Russel's syndrome: a case with precocious puberty development

Castro, Eliana Maria de; Silva, Ivani Novato; Perpétuo, Francisco Otaviano Lima

doi:10.1590/S0004-282X1987000100010

Abstracts

The Russel's syndrome is characterized by emaciation and hyperactivity in children and is caused by hypothalamic expansive process. This report presents the typical signs of the syndrome associated with growth hormone secretion disorders and precocious puberty pattern appearing at the age of four. The pathogenesis of the disorder is discussed and related to the hypothalamus-pituitary axis dysfunction caused by the tumor growth.

A síndrome de Russel é caracterizada por emaciação e hiperatividade da criança e é decorrente de processo expansivo hipotalâmico. O caso relatado apresenta os sinais típicos da síndrome associados a distúrbios na secreção do hormônio do crescimento e quadro de puberdade precoce desenvolvido aos 4 anos de idade. A patogênese das alterações é discutida e relacionada com a disfunção do eixo hipotálamo-hipofisário, produzida pela evolução do crescimento tumoral.

Síndrome de russel: apresentação de um caso com desenvolvimento de puberdade precoce

Russel's syndrome: a case with precocious puberty development

Eliana Maria de Castro^I; Ivani Novato Silva^II; Francisco Otaviano Lima Perpétuo^III

^IProfessora Assistente de Neurologia do Departamento de Psiquiatria e Neurologia da Faculdade de Medicina da Universidade Federal de Minas Gerais (FMUFMG)

^IIProfessora Assistente e Membro do Grupo de Endocrinologia Infantil do Departamento de Pediatria da FMUFMG

^IIIProfessor Adjunto de Neurocirurgia do Departamento de Psiquiatria e Neurologia da FMUFMG

RESUMO

A síndrome de Russel é caracterizada por emaciação e hiperatividade da criança e é decorrente de processo expansivo hipotalâmico. O caso relatado apresenta os sinais típicos da síndrome associados a distúrbios na secreção do hormônio do crescimento e quadro de puberdade precoce desenvolvido aos 4 anos de idade. A patogênese das alterações é discutida e relacionada com a disfunção do eixo hipotálamo-hipofisário, produzida pela evolução do crescimento tumoral.

SUMMARY

The Russel's syndrome is characterized by emaciation and hyperactivity in children and is caused by hypothalamic expansive process. This report presents the typical signs of the syndrome associated with growth hormone secretion disorders and precocious puberty pattern appearing at the age of four. The pathogenesis of the disorder is discussed and related to the hypothalamus-pituitary axis dysfunction caused by the tumor growth.

Texto completo disponível apenas em PDF.

Full text available only in PDF format.

Departamento de Psiquiatria e Neurología, Faculdade de Medicina, UFMG - Av. Alfredo Balena, 190 - 30130, Belo Horizonte, MG - Brasil.

1. ADDY, D.P. & HUDSON, F.P. - Diencephalic syndrome of infantile emaciation. Arch. Dis. Child. 47:388, 1972.
2. ANDLER, W. ; STOLECK, H. & SIRANG, H. - Endocrine dysfunction in the diencephalic syndrome of emaciation in infancy. Helv. paediatr. Acta. 33:393, 1978.
3. BAIN, H.W. ; DARTZ, J.M.M. ; KEITH, W.S. & KRUYFF, E. - The diencephalic syndrome of early infancy due to silent brain tumor: with special reference to treatment. Pediatrics 38:473, 1966.
4. BRAUN, F.C. & FORNEY, W.R. - Diencephalic syndrome of early infancy associated with brain tumor. Pediatrics 10:609, 1959.
5. BURR, I.M.; SLONIN, A.E.; DANISH, R.K. ; GADOTH, N. & BUTLER, I.J. - Diencephalic syndrome revisited. J. Pediatr. 88:439, 1976.
6. CHEMALE, I.M.; MEDINA, G.R.M. & FARINATTT, F. - Síndrome de Russel: relato de um caso. Acta neurol. latinoamer. 26 : 275, 1980.
7. CHOUX, M.; BAURAND, C. ; PIERRON, H. & VIGOUROUX, R.P. - Lipoatrophie cachectisante par tumeur de la partie antérieure du plancher du IHe. ventricule (Syndrome de Russel). Neuro-Chirurgie 15:59, 1969.
8. DANZIGER, J. & BLOCH, S. - Hypothalamic tumours presenting as the diencephalic syndrome. Clin. Radiol. 25:153, 1974.
9. DODS, L. - A diencephalic syndrome of early infancy. Med. J. Aust. 9:689, 1957.
10. DODS, L. - A diencephalic syndrome of early infancy. Med. J. Aust. 4:222, 1967.
11. DROP, S.L.S. ; GUYDA, H.J. & COLLE, E. - Inappropriate growth hormone release in the diencephalic syndrome of childhood: case report and 4 year endocrinological follow-up. Clin. Endocrinol. 13:181, 1980.
12. FERREIRA, N.P. & OLIVEIRA, E.A. - Síndrome de Russel ou síndrome diencefálica de emagrecimento. Arq. Neuro-Psiquiatr. (São Paulo) 29:207, 1971.
13. FISHMAN, M.A. & PEAKE, G.T. - Paradoxical growth in a patient with the diencephalic syndrome. Pediatrics 45:973, 1970.
14. FRAS1ER, S.D. - Abnormalities of sexual development. In Pediatric Endocrinology. Grune & Stratton, New York, 1980, pg. 147.
15. GREULICH, W.W. & PYLE, S.I. - Radiographic Atlas of Skeletal Development of the Hand and Wrist. Ed. 2. Stanford Univ. Press, Stanford, 1959.
16. GUDERMAN, J.R. - The diencephalic syndrome with cerebro-spinal fluid pleocytosis. J. Flor. med. Assoc. 64:365, 1977.
17. HÄGER, A. - The diencephalic syndrome of emaciation: a case report. Eur. Neurol. 7:130, 1972.
18. HÄGER, A. & THORELL, J.I. - Studies on growth hormone secretion in a patient with the diencephalic syndrome of emaciation. Acta paediatr. scand. 62: 231, 1973.
19. KAGAN, H. - Anorexia and severe inanition associated with a tumour involving the hypothalamus. Arch. Dis. Child. 257, 1957.
20. LIN, J.P.; PAY, N. ; NAIDICH, T.P. ; KRICHEFF, I.I. & WIGGLI, TJ. - Computed tomography in the postoperative care of neurosurgical patients. Neuroradiololy 12:185, 1977.
21. MARKESBERY, W.R & McDONALD. J.V. - Diencephalic syndrome: long-term survival. Amer. J. Dis. Child. 125:123, 1973.
22. PELC, S. - The diencephalic syndrome in infants: a review in relation to optic nerve glioma. Eur. Neurol. 7:321, 1972.
23. PELC, S. ; SEGERS-CADRANEL, A.; PIEPSZ, A. & DE COSTRE, P.L. - Brain scanning in the diencephalic syndrome. Neuroradiology 7:109, 1974.
24. PINSTONE, B.L. ; SOBEL, I.; MEYER, E. & EALE, D. - Secretion of growth hormone in the diencephalic syndrome of childhood. J. Pediatr. 76:886, 1970.
25. PITLYK, P.J. ; MILLER, R.H. & JOHNSON, G.M. - Diencephalic syndrome of infancy presenting with anorexia and emaciation: report of a case. Mayo Clin. Proc. 40:327, 1965.
26. POZNANSKI, A.K. & MANSON, G. - Radiographic appearance of the soft tissues in the diencephalic syndromes of infancy. Radiology 81:101, 1963.
27. REICHLIN, S. - Neuroendocrinoly. In R.H. Williams, J.D. Wilson & D.W. Foster (eds.): Textbook of Endocrinology. Ed. 7. W.B. Saunders, Philadelphia, 1985, pg. 492.
28. RUSSEL, A. - A diencephalic syndrome of emaciation in infancy and childhood. Arch. Dis. Child. 26:274, 1951.
29. SALMON, M.A. - Russel's diencephalic syndrome of early childhood. J. Neurol. Neurosurg. Psychiat. 35:196, 1972.
30. SCHULTZ, I.S. - Síndrome de Russel ou síndrome diencefálica caquetizante do lactente e da primeira infancia. Tese. Rio de Janeiro. Resumo JBM 09/1974.
31. WAGA, S. ; SHIMIZU, T. & SAKAKURA, M. - Diencephalic syndrome of emaciation (Russel's syndrome). Surg. Neurol. 17:141, 1982.
32. WHITE, Ph. T. & ROSS, A.T. - Inanition syndrome in infants with anterior hypothalamic neoplasm. Neurology 13:974, 1963.

Publication Dates

Publication in this collection
22 June 2011
Date of issue
Mar 1987

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

[1] 1. ADDY, D.P. & HUDSON, F.P. - Diencephalic syndrome of infantile emaciation. Arch. Dis. Child. 47:388, 1972.

[2] 2. ANDLER, W. ; STOLECK, H. & SIRANG, H. - Endocrine dysfunction in the diencephalic syndrome of emaciation in infancy. Helv. paediatr. Acta. 33:393, 1978.

[3] 3. BAIN, H.W. ; DARTZ, J.M.M. ; KEITH, W.S. & KRUYFF, E. - The diencephalic syndrome of early infancy due to silent brain tumor: with special reference to treatment. Pediatrics 38:473, 1966.

[4] 4. BRAUN, F.C. & FORNEY, W.R. - Diencephalic syndrome of early infancy associated with brain tumor. Pediatrics 10:609, 1959.

[5] 5. BURR, I.M.; SLONIN, A.E.; DANISH, R.K. ; GADOTH, N. & BUTLER, I.J. - Diencephalic syndrome revisited. J. Pediatr. 88:439, 1976.

[6] 6. CHEMALE, I.M.; MEDINA, G.R.M. & FARINATTT, F. - Síndrome de Russel: relato de um caso. Acta neurol. latinoamer. 26 : 275, 1980.

[7] 7. CHOUX, M.; BAURAND, C. ; PIERRON, H. & VIGOUROUX, R.P. - Lipoatrophie cachectisante par tumeur de la partie antérieure du plancher du IHe. ventricule (Syndrome de Russel). Neuro-Chirurgie 15:59, 1969.

[8] 8. DANZIGER, J. & BLOCH, S. - Hypothalamic tumours presenting as the diencephalic syndrome. Clin. Radiol. 25:153, 1974.

[9] 9. DODS, L. - A diencephalic syndrome of early infancy. Med. J. Aust. 9:689, 1957.

[10] 10. DODS, L. - A diencephalic syndrome of early infancy. Med. J. Aust. 4:222, 1967.

[11] 11. DROP, S.L.S. ; GUYDA, H.J. & COLLE, E. - Inappropriate growth hormone release in the diencephalic syndrome of childhood: case report and 4 year endocrinological follow-up. Clin. Endocrinol. 13:181, 1980.

[12] 12. FERREIRA, N.P. & OLIVEIRA, E.A. - Síndrome de Russel ou síndrome diencefálica de emagrecimento. Arq. Neuro-Psiquiatr. (São Paulo) 29:207, 1971.

[13] 13. FISHMAN, M.A. & PEAKE, G.T. - Paradoxical growth in a patient with the diencephalic syndrome. Pediatrics 45:973, 1970.

[14] 14. FRAS1ER, S.D. - Abnormalities of sexual development. In Pediatric Endocrinology. Grune & Stratton, New York, 1980, pg. 147.

[15] 15. GREULICH, W.W. & PYLE, S.I. - Radiographic Atlas of Skeletal Development of the Hand and Wrist. Ed. 2. Stanford Univ. Press, Stanford, 1959.

[16] 16. GUDERMAN, J.R. - The diencephalic syndrome with cerebro-spinal fluid pleocytosis. J. Flor. med. Assoc. 64:365, 1977.

[17] 17. HÄGER, A. - The diencephalic syndrome of emaciation: a case report. Eur. Neurol. 7:130, 1972.

[18] 18. HÄGER, A. & THORELL, J.I. - Studies on growth hormone secretion in a patient with the diencephalic syndrome of emaciation. Acta paediatr. scand. 62: 231, 1973.

[19] 19. KAGAN, H. - Anorexia and severe inanition associated with a tumour involving the hypothalamus. Arch. Dis. Child. 257, 1957.

[20] 20. LIN, J.P.; PAY, N. ; NAIDICH, T.P. ; KRICHEFF, I.I. & WIGGLI, TJ. - Computed tomography in the postoperative care of neurosurgical patients. Neuroradiololy 12:185, 1977.

[21] 21. MARKESBERY, W.R & McDONALD. J.V. - Diencephalic syndrome: long-term survival. Amer. J. Dis. Child. 125:123, 1973.

[22] 22. PELC, S. - The diencephalic syndrome in infants: a review in relation to optic nerve glioma. Eur. Neurol. 7:321, 1972.

[23] 23. PELC, S. ; SEGERS-CADRANEL, A.; PIEPSZ, A. & DE COSTRE, P.L. - Brain scanning in the diencephalic syndrome. Neuroradiology 7:109, 1974.

[24] 24. PINSTONE, B.L. ; SOBEL, I.; MEYER, E. & EALE, D. - Secretion of growth hormone in the diencephalic syndrome of childhood. J. Pediatr. 76:886, 1970.

[25] 25. PITLYK, P.J. ; MILLER, R.H. & JOHNSON, G.M. - Diencephalic syndrome of infancy presenting with anorexia and emaciation: report of a case. Mayo Clin. Proc. 40:327, 1965.

[26] 26. POZNANSKI, A.K. & MANSON, G. - Radiographic appearance of the soft tissues in the diencephalic syndromes of infancy. Radiology 81:101, 1963.

[27] 27. REICHLIN, S. - Neuroendocrinoly. In R.H. Williams, J.D. Wilson & D.W. Foster (eds.): Textbook of Endocrinology. Ed. 7. W.B. Saunders, Philadelphia, 1985, pg. 492.

[28] 28. RUSSEL, A. - A diencephalic syndrome of emaciation in infancy and childhood. Arch. Dis. Child. 26:274, 1951.

[29] 29. SALMON, M.A. - Russel's diencephalic syndrome of early childhood. J. Neurol. Neurosurg. Psychiat. 35:196, 1972.

[30] 30. SCHULTZ, I.S. - Síndrome de Russel ou síndrome diencefálica caquetizante do lactente e da primeira infancia. Tese. Rio de Janeiro. Resumo JBM 09/1974.

[31] 31. WAGA, S. ; SHIMIZU, T. & SAKAKURA, M. - Diencephalic syndrome of emaciation (Russel's syndrome). Surg. Neurol. 17:141, 1982.

[32] 32. WHITE, Ph. T. & ROSS, A.T. - Inanition syndrome in infants with anterior hypothalamic neoplasm. Neurology 13:974, 1963.

Brasil

Brasil

Russel's syndrome: a case with precocious puberty development

Síndrome de Russel: apresentação de um caso com desenvolvimento de puberdade precoce

Abstracts

Publication Dates