Agregados tubulares em caso de atrofia espinhal cronica proximal

Werneck, Lineu Cesar; Silvado, Carlos Eduardo; Boer, Carlos Alberto

doi:10.1590/S0004-282X1979000100008

Resumos

Relato de um caso de atrofia muscular espinhal crônica proximal em paciente de 35 anos de idade, que apresentava, na biópsia muscular processada pela histoquímica, estruturas identificadas como agregados tubulares. São feitas considerações sobre a patogenia e função dos agregados tubulares. No caso descrito, acreditam os autores que os mesmos foram induzidos pelo uso contínuo de anticonvulsivantes por 20 anos, associada à denervação muscular e ao uso constante de álcool.

The case of a 35 years-old man, with chronic proximal muscle atrophy in which at the muscle biopsy tubular aggregates were found by histochemistry procedures is reported. The tubular aggregates stained positive with the modified Gomori trichrome, haematoxylin-eosin, DPNH-diaphorase, non specific esterases, phosphorylase, P.A.S., oil red O and lactate dehydrogenase. They did not show in the routine and acid pre-incubated ATPase, acid and alkaline phosphatases and succinate dehydrogenase. Only found in type II fibers. A brief discussion about the pathogenesis and function of the tubular aggregates is made. The authors believe that the tubular aggregates in this case are secondary to prolonged use of phenobarbital and diphenylhydantoin, associated with the basic denervation process and alcohol abuse.

Agregados tubulares em caso de atrofia espinhal cronica proximal

Tubular aggregates in a case of chronic proximal spinal muscle atrophy

Lineu Cesar Werneck^I; Carlos Eduardo Silvado^II; Carlos Alberto Boer^II

^IProfessor Assistente. Departamento de Clínica Médica da Universidade Federal do Paraná, Curitiba, PR

^IIResidentes. Departamento de Clínica Médica da Universidade Federal do Paraná, Curitiba, PR

RESUMO

Relato de um caso de atrofia muscular espinhal crônica proximal em paciente de 35 anos de idade, que apresentava, na biópsia muscular processada pela histoquímica, estruturas identificadas como agregados tubulares. São feitas considerações sobre a patogenia e função dos agregados tubulares. No caso descrito, acreditam os autores que os mesmos foram induzidos pelo uso contínuo de anticonvulsivantes por 20 anos, associada à denervação muscular e ao uso constante de álcool.

SUMMARY

The case of a 35 years-old man, with chronic proximal muscle atrophy in which at the muscle biopsy tubular aggregates were found by histochemistry procedures is reported. The tubular aggregates stained positive with the modified Gomori trichrome, haematoxylin-eosin, DPNH-diaphorase, non specific esterases, phosphorylase, P.A.S., oil red O and lactate dehydrogenase. They did not show in the routine and acid pre-incubated ATPase, acid and alkaline phosphatases and succinate dehydrogenase. Only found in type II fibers. A brief discussion about the pathogenesis and function of the tubular aggregates is made. The authors believe that the tubular aggregates in this case are secondary to prolonged use of phenobarbital and diphenylhydantoin, associated with the basic denervation process and alcohol abuse.

Texto completo disponível apenas em PDF.

Full text available only in PDF format.

Trabalho realizado na Disciplina de Neurologia do Departamento de Clínica Médica da Universidade Federal do Paraná, Curitiba, PR.

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

1. BERGMAN, R. A.; AFIFI, A. K.; DUNKIE L. M. & JOHNS, R. J. - Muscle pathology in hypokalemic periodic paralysis with hyperthyroidism. Hopkins Med. J. 126:100, 1970.
2. BUNDEY, S. & LOVELACE, R. E. - A clinical and genetic study of chronic proximal spinal muscular atrophy. Brain 98:445, 1975.
3. CHUI, L. A.; NEUSTEIN, H. & MUNSAT, T. L. - Tubular aggregates in sub-clinical alcoholic myopathy. Neurology (Minneapolis) 25:405, 1975.
4. DUBOWITZ, V. - Benign infantile spinal muscular atrophy. Dev. Med. Child Neurol. 16:672, 1974.
5. DUBOWITZ, V. & BROOKE, M. H. - Muscle Biopsy: A Modern Approach. W. B. Saunders Co. Ltd., London, 1973.
6. DUCHEN, L. W. - The local effects of tetanus toxin on the electron microscopic struture of skeletal muscle fibers of the mouse. J. Neurol. Sci. 19:169, 1973.
7. DUNKLE, L. M.; DIGGS, C. H.; BERGMAN, R. A. & JOHNS, R. J. - A light and electron microscopic study of a second case of hypokalemic periodic paralysis with hyperthyroidism. Hopkins Med. J. 126:225, 1970.
8. ENGEL, W. K. - Mitochondria aggregates in muscle disease. J. Histoch. Cytochem. 12:46, 1964.
9. ENGEL, W. K. - Selective and nonselective susceptibility of muscle fiber type. Arch. Neurol. (Chicago) 22:97, 1970.
10. ENGEL, W. K.; BISHOP, D. W. & CUNNINGHAN, G. C. - Tubular aggregates in type II muscle fibers: ultrastructural and histochemical correlation. J. Ultrastructure Research 31:507, 1970.
11. ENGEL, W. K & BROOKE, M. H. - Muscle biopsy as a clinical diagnostic aid. In Fields, W. S. - Neurological Diagnostic Techniques. Charles C. Thomas Publ., Springfield (Illinois), 1966.
12. GALLAI, M. - Myopathy with hyperaldosteronism: an electron-microscopic study. J. Neurol. Sci. 32:337, 1977.
13. JONES, A. L. & FAWCETT, D. W. - Hypertrophy of the agranular endoplasmic reticulum in hamster liver induced by phenobarbital. J. Histochem. Cytochem. 14:215, 1966.
14. LEWIS, P. D.; PALLIS, C. & PEARSE, A.G.E. - Myopathy with tubular aggregates. J. Neurol. Sci. 13:381, 1971.
15. MACDONALD, R. D.: REWCASTLE, N. B. & HUMPHREY, J. G. - The myopathy of hyperkalemic periodic paralysis: an electron microscopic study. Arch. Neurol. (Chicago) 19:274, 1968.
16. MORGHAN-HUGHES, J. A.; MAIR, W.G.P. & LASCELLES, P. T. - A disorder of skeletal muscle associated with tubular aggregates. Brain 93:873, 1970.
17. ORRENIUS, S.; ERICSSON. J. L. E. & ERNSTER, L. - Phenobarbital-induced synthesis of the microsomal drug-metabolizing enzyme system and its relationship to the proliferation of endoplasmic membranes. J. Cell Biol. 25:627, 1965.
18. ORRENIUS. S. & ERICSSON. J. L. E. - Enzme-membrane relationship in phenobarbital induction of synthesis of drug-metabolizing enzyme system and proliferation of endoplasmic membranes. J. Cell Biol. 28:181, 1966.
19. ORRENIUS, S. & ERICSSON, J. L. E. - On the relationship of liver glicose-6-phosphatase to the proliferation of endoplasmic reticulum in phenobarbital induction. J. Cell Biol. 31:243, 1968.
20. PELLEGRINO, C. & FRANZINI, C. - An electron microscope study of denervation atrophy in red and white skeletal muscle fibers. J. Cell Biol. 17:327, 1963.
21. PRICE, H. M.; HOWES, E. L. & BLUMBERG, J. M. - Ultrastructural alterations in skeletal muscle fibers injured by cold: the acute degenerative changes. Lab. Invest. 13:1264, 1964.
22. RUBIN, E.; HUTTERER, F. & LIEBER, C. S. - Ethanol increases hepatic smooth endoplasmic reticulum and drug-metabolizing enzymes. Sciences 159:1469, 1968.
23. VAN WIJNGAARDEN, G. K. & BETHLEM, J. - Benign infantile spinal muscular atrophy. Brain 96:163, 1973.

Datas de Publicação

Publicação nesta coleção
27 Ago 2012
Data do Fascículo
Mar 1979

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

[1] 1. BERGMAN, R. A.; AFIFI, A. K.; DUNKIE L. M. & JOHNS, R. J. - Muscle pathology in hypokalemic periodic paralysis with hyperthyroidism. Hopkins Med. J. 126:100, 1970.

[2] 2. BUNDEY, S. & LOVELACE, R. E. - A clinical and genetic study of chronic proximal spinal muscular atrophy. Brain 98:445, 1975.

[3] 3. CHUI, L. A.; NEUSTEIN, H. & MUNSAT, T. L. - Tubular aggregates in sub-clinical alcoholic myopathy. Neurology (Minneapolis) 25:405, 1975.

[4] 4. DUBOWITZ, V. - Benign infantile spinal muscular atrophy. Dev. Med. Child Neurol. 16:672, 1974.

[5] 5. DUBOWITZ, V. & BROOKE, M. H. - Muscle Biopsy: A Modern Approach. W. B. Saunders Co. Ltd., London, 1973.

[6] 6. DUCHEN, L. W. - The local effects of tetanus toxin on the electron microscopic struture of skeletal muscle fibers of the mouse. J. Neurol. Sci. 19:169, 1973.

[7] 7. DUNKLE, L. M.; DIGGS, C. H.; BERGMAN, R. A. & JOHNS, R. J. - A light and electron microscopic study of a second case of hypokalemic periodic paralysis with hyperthyroidism. Hopkins Med. J. 126:225, 1970.

[8] 8. ENGEL, W. K. - Mitochondria aggregates in muscle disease. J. Histoch. Cytochem. 12:46, 1964.

[9] 9. ENGEL, W. K. - Selective and nonselective susceptibility of muscle fiber type. Arch. Neurol. (Chicago) 22:97, 1970.

[10] 10. ENGEL, W. K.; BISHOP, D. W. & CUNNINGHAN, G. C. - Tubular aggregates in type II muscle fibers: ultrastructural and histochemical correlation. J. Ultrastructure Research 31:507, 1970.

[11] 11. ENGEL, W. K & BROOKE, M. H. - Muscle biopsy as a clinical diagnostic aid. In Fields, W. S. - Neurological Diagnostic Techniques. Charles C. Thomas Publ., Springfield (Illinois), 1966.

[12] 12. GALLAI, M. - Myopathy with hyperaldosteronism: an electron-microscopic study. J. Neurol. Sci. 32:337, 1977.

[13] 13. JONES, A. L. & FAWCETT, D. W. - Hypertrophy of the agranular endoplasmic reticulum in hamster liver induced by phenobarbital. J. Histochem. Cytochem. 14:215, 1966.

[14] 14. LEWIS, P. D.; PALLIS, C. & PEARSE, A.G.E. - Myopathy with tubular aggregates. J. Neurol. Sci. 13:381, 1971.

[15] 15. MACDONALD, R. D.: REWCASTLE, N. B. & HUMPHREY, J. G. - The myopathy of hyperkalemic periodic paralysis: an electron microscopic study. Arch. Neurol. (Chicago) 19:274, 1968.

[16] 16. MORGHAN-HUGHES, J. A.; MAIR, W.G.P. & LASCELLES, P. T. - A disorder of skeletal muscle associated with tubular aggregates. Brain 93:873, 1970.

[17] 17. ORRENIUS, S.; ERICSSON. J. L. E. & ERNSTER, L. - Phenobarbital-induced synthesis of the microsomal drug-metabolizing enzyme system and its relationship to the proliferation of endoplasmic membranes. J. Cell Biol. 25:627, 1965.

[18] 18. ORRENIUS. S. & ERICSSON. J. L. E. - Enzme-membrane relationship in phenobarbital induction of synthesis of drug-metabolizing enzyme system and proliferation of endoplasmic membranes. J. Cell Biol. 28:181, 1966.

[19] 19. ORRENIUS, S. & ERICSSON, J. L. E. - On the relationship of liver glicose-6-phosphatase to the proliferation of endoplasmic reticulum in phenobarbital induction. J. Cell Biol. 31:243, 1968.

[20] 20. PELLEGRINO, C. & FRANZINI, C. - An electron microscope study of denervation atrophy in red and white skeletal muscle fibers. J. Cell Biol. 17:327, 1963.

[21] 21. PRICE, H. M.; HOWES, E. L. & BLUMBERG, J. M. - Ultrastructural alterations in skeletal muscle fibers injured by cold: the acute degenerative changes. Lab. Invest. 13:1264, 1964.

[22] 22. RUBIN, E.; HUTTERER, F. & LIEBER, C. S. - Ethanol increases hepatic smooth endoplasmic reticulum and drug-metabolizing enzymes. Sciences 159:1469, 1968.

[23] 23. VAN WIJNGAARDEN, G. K. & BETHLEM, J. - Benign infantile spinal muscular atrophy. Brain 96:163, 1973.