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MeSH Review

Optic Nerve Glioma

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Disease relevance of Optic Nerve Glioma

  • The peripheral CSF-intensity signal in orbital optic glioma correlates with the histopathological finding of perineural arachnoidal gliomatosis and serves as a neuroradiologic marker for neurofibromatosis [1].
  • In the present study, patients with optic gliomas and Type 1 neurofibromatosis (NF-1) were compared with patients with optic gliomas without NF-1, with respect to survival, time to tumor progression, and tumor location [2].
  • Although many of their complications are unfortunately still difficult or impossible to treat effectively, there may be significant benefit to the patient from prompt detection of optic glioma in NF, retinal angiomas in VHL disease, and glaucoma in SWS [3].
  • The association of optic glioma and other intracranial neoplasms in patients with NF1 suggests that there are fundamental pathophysiological differences between patients with and without optic glioma [4].
  • We identified a large deletion in the NF1 gene in a patient with a rhabdomyosarcoma as well as a variation in intron 22 in a patient with an optic glioma [5].

High impact information on Optic Nerve Glioma


Chemical compound and disease context of Optic Nerve Glioma


Biological context of Optic Nerve Glioma


Anatomical context of Optic Nerve Glioma


Gene context of Optic Nerve Glioma

  • CASE: A 4-year-old girl with neurofibromatosis type 1 and GH excess associated with optic glioma received pegvisomant injections (10 mg subcutaneously) with increasing intervals from daily to every 4th day [15].
  • Frequent eye disorders were: Lisch nodules (Iris hamartomas, IH) 78%, hyperthelorism 19.7%, bulbomotoric disorders 15.9%, disorders of the optic disc 16.7% and optic gliomas (18.9%) [16].

Analytical, diagnostic and therapeutic context of Optic Nerve Glioma


  1. The "pseudo-CSF" signal of orbital optic glioma on magnetic resonance imaging: a signature of neurofibromatosis. Brodsky, M.C. Survey of ophthalmology. (1993) [Pubmed]
  2. Prognostic significance of type 1 neurofibromatosis (von Recklinghausen Disease) in childhood optic glioma. Deliganis, A.V., Geyer, J.R., Berger, M.S. Neurosurgery (1996) [Pubmed]
  3. Ocular and dermatologic manifestation of neurocutaneous syndromes. Greenwald, M.J., Paller, A.S. Dermatologic clinics. (1992) [Pubmed]
  4. An association between optic glioma and other tumours of the central nervous system in neurofibromatosis type 1. Friedman, J.M., Birch, P. Neuropediatrics. (1997) [Pubmed]
  5. Molecular genetic analyses in neurofibromatosis type 1 patients with tumors. Oguzkan, S., Terzi, Y.K., Cinbis, M., Anlar, B., Aysun, S., Ayter, S. Cancer Genet. Cytogenet. (2006) [Pubmed]
  6. Inactivation of NF1 in CNS causes increased glial progenitor proliferation and optic glioma formation. Zhu, Y., Harada, T., Liu, L., Lush, M.E., Guignard, F., Harada, C., Burns, D.K., Bajenaru, M.L., Gutmann, D.H., Parada, L.F. Development (2005) [Pubmed]
  7. A clinical study of type 1 neurofibromatosis in north west England. McGaughran, J.M., Harris, D.I., Donnai, D., Teare, D., MacLeod, R., Westerbeek, R., Kingston, H., Super, M., Harris, R., Evans, D.G. J. Med. Genet. (1999) [Pubmed]
  8. Methylphenidate and melatonin for sleep disorder with optic glioma. Zotter, H., Kerbl, R., Millner, M., Kurz, R. Journal of the American Academy of Child and Adolescent Psychiatry. (2001) [Pubmed]
  9. Spontaneous regression of optic gliomas: thirteen cases documented by serial neuroimaging. Parsa, C.F., Hoyt, C.S., Lesser, R.L., Weinstein, J.M., Strother, C.M., Muci-Mendoza, R., Ramella, M., Manor, R.S., Fletcher, W.A., Repka, M.X., Garrity, J.A., Ebner, R.N., Monteiro, M.L., McFadzean, R.M., Rubtsova, I.V., Hoyt, W.F. Arch. Ophthalmol. (2001) [Pubmed]
  10. Chiasmatic optic glioma treated with chemotherapy. A preliminary report. Rosenstock, J.G., Packer, R.J., Bilaniuk, L., Bruce, D.A., Radcliffe, J.L., Savino, P. J. Neurosurg. (1985) [Pubmed]
  11. Carboplatin for the treatment of children with newly diagnosed optic chiasm gliomas: a phase II study. Aquino, V.M., Fort, D.W., Kamen, B.A. J. Neurooncol. (1999) [Pubmed]
  12. Precocious puberty associated with neurofibromatosis and optic gliomas. Treatment with luteinizing hormone releasing hormone analogue. Laue, L., Comite, F., Hench, K., Loriaux, D.L., Cutler, G.B., Pescovitz, O.H. Am. J. Dis. Child. (1985) [Pubmed]
  13. No aberrant methylation of neurofibromatosis 1 gene (NF1) promoter in pilocytic astrocytoma in childhood. Ebinger, M., Senf, L., Wachowski, O., Scheurlen, W. Pediatric hematology and oncology. (2005) [Pubmed]
  14. Optic gliomas in neurofibromatosis type 1: role of visual evoked potentials. North, K., Cochineas, C., Tang, E., Fagan, E. Pediatric neurology. (1994) [Pubmed]
  15. Pegvisomant treatment in a 4-year-old girl with neurofibromatosis type 1. Main, K.M., Sehested, A., Feldt-Rasmussen, U. Horm. Res. (2006) [Pubmed]
  16. Eye disorders in neurofibromatosis (NF1). Kordić, R., Sabol, Z., Cerovski, B., Katusić, D., Jukić, T. Collegium antropologicum. (2005) [Pubmed]
  17. Dural ectasia of the optic nerve sheath in neurofibromatosis type 1: CT and MR features. Lövblad, K.O., Remonda, L., Ozdoba, C., Huber, P., Schroth, G. Journal of computer assisted tomography. (1994) [Pubmed]
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