 24:273-286.
11. Meyers K, Gilden D, Rinaldi C. Periodic muscle weakness, normokalemia and tubular aggregates. Neurology 1972, 22:269-279.
12. Rohkamm R, Boxler K, Ricker K, Jerusalem F. Dominantly inherited myopathy with excessive tubular aggregates. Neurology 1983, 33:331-336.
13. Shagiq SA, Milhorat AT, Garyk MA. Fine structure of human muscle in neurogenic atrophy. Neurology 1977, 29:996-1005.
14. Schiffer D, Giordona MT, Mons G, Mollo F. Histochemistry and electron microscopy of muscle fibers in a case of congenital paramyotonia. J Neurol 1976, 211:125-133.
15. Schroeder JM, Adams RD. The ultrastructural morphology of the muscle fiber in myotonic dystrophy. Acta Neuropathol 1968, 10:218-241.
16. Stroebner P, Sengel A, Jesel M, Isch F. Ultrastructure de certeins polymyosites myas-theniformes. Proc 6th Int Congr Neuropathol. Paris: Masson, 1970, p 1078-1079.
17. Tomé F, Mair WGP. Electron microscopical and histochemical studies of muscle in a case of neuropathy with target fibres and laminar cytoplasmic structures. Proc 6th Int Congr Neuropathol. Paris: Masson, 1970, 1070-1071.
18. Werneck LC. O valor da biópsia muscular em neurología. Clínica e Terapêutica 1981, número especial (março):1-24.

Miopatias associadas a agregados tubulares

Myopathies associated to tubular aggregates

Datas de Publicação

Publicação nesta coleção
19 Jan 2011
Data do Fascículo
Set 1993

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

[1] 1. Assis JL, Marchiori PE. Miastenia grave: fisiopatologia. In Assis JL. Miastenia grave. São Paulo: Sarvier, 1990, p 206.

[2] 2. Banker BQ. The congenital myopathies. In Engel A, Banker B. Myology. New York: McGraw-Hill 1986, p 1554-1556.

[3] 3. Bergman RB, Johns RJ. Ultrastructural alteration in muscles from patients with myasthenia gravis and Eaton-Lambert syndrome. Ann NY Acad Sci 1987, 183:88-122.

[4] 4. Bradley W. Ultrastructural changes in adynamia epsodica hereditaria and normokalemic familial periodic paralysis. Brain 1969, 93:379-390.

[5] 5. Brumback RA, Staton RG, Susag ME. Exercise induced pain, stiffness and tubular aggregation in skeletal muscle. J Neurol Neurosurg Psychiatry 1981, 44:250-254.

[6] 6. Duchen WH, The local effects of tetanus toxin on the electron microscopic structure of skeletal muscle fiber of the mouse. J Neurol Sci 1973, 19:169-177.

[7] 7. Engel AG, Lamber EH, Mulder DM, Torres CF, Sbashi K, Bertonini TE, Whitaker JN. Investigation of three oases of newly recognized familial congenital myasthenic syndrome. Ann Neurol 1971, 6:146-147.

[8] 8. Fardeau M, Tomé FM, Simon P. Muscle and nerve changes induced by perhexilene in man and mice. Muscle Nerve 1979, 2:24-36.

[9] 9. Groot JG, Arts WF. Familial myopathy tubular aggregates. J Neurol 1987, 227:35-39.

[10] 10. Mastaglia FL. Pathological changes in skeletal muscle in acromegaly. Acta Neuropathol 1973, 24:273-286.

[11] 11. Meyers K, Gilden D, Rinaldi C. Periodic muscle weakness, normokalemia and tubular aggregates. Neurology 1972, 22:269-279.

[12] 12. Rohkamm R, Boxler K, Ricker K, Jerusalem F. Dominantly inherited myopathy with excessive tubular aggregates. Neurology 1983, 33:331-336.

[13] 13. Shagiq SA, Milhorat AT, Garyk MA. Fine structure of human muscle in neurogenic atrophy. Neurology 1977, 29:996-1005.

[14] 14. Schiffer D, Giordona MT, Mons G, Mollo F. Histochemistry and electron microscopy of muscle fibers in a case of congenital paramyotonia. J Neurol 1976, 211:125-133.

[15] 15. Schroeder JM, Adams RD. The ultrastructural morphology of the muscle fiber in myotonic dystrophy. Acta Neuropathol 1968, 10:218-241.

[16] 16. Stroebner P, Sengel A, Jesel M, Isch F. Ultrastructure de certeins polymyosites myas-theniformes. Proc 6th Int Congr Neuropathol. Paris: Masson, 1970, p 1078-1079.

[17] 17. Tomé F, Mair WGP. Electron microscopical and histochemical studies of muscle in a case of neuropathy with target fibres and laminar cytoplasmic structures. Proc 6th Int Congr Neuropathol. Paris: Masson, 1970, 1070-1071.

[18] 18. Werneck LC. O valor da biópsia muscular em neurología. Clínica e Terapêutica 1981, número especial (março):1-24.