Associação singular de síndrome de Kallmann e cisto aracnóide da fossa média: relato de caso

Fernandes, Yvens B.; Guerra Jr, Gil; Lemos, Sofia H. V.; Matias, Maria T.; Damasceno, Benito P.; Hamamoto, Osmi; Marconi Jr, Ary; Honorato, Donizetti C.; Marins, José L. C.

doi:10.1590/S0004-282X1995000400020

Resumos

O hipogonadismo hipogonadotrófico pode resultar de diferentes anomalias do sistema nervoso central, apresentando sinais clínicos que dependem da idade de aparecimento, bem como do grau de deficiência gonadotrófica e de sua associação com outras deficiências hipofisárias. Relatamos o caso de um rapaz de 18 anos com atraso puberal, retardo de crescimento estatural a partir de 10 anos e história de dificuldade de aprendizado escolar. Ao exame apresentava discreto aspecto eunucóide, 162 cm de altura (z score = -2,17), estadiamento puberal GII, PII, testículos de 4 cm³. A avaliação complementar demonstrou níveis pré-puberais de testosterona, megateste com resposta normal, exceto ao teste de estímulo com GnRH agudo e prolongado. CT de crânio mostrou cisto aracnóide da fossa média esquerda com extensão supra-selar. Foi feito o diagnóstico de hipogonadismo hipogonadotrófico provavelmente secundário à compressão pelo cisto aracnóide e por isso, optou-se por derivação cisto-peritoneal. Após a cirurgia não houve retomada do desenvolvimento puberal, sendo então verificada anosmia bilateral e, portanto, diagnosticada Síndrome de Kallmann, que foi confirmada pelos achados da RNM, embora os resultados hormonais não sejam totalmente compatíveis com a referida síndrome. Não encontramos na literatura descrição da associação entre Síndrome de Kallmnann e cisto aracnóide e acreditamos que neste caso os resultados da avaliação hormonal possam ser devidos a tal associação que provocou adicionalmente uma disfunção hipofisária.

hipogonadismo; cisto aracnóide; Síndrome de Kallmann

Hypogonadotrophic hypogonadism can result from different abnormalities in the central nervous system. The clinical picture depends upon the time of onset the deficiency, the magnitude of the gonadotropins deficiency and whether there are other pituitary hormone deficiencies as well. We report on a 18-year-old boy, who was investigated because of pubertal and growth delay. He also had learning disabilities. On physical examination he exhibited mild eunuchoid aspect, 162 cm height (z score = -2,17), pubertal development on stage G II, P II, and 4 cm5 testis. Laboratory investigation revealed pre-pubertal levels of testosterone and normal results of the combined test of anterior pituitary function, except for in GnRH acute and prolonged test. Brain CT showed an arachnoid cyst on left middle fossa with expansion to suprasellar cisterna. He was diagnosed as having hypogonadotrophic hypogonadism secondary to compression by the cyst, and a cyst-peritoneal derivation was performed. After surgery there was no improvement of the pubertal state and bilateral anosmia was discovered, so Kallmann's syndrome was then diagnosed and was confirmed by MRI, even though the hormonal results are not totally matched with the refered syndrome. We did not find in the literature any description of the association between Kallmann's syndrome and arachnoid cyst and we believe that in this case the results of the hormonal measurement may be due to such association that provoked an additional hypophysis dysfunction.

hypogonadism; arachnoid cyst; Kallmann's Syndrome

Associação singular de síndrome de Kallmann e cisto aracnóide da fossa média, relato de caso

Singular association of Kallmann's syndrome and arachnoid cyst of middle fossa: case report

Yvens B. Fernandes ^I; Gil Guerra Jr^II; Sofia H. V. Lemos^II; Maria T. Matias^II; Benito P. Damasceno^I; Osmihamamoto^I; Ary Marconi Jr^I; Donizetti C. Honorato ^I; José L. C. Marins ^III

^IDisciplina de Neurocirurgia, Departamento de Neurologia. Estudo realizado na Faculdade de Ciências Médicas (FCM) da Universidade Estadual de Campinas (UNICAMP)

^IISetor de Endocrinologia Pediátrica, Departamento de PediatriaEstudo realizado na Faculdade de Ciências Médicas (FCM) da Universidade Estadual de Campinas (UNICAMP)

^IIIDepartamento de Radiologia. Estudo realizado na Faculdade de Ciências Médicas (FCM) da Universidade Estadual de Campinas (UNICAMP)

RESUMO

O hipogonadismo hipogonadotrófico pode resultar de diferentes anomalias do sistema nervoso central, apresentando sinais clínicos que dependem da idade de aparecimento, bem como do grau de deficiência gonadotrófica e de sua associação com outras deficiências hipofisárias. Relatamos o caso de um rapaz de 18 anos com atraso puberal, retardo de crescimento estatural a partir de 10 anos e história de dificuldade de aprendizado escolar. Ao exame apresentava discreto aspecto eunucóide, 162 cm de altura (z score = -2,17), estadiamento puberal GII, PII, testículos de 4 cm³. A avaliação complementar demonstrou níveis pré-puberais de testosterona, megateste com resposta normal, exceto ao teste de estímulo com GnRH agudo e prolongado. CT de crânio mostrou cisto aracnóide da fossa média esquerda com extensão supra-selar. Foi feito o diagnóstico de hipogonadismo hipogonadotrófico provavelmente secundário à compressão pelo cisto aracnóide e por isso, optou-se por derivação cisto-peritoneal. Após a cirurgia não houve retomada do desenvolvimento puberal, sendo então verificada anosmia bilateral e, portanto, diagnosticada Síndrome de Kallmann, que foi confirmada pelos achados da RNM, embora os resultados hormonais não sejam totalmente compatíveis com a referida síndrome. Não encontramos na literatura descrição da associação entre Síndrome de Kallmnann e cisto aracnóide e acreditamos que neste caso os resultados da avaliação hormonal possam ser devidos a tal associação que provocou adicionalmente uma disfunção hipofisária.

Palavras-chave:hipogonadismo, cisto aracnóide, Síndrome de Kallmann.

SUMMARY

Hypogonadotrophic hypogonadism can result from different abnormalities in the central nervous system. The clinical picture depends upon the time of onset the deficiency, the magnitude of the gonadotropins deficiency and whether there are other pituitary hormone deficiencies as well. We report on a 18-year-old boy, who was investigated because of pubertal and growth delay. He also had learning disabilities. On physical examination he exhibited mild eunuchoid aspect, 162 cm height (z score = -2,17), pubertal development on stage G II, P II, and 4 cm⁵ testis. Laboratory investigation revealed pre-pubertal levels of testosterone and normal results of the combined test of anterior pituitary function, except for in GnRH acute and prolonged test. Brain CT showed an arachnoid cyst on left middle fossa with expansion to suprasellar cisterna. He was diagnosed as having hypogonadotrophic hypogonadism secondary to compression by the cyst, and a cyst-peritoneal derivation was performed. After surgery there was no improvement of the pubertal state and bilateral anosmia was discovered, so Kallmann's syndrome was then diagnosed and was confirmed by MRI, even though the hormonal results are not totally matched with the refered syndrome. We did not find in the literature any description of the association between Kallmann's syndrome and arachnoid cyst and we believe that in this case the results of the hormonal measurement may be due to such association that provoked an additional hypophysis dysfunction.

Key words:hypogonadism, arachnoid cyst, Kallmann's Syndrome.

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Full text available only in PDF format.

Aceite: 25-abril-1995.

Dr. Yvens Barbosa Fernandes - Departamento de Neurologia. Hospital de Clínicas da UNICAMP - Caixa Postal 6111 - 13081-970 Campinas SP - Brasil.

1. Aicardi J, Bauman F. Supratentorial extracerebral cysts in infants and children. J Neurol Neurosurg Psychiatry 1975, 38:57-68.
2. Bell J, Spitz I, Slonin A. Heterogeneity of gonadotropin response to LHRH in hypogonadotropic hypogonadism. J Clin Endocrinol Metab 1973, 36:791-794.
3. Binitie O, Williams B, Case CP. A suprasellar subarachnoid pouch: aetiological considerations. J Neurol Neurosurg Psyquiatry 1984, 47:1066-1074.
4. Boyar RM, Finkelstein JW, Witkin M. Studies of endocrine function in "isolated" gonadotropin deficiency. J Clin Endocrinol Metab 1973, 36:64-72.
5. Choux M, Raybaud C, Pinsard N, Hassoun J, Gambarelli D. Intracranial supratentorial cysts in children excluding tumor and parasite cysts. Child Brain 1978, 4:15-32.
6. Christensen AL. Luna's neuropsychological investigation. Copenhagen:Munksgaard, 1979.
7. Di Rocco C. Arachnoid cysts. In Youmans JR (ed). Neurological surgery.Ed 3. Philadelphia: W. B. Saunders, 1990, p 1299-1325.
8. Klingmüller D, Dewe W, Krahe T, Brecht G, Scheikert H. Magnetic resonance imaging of the brain in patients with anosmia and hypothalamic hypogonadism (Kallmann's syndrome). J Clin Endocrinol Metab 1987, 65:581-584.
9. Lieblich JM, Rogol AD, White BJ. Syndrome of anosmia with hypogonadotropic hypogonadism (Kallmann's syndrome): clinical and laboratory studies in 23 cases. Am J Med 1982, 73:506-519.
10. Marshall JC, Harsoulis P, Anderson DC. Isolated pituitary gonadotrophin deficiency: gonadotrophin secretion after synthetic luteinizing hormone and follicle stimulating hormone-releasing hormone. Br Med J 1972, 4:643-645.
11. Oettinger M, Bruneteau DW, Pasaroudakis A. FSH and LH response to LHRF in Kallmann's syndrome. Obstetr Gynecol 1976, 47: 233-236.
12. Pierre-Kahn A, Capelle L, Brauner R, Sainte-Rose C, Renier D, Rappaport R, Hirsch JF. Presentation and management of suprasellar arachnoid cysts. J Neurosurg 1990, 73:355-359.
13. Reitano JF, Caminos-Torres R, Snyder PJ. Serum LH and FSH responses to the repetitive administration of gonadotropin-releasing hormone in patients with idiopatic hypogonadotropic hypogonadism. J Clin Endocrinol Metab 1975, 41:1035-1042.
14. Robinson RG. The temporal lobe agenesia syndrome. Brain 1964, 87:87-106.
15. Robinson RG. Congenital cysts of the brain: arachnoid malformation. Progr Neurol Surg 1971,4:133-174.
16. Snyder PJ, Rudenstein RS, Gardner DF. Repetitive infusion of gonadotropin-releasing hormone distinguishes hypothalamic from pituitary hypogonadism. J Clin Endocrinol Meiab 1979, 48:864-868.
17. Starkman SP, Brown TC, Linell EA. Cerebral arachnoid cysts. J Neuropathol Exp Neurol 1958, 17:484-500.
18. Styne DM. Puberty and its disorders in boys. Endocrinol Metab Clin North Am 1991, 20:43-70.
19. Van der Meché FGA, Braakman R. Arachnoid cysts in the middle fossaxause and treatment symptoms. J Neurol Neurosurg Psychiatry 1983, 46:1102-1107.

Datas de Publicação

Publicação nesta coleção
21 Dez 2010
Data do Fascículo
Set 1995

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

[1] 1. Aicardi J, Bauman F. Supratentorial extracerebral cysts in infants and children. J Neurol Neurosurg Psychiatry 1975, 38:57-68.

[2] 2. Bell J, Spitz I, Slonin A. Heterogeneity of gonadotropin response to LHRH in hypogonadotropic hypogonadism. J Clin Endocrinol Metab 1973, 36:791-794.

[3] 3. Binitie O, Williams B, Case CP. A suprasellar subarachnoid pouch: aetiological considerations. J Neurol Neurosurg Psyquiatry 1984, 47:1066-1074.

[4] 4. Boyar RM, Finkelstein JW, Witkin M. Studies of endocrine function in "isolated" gonadotropin deficiency. J Clin Endocrinol Metab 1973, 36:64-72.

[5] 5. Choux M, Raybaud C, Pinsard N, Hassoun J, Gambarelli D. Intracranial supratentorial cysts in children excluding tumor and parasite cysts. Child Brain 1978, 4:15-32.

[6] 6. Christensen AL. Luna's neuropsychological investigation. Copenhagen:Munksgaard, 1979.

[7] 7. Di Rocco C. Arachnoid cysts. In Youmans JR (ed). Neurological surgery.Ed 3. Philadelphia: W. B. Saunders, 1990, p 1299-1325.

[8] 8. Klingmüller D, Dewe W, Krahe T, Brecht G, Scheikert H. Magnetic resonance imaging of the brain in patients with anosmia and hypothalamic hypogonadism (Kallmann's syndrome). J Clin Endocrinol Metab 1987, 65:581-584.

[9] 9. Lieblich JM, Rogol AD, White BJ. Syndrome of anosmia with hypogonadotropic hypogonadism (Kallmann's syndrome): clinical and laboratory studies in 23 cases. Am J Med 1982, 73:506-519.

[10] 10. Marshall JC, Harsoulis P, Anderson DC. Isolated pituitary gonadotrophin deficiency: gonadotrophin secretion after synthetic luteinizing hormone and follicle stimulating hormone-releasing hormone. Br Med J 1972, 4:643-645.

[11] 11. Oettinger M, Bruneteau DW, Pasaroudakis A. FSH and LH response to LHRF in Kallmann's syndrome. Obstetr Gynecol 1976, 47: 233-236.

[12] 12. Pierre-Kahn A, Capelle L, Brauner R, Sainte-Rose C, Renier D, Rappaport R, Hirsch JF. Presentation and management of suprasellar arachnoid cysts. J Neurosurg 1990, 73:355-359.

[13] 13. Reitano JF, Caminos-Torres R, Snyder PJ. Serum LH and FSH responses to the repetitive administration of gonadotropin-releasing hormone in patients with idiopatic hypogonadotropic hypogonadism. J Clin Endocrinol Metab 1975, 41:1035-1042.

[14] 14. Robinson RG. The temporal lobe agenesia syndrome. Brain 1964, 87:87-106.

[15] 15. Robinson RG. Congenital cysts of the brain: arachnoid malformation. Progr Neurol Surg 1971,4:133-174.

[16] 16. Snyder PJ, Rudenstein RS, Gardner DF. Repetitive infusion of gonadotropin-releasing hormone distinguishes hypothalamic from pituitary hypogonadism. J Clin Endocrinol Meiab 1979, 48:864-868.

[17] 17. Starkman SP, Brown TC, Linell EA. Cerebral arachnoid cysts. J Neuropathol Exp Neurol 1958, 17:484-500.

[18] 18. Styne DM. Puberty and its disorders in boys. Endocrinol Metab Clin North Am 1991, 20:43-70.

[19] 19. Van der Meché FGA, Braakman R. Arachnoid cysts in the middle fossaxause and treatment symptoms. J Neurol Neurosurg Psychiatry 1983, 46:1102-1107.