Síndrome de Kocher-Debré-Semelaigne

Fortes-Rego, J.

doi:10.1590/S0004-282X1974000400009

Resumos

É relatado um caso de síndrome de Kocher-Debré-Semelaigne diagnosticado em um paciente de 8 anos, não tendo sido encontrados relatos similares na literatura nacional consultada. Apesar do início tardio do tratamento, a evolução mostrou-se bastante favorável.

A case of Kocher-Debré-Semelaigne syndrome is reported and a critical review of the literature is made. The patient responded well to extract thyroid with improvement of hypothyroidism and diminution in the size of the muscles.

Síndrome de Kocher-Debré-Semelaigne

Kocher-Debré-Semelaigne syndrome: a case report

J. Fortes-Rego

Departamento de Medicina Especializada da Faculdade de Ciências da Saúde da Universidade de Brasilia: Professor Assistente

RESUMO

É relatado um caso de síndrome de Kocher-Debré-Semelaigne diagnosticado em um paciente de 8 anos, não tendo sido encontrados relatos similares na literatura nacional consultada. Apesar do início tardio do tratamento, a evolução mostrou-se bastante favorável.

SUMMARY

A case of Kocher-Debré-Semelaigne syndrome is reported and a critical review of the literature is made. The patient responded well to extract thyroid with improvement of hypothyroidism and diminution in the size of the muscles.

Texto completo disponível apenas em PDF.

Full text available only in PDF format.

Universidade de Brasília Unidade Integrada de Saúde de Sobradinho 70400 Sobradinho DF Brasil.

Trabalho do Departamento de Medicina Especializada da Faculdade de Ciências da Saúde da Universidade de Brasilia, apresentado ao VI Congresso da Academia Brasileira de Neurologia (Rio de Janeiro, julho de 1974)

1. ASTROM, K. E.; KUGELBERG, E. & MULLER, R. Hypothyroid myopathy. Arch. Neurol. (Chicago) 5:472, 1961.
2. BERGOUIGNAN, M.; VITAL, CL. & BATAILLE, J. M. Les myopathies hypothyroidiènnes. Aspects cliniques et histopathologiques. Prèsse Méd. 75:1551, 1967.
3. BRAVERMAN, L. E.; INGBAR, S. H. & STERLING, K. Conversion of thyroxine (T4) to triiodothyronine (T3) in athyreotic human subjects. J. Clin. Invest. 49:855, 1970.
4. COTTON, G. E.; GORMAN, C. A. & MAYBERRY, W. E. Supression of thyrotropin (h-TSH) in serums of patients with myxedema of varying etiology treated with thyroid hormones. New England J. Med. 285:529, 1971.
5. DARRÉ, H.; MOLLARET, P.; ZAGDOUN, M. & OEHMICHEN, M. Hypertrophic musculaire généralisée du nourrison et hypothyroidie congénitale. Rev. Neurol. (Paris) 72:249, 1939.
6. DEBRÊ, R. & SEMELAIGNE, G. Hypertrophic musculaire généralisée du petit enfant. Bull. Soc. Péd. de Paris 33:699, 1934.
7. DEBRÉ, R. & SEMELAIGNE, G. Syndrome of diffuse muscular hypertrophy in infants causing athletic appearance. Amer. J. Dis. Child. 50:1351, 1935.
8. EVERED, D.; YOUNG, E. T.; ORMSTON, B. J.; MENZIES, R.; SMITH, P. A. & HALL, R Treatment of hypothyroidism: a reappraisal of thyroxine therapy. Brit. Med. J. 2:131, 1973.
9. FORD, F. R. Enfermedades del Sistema Nervioso en la Infancia, Niñez y Adolescencia. 2Ş Ed. castelhana. Editorial La Médica, Cordoba, Argentina, 1967.
10. FOSTER, M. & BARR, D. Myxedema: record of an autopsied case with special emphasis upon lesion of muscles. J. Clin. Endocrin. 4:417, 1944.
11. FOWLER, P. B. S. Treatment of hypothyroidism. Brit. Med. J. 2:352, 1973.
12. FOWLER, P. B. S.; SWALE, J. & ANDREWS, H. Hypercholesterolemia in borderline hypothyroidism. Lancet 11:488, 1970.
13. GIOVANNUCCI, M. L. La sindrome di Kocher-Debré-Semelaigne. Min. Pediat. 22:1101, 1970.
14. HALL, B. E.; SUNDERMAN, F. W. & GITTINGS, J. C. Congenital muscular hypertrophy. Amer. J. Dis. Child. 52:773, 1936.
15. HEUYER, G.; LEBOUICI, S.; KOUPERNIK, C. & MARTINETTI, J. Myxoedème congénital, hypertrophie musculaire généralisée, malformation vertébrale et atrophie corticale. Arch. Franc. Pédiat. 7:968, 1950.
16. INDIRA BAI, K.; SUBHA RAO, K. V. & VIJAYALAKSHMI, B. The Kocher-Debré-Semelaigne syndrome. Ind. Pediat. 8:852, 1971.
17. INGBAR, S. H. & WOEBER, K. A. Tratado de Endocrinologia. Editado por R. H. Williams. 3Ş Ed. castelhana. Salvat Editores, Barcelona, 1969.
18. KIRCHHEINER, B. Specific muscle lesions in pituitary-thyroid disorders. Acta Med. Scandinavica 172:539, 1962.
19. KOBIELOWA, Z.; NIWELINSKI, S.; KRZANOWSKA-DYRAS, M. & MAZUREK, A. Muscle hypertrophy in hypothyroidism (Debré-Semelaigne syndrome). Polish Endocrin. 21:388, 1970.
20. LARSEN, P. R. Triiodothyronine: review of recent studies of its physiology and pathophysiology in man. Metab. Clin. Experim. 21:1073, 1972.
21. NAJJAR, S. S. & NACHMAN, H. S. The Kocher-Debré-Semelaigne syndrome. J. Pediat. 66:901, 1965.
22. NORRIS, F. H. & PANNER, B. J. Hypothyroid myopathy. Arch. Neurol. (Chicago) 14:574, 1966.
23. NORMS, S. H.; PANNER, B. J. & STORMONT, J. H. Thyrotoxic periodic paralysis. Arch. Neurol. (Chicago) 19:88, 1968.
24. ORMSTON, B. J.; GARRY, R.; CRYER, R. J.; BESSER, G. M. & HALL, R. Thyrotrophin-releasing hormone as a thyroid-function test. Lancet 11:10, 1971.
25. PITTMAN, C. S.; CHAMBERS, J. B. & READ, V. H. The extrathyroidal conversion rate of thyroxine to triodothyronine in normal man. J. Clin. Investig. 50:1187, 1971.
26. PUROHIT, K. R. & MURTHY, D. The Kocher-Debré-Semelaigne syndrome: hypothyroidism with muscular "hypertrophy". Ind. Pediat. 6:338, 1969.
27. RIMBAUD, L. & PASSOUANT, P. Les dystrophies musculaires des hypothyroidiens. Rev. Neurol. (Paris) 79:81, 1947.
28. SCHWARZ, G. A. & ROSE, E. Neuromyopathies and thyroid dysfunction. Arch. Internal Med. 112:555, 1963.
29. SPIRO, A. J.; HIRANO, A.; BEILIN, R. L. & FINKELSTEIN, J. W. Cretinism with muscular hypertrophy (Kocher-Debré-Semelaigne syndrome). Arch. Neurol. (Chicago) 23:340, 1970.
30. WILSON, J. & WALTON, J. N. Some muscular manifestitions of hypothyroidism. J. Neurol. Neurosurg. Psychiat. 22:320, 1959.

Datas de Publicação

Publicação nesta coleção
09 Abr 2013
Data do Fascículo
Dez 1974

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

[1] 1. ASTROM, K. E.; KUGELBERG, E. & MULLER, R. Hypothyroid myopathy. Arch. Neurol. (Chicago) 5:472, 1961.

[2] 2. BERGOUIGNAN, M.; VITAL, CL. & BATAILLE, J. M. Les myopathies hypothyroidiènnes. Aspects cliniques et histopathologiques. Prèsse Méd. 75:1551, 1967.

[3] 3. BRAVERMAN, L. E.; INGBAR, S. H. & STERLING, K. Conversion of thyroxine (T4) to triiodothyronine (T3) in athyreotic human subjects. J. Clin. Invest. 49:855, 1970.

[4] 4. COTTON, G. E.; GORMAN, C. A. & MAYBERRY, W. E. Supression of thyrotropin (h-TSH) in serums of patients with myxedema of varying etiology treated with thyroid hormones. New England J. Med. 285:529, 1971.

[5] 5. DARRÉ, H.; MOLLARET, P.; ZAGDOUN, M. & OEHMICHEN, M. Hypertrophic musculaire généralisée du nourrison et hypothyroidie congénitale. Rev. Neurol. (Paris) 72:249, 1939.

[6] 6. DEBRÊ, R. & SEMELAIGNE, G. Hypertrophic musculaire généralisée du petit enfant. Bull. Soc. Péd. de Paris 33:699, 1934.

[7] 7. DEBRÉ, R. & SEMELAIGNE, G. Syndrome of diffuse muscular hypertrophy in infants causing athletic appearance. Amer. J. Dis. Child. 50:1351, 1935.

[8] 8. EVERED, D.; YOUNG, E. T.; ORMSTON, B. J.; MENZIES, R.; SMITH, P. A. & HALL, R Treatment of hypothyroidism: a reappraisal of thyroxine therapy. Brit. Med. J. 2:131, 1973.

[9] 9. FORD, F. R. Enfermedades del Sistema Nervioso en la Infancia, Niñez y Adolescencia. 2Ş Ed. castelhana. Editorial La Médica, Cordoba, Argentina, 1967.

[10] 10. FOSTER, M. & BARR, D. Myxedema: record of an autopsied case with special emphasis upon lesion of muscles. J. Clin. Endocrin. 4:417, 1944.

[11] 11. FOWLER, P. B. S. Treatment of hypothyroidism. Brit. Med. J. 2:352, 1973.

[12] 12. FOWLER, P. B. S.; SWALE, J. & ANDREWS, H. Hypercholesterolemia in borderline hypothyroidism. Lancet 11:488, 1970.

[13] 13. GIOVANNUCCI, M. L. La sindrome di Kocher-Debré-Semelaigne. Min. Pediat. 22:1101, 1970.

[14] 14. HALL, B. E.; SUNDERMAN, F. W. & GITTINGS, J. C. Congenital muscular hypertrophy. Amer. J. Dis. Child. 52:773, 1936.

[15] 15. HEUYER, G.; LEBOUICI, S.; KOUPERNIK, C. & MARTINETTI, J. Myxoedème congénital, hypertrophie musculaire généralisée, malformation vertébrale et atrophie corticale. Arch. Franc. Pédiat. 7:968, 1950.

[16] 16. INDIRA BAI, K.; SUBHA RAO, K. V. & VIJAYALAKSHMI, B. The Kocher-Debré-Semelaigne syndrome. Ind. Pediat. 8:852, 1971.

[17] 17. INGBAR, S. H. & WOEBER, K. A. Tratado de Endocrinologia. Editado por R. H. Williams. 3Ş Ed. castelhana. Salvat Editores, Barcelona, 1969.

[18] 18. KIRCHHEINER, B. Specific muscle lesions in pituitary-thyroid disorders. Acta Med. Scandinavica 172:539, 1962.

[19] 19. KOBIELOWA, Z.; NIWELINSKI, S.; KRZANOWSKA-DYRAS, M. & MAZUREK, A. Muscle hypertrophy in hypothyroidism (Debré-Semelaigne syndrome). Polish Endocrin. 21:388, 1970.

[20] 20. LARSEN, P. R. Triiodothyronine: review of recent studies of its physiology and pathophysiology in man. Metab. Clin. Experim. 21:1073, 1972.

[21] 21. NAJJAR, S. S. & NACHMAN, H. S. The Kocher-Debré-Semelaigne syndrome. J. Pediat. 66:901, 1965.

[22] 22. NORRIS, F. H. & PANNER, B. J. Hypothyroid myopathy. Arch. Neurol. (Chicago) 14:574, 1966.

[23] 23. NORMS, S. H.; PANNER, B. J. & STORMONT, J. H. Thyrotoxic periodic paralysis. Arch. Neurol. (Chicago) 19:88, 1968.

[24] 24. ORMSTON, B. J.; GARRY, R.; CRYER, R. J.; BESSER, G. M. & HALL, R. Thyrotrophin-releasing hormone as a thyroid-function test. Lancet 11:10, 1971.

[25] 25. PITTMAN, C. S.; CHAMBERS, J. B. & READ, V. H. The extrathyroidal conversion rate of thyroxine to triodothyronine in normal man. J. Clin. Investig. 50:1187, 1971.

[26] 26. PUROHIT, K. R. & MURTHY, D. The Kocher-Debré-Semelaigne syndrome: hypothyroidism with muscular "hypertrophy". Ind. Pediat. 6:338, 1969.

[27] 27. RIMBAUD, L. & PASSOUANT, P. Les dystrophies musculaires des hypothyroidiens. Rev. Neurol. (Paris) 79:81, 1947.

[28] 28. SCHWARZ, G. A. & ROSE, E. Neuromyopathies and thyroid dysfunction. Arch. Internal Med. 112:555, 1963.

[29] 29. SPIRO, A. J.; HIRANO, A.; BEILIN, R. L. & FINKELSTEIN, J. W. Cretinism with muscular hypertrophy (Kocher-Debré-Semelaigne syndrome). Arch. Neurol. (Chicago) 23:340, 1970.

[30] 30. WILSON, J. & WALTON, J. N. Some muscular manifestitions of hypothyroidism. J. Neurol. Neurosurg. Psychiat. 22:320, 1959.