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Late MR follow-up of hypothalamic hamartomas

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Abstract

The absence of changes over time in the diagnostic features of suspected hypothalamic hamartomas is of paramount importance. Since magnetic resonance (MR) imaging is very sensitive to modifications in the brain parenchyma, a late MR follow-up study was performed in five children. In all cases, the diagnosis of hypothalamic hamartoma has been suspected on the basis of the association of central precocious puberty and the presence of a mass in the inferior aspect of the hypothalamus, demonstrated on previous MR studies. Late MR evaluation (after a mean of 39 months) demonstrated stability of the lesions in shape, size, and signal intensity. In three cases the lesions demonstrated a rim of isointense signal with a hyperintense center on T2-weighted sequences. In two cases a Chiari I malformation was found in association with the hypothalamic malformation. In one case a pineal cyst was demonstrated. These unusual findings are discussed. Late MR follow-up showed the absence of changes in the lesions over time, allowing the diagnosis to be confirmed.

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References

  1. Barral V, Brunelle F, Brauner R, Rappaport R, Lallemand D (1988) MRI of hypothalamic hamartomas in children. Pediatr Radiol 18:449–452

    PubMed  Google Scholar 

  2. Beningfield SJ, Bonnici F, Cremin BJ (1988) Magnetic resonance imaging of hypothalamic hamartomas. Br J Radiol 61:1177–1180

    PubMed  Google Scholar 

  3. Berkovic SF, Andermann F, Melanson D, Ethier RE, Feindel W, Gloor P (1988) Hypothalamic hamartomas and ictal laughter: evolution of a characteristic epileptic syndrome and diagnostic value of magnetic resonance imaging. Ann Neurol 23:429–439

    PubMed  Google Scholar 

  4. Boyko OB, Curnes JT, Oakes WJ, Burger PC (1991) Hamartomas of the tuber cinereum: CT, MR, and pathologic findings. AJNR 12:309–314

    PubMed  Google Scholar 

  5. Burton EM, Ball WS Jr, Crone K, Dolan LM (1989) Hamartoma of the tuber cinereum: a comparison of MR and CT findings in four cases. AJNR 10:497–501

    PubMed  Google Scholar 

  6. Clarren SK, Alvord EC Jr, Hall JC (1980) Congenital hypothalamic hamartoblastoma, hypopituitarism, imperforate anus, and postaxial polydactyly: a new syndrome? II. Neuropathological considerations. Am J Med Genet 7:75–83

    PubMed  Google Scholar 

  7. Diaz LL, Grech KF, Prados MD (1991) Hypothalamic hamartoma associated with Laurence-Moon-Biedl syndrome. Case report and review of the literature. Pediatr Neurosurg 17:30–33

    PubMed  Google Scholar 

  8. Diebler C, Ponsot G (1983) Hamartomas of the tuber cinereum. Neuroradiology 25:93–101

    PubMed  Google Scholar 

  9. Fitz CR (1982) Midline anomalies of the brain and spine. Radiol Clin North Am 20:95–104

    PubMed  Google Scholar 

  10. Gitlin G, Behar AJ (1986) Meningeal angiomatosis, arrhinencephaly agenesis of the corpus callosum and large hamartoma of the brain with neoplasms in an infant with bilateral nasal proboscis. Acta Anat (Basel) 41:56–79

    Google Scholar 

  11. Hahn FJ, Leibrock LG, Huseman CA, Makos MM (1988) The MR appearance of hypothalamic hamartoma. Neuroradiology 30:65–68

    PubMed  Google Scholar 

  12. Hall JG, Pallister PD, Clarren SK, Beckwith JB, Wiglesworth FW, Fraser FC, Cho S, Benke PJ, Reed SD (1980) Congenital hypothalamic hamartoblastoma, hypopituitarism, imperforate anus, and postaxial polydactyly: a new syndrome? I. Clinical, causal, and pathogenetic considerations. Am J Med Genet 7:47–74

    PubMed  Google Scholar 

  13. Hennekam RC, Beemer FA, Van Merrienboer F, Van Ketel BA, Kramer PP (1986) Congenital hypothalamic hamartoma associated with severe midline defect: a developmental field defect. Report of a case. Am J Med Genet [Suppl 2]:45–52

  14. Hubbard AM, Egelhoff JC (1989) MR imaging of large hypothalamic hamartomas in two infants. AJNR 10:1277

    PubMed  Google Scholar 

  15. Hunter JV, Youl BD, Moseley IF (1992) MRI demonstration of midbrain deformity in association with Chiari malformation. Neuroradiology 34:399–401

    PubMed  Google Scholar 

  16. Kortman KE, Bradley WG Jr (1988) Supratentorial neoplasms. In: Stark DD, Bradley WG Jr (eds) Magnetic resonance imaging. Mosby, St Louis, pp 410–411

    Google Scholar 

  17. Lange-Cosack M (1951) Verschiedene Gruppen der hypothalamischen Pubertas praecox, 1. Mitteilung. Dtsch Z Nervenheilkd 166:499–545

    PubMed  Google Scholar 

  18. Lin SR, Bryson MM, Gobien RP, Fitz CR, Lee YY (1978) Radiologic findings of hamartomas of the tuber cinereum and hypothalamus. Radiology 127:697–703

    PubMed  Google Scholar 

  19. List CF, Dowman CE, Bagchi BK, et al (1958) Posterior hypothalamic hamartomas and gangliomas causing precocious puberty. Neurology 3:164–174

    Google Scholar 

  20. Lona Soto A, Takahashi M, Yamashita Y, Sakamoto Y, Shinzato J, Yoshizumi K (1991) MRI findings of hypothalamic hamartoma: report of five cases and review of the literature. Comput Med Imaging Graph 15:415–421

    PubMed  Google Scholar 

  21. Marcuse PH, Burger RA, Salmon GN (1953) Hamartoma of the hypothalamus. J Pediatr 43:301–308

    PubMed  Google Scholar 

  22. Nishio S, Fujiwara S, Aiko Y, Takeshita I, Fukui M (1989) Hypothalamic hamartoma. Report of two cases. Neurosurgery 70:640–645

    Google Scholar 

  23. Paillas JE, Roger J, Toga M, Soulayrol R, Salamon G, Dravet C, Bureau M (1969) Hamartomes de l'hypothalamus. Rev Neurol 120:177–194

    PubMed  Google Scholar 

  24. Peterman SB, Steiner RE, Bydder GM (1984) Magnetic resonance imaging of intracranial tumors in children and adolescents. AJNR 5:703–709

    PubMed  Google Scholar 

  25. Rieth KG, Comite F, Dwyer AJ, Nelson MJ, Pescovitz O, Shawker TH, Cutler GB, Loriaux DL (1987) CT of cerebral abnormalities in precocious puberty. AJNR 8:283–290

    Google Scholar 

  26. Sato M, Ushio Y, Arita N, Mogami H (1985) Hypothalamic hamartoma: report of two cases. Neurosurgery 16:198–206

    PubMed  Google Scholar 

  27. Schmidt E, Hallervorden J, Spatz H (1958) Die Entstehung der Hamartome am Hypothalamus mit und ohne Pubertas praecox. Dtsch Z Nervenheilkd 177:235–262

    PubMed  Google Scholar 

  28. Weinberger L, Grant F (1941) Precocious puberty and tumors of the hypothalamus. Arch Intern Med 67:762–79

    Google Scholar 

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Turjman, F., Xavier, J.L., Froment, J.C. et al. Late MR follow-up of hypothalamic hamartomas. Child's Nerv Syst 12, 63–68 (1996). https://doi.org/10.1007/BF00819496

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