Literature DB >> 17447073

Hypothalamic hamartoma with bilateral anophthalmia.

Burçak Bilginer1, Atilla Akbay, Nejat Akalan.   

Abstract

INTRODUCTION: Hypothalamic hamartomas are congenital malformations. Clinically, they can be asymptomatic, but they cause seizures, mental retardation and precocious puberty in many cases. CASE REPORT: A 20-day-old boy with hypothalamic hamartoma and bilateral anophthalmia was presented. Except those, no other congenital anomaly was detected.
CONCLUSION: This is a rare case of hypothalamic hamartoma with bilateral anophthalmia. The mutations at SOX2 has an important role in the developing brain and eyes.

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Year:  2007        PMID: 17447073     DOI: 10.1007/s00381-007-0337-2

Source DB:  PubMed          Journal:  Childs Nerv Syst        ISSN: 0256-7040            Impact factor:   1.532


  9 in total

1.  Bilateral anophthalmia and brain malformations caused by a 20-bp deletion in the SOX2 gene.

Authors:  J C Zenteno; G Gascon-Guzman; J L Tovilla-Canales
Journal:  Clin Genet       Date:  2005-12       Impact factor: 4.438

2.  Cranial MR imaging findings in Waardenburg syndrome: anophthalmia, and hypothalamic hamartoma.

Authors:  R N Sener
Journal:  Comput Med Imaging Graph       Date:  1998 Sep-Oct       Impact factor: 4.790

3.  Pallister-Hall syndrome.

Authors:  L G Biesecker; J M Graham
Journal:  J Med Genet       Date:  1996-07       Impact factor: 6.318

4.  SOX2 mutation causes anophthalmia, hearing loss, and brain anomalies.

Authors:  Stephanie A Hagstrom; Gayle J T Pauer; Janet Reid; Ellen Simpson; Sue Crowe; Irene H Maumenee; Elias I Traboulsi
Journal:  Am J Med Genet A       Date:  2005-10-01       Impact factor: 2.802

5.  Diencephalic neuronal hamartoma associated with congenital obstructive hydrocephalus, anophthalmia, cleft lip and palate and severe mental retardation: a possible new syndrome.

Authors:  J P Rossiter; M M Khalifa; S Nag
Journal:  Acta Neuropathol       Date:  2000-06       Impact factor: 17.088

6.  Hypothalamic hamartoma: report of two cases.

Authors:  M Sato; Y Ushio; N Arita; H Mogami
Journal:  Neurosurgery       Date:  1985-02       Impact factor: 4.654

7.  Hypothalamic hamartoma: anatomic, immunohistochemical and ultrastructural features.

Authors:  H K Inoue; H Kanazawa; H Kohga; A Zama; N Ono; M Nakamura; C Ohye
Journal:  Noshuyo Byori       Date:  1995-03

8.  SOX2 anophthalmia syndrome.

Authors:  Nicola K Ragge; Birgit Lorenz; Adele Schneider; Kate Bushby; Luisa de Sanctis; Ugo de Sanctis; Alison Salt; J Richard O Collin; Anthony J Vivian; Samantha L Free; Pamela Thompson; Kathleen A Williamson; Sanjay M Sisodiya; Veronica van Heyningen; David R Fitzpatrick
Journal:  Am J Med Genet A       Date:  2005-05-15       Impact factor: 2.802

9.  Mutations within Sox2/SOX2 are associated with abnormalities in the hypothalamo-pituitary-gonadal axis in mice and humans.

Authors:  Daniel Kelberman; Karine Rizzoti; Ariel Avilion; Maria Bitner-Glindzicz; Stefano Cianfarani; Julie Collins; W Kling Chong; Jeremy M W Kirk; John C Achermann; Richard Ross; Danielle Carmignac; Robin Lovell-Badge; Iain C A F Robinson; Mehul T Dattani
Journal:  J Clin Invest       Date:  2006-08-24       Impact factor: 14.808

  9 in total

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