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dc.contributor.author
Bernardini, Francesco P.
dc.contributor.author
Croxatto, Juan Oscar
dc.contributor.author
Nozza, Paolo
dc.contributor.author
Rossi, Andrea
dc.contributor.author
Capris, Paolo
dc.date.available
2017-02-01T20:17:09Z
dc.date.issued
2013-03
dc.identifier.citation
Bernardini, Francesco P.; Croxatto, Juan Oscar; Nozza, Paolo; Rossi, Andrea; Capris, Paolo; Primary Diffuse Leptomeningeal Gliomatosis in Children: A Clinical Pathologic Correlation; Lippincott Williams; Ophthalmic Plastic And Reconstructive Surgery.; 29; 2; 3-2013; 93-97
dc.identifier.issn
0740-9303
dc.identifier.uri
http://hdl.handle.net/11336/12316
dc.description.abstract
Purpose: To describe a rare case of primary diffuse leptomeningeal gliomatosis (PDLG) presenting with progressive proptosis and direct involvement of the optic nerve sheath in a child and review of the relevant literature. Methods: Retrospective review of a single case and systematic literature review of 26 biopsy-proven cases reported in the MEDLINE-indexed English literature. A 10-year-old girl developed proptosis and progressive visual loss associated with thickening of the optic nerve sheaths and dilation of the subarachnoid spaces with multilobulated appearance of the brain meninges and thickened peripheral nerve root sheaths. Biopsy of the optic nerve sheath was diagnostic. The patient underwent chemotherapy combined with oral temozolomide and conformational radiotherapy to the brain and spine. She died 3 years after the onset of the disease. An extensive review of the published literature using the key words “primary diffuse leptomeningeal gliomatosis” and “optic nerve” confirmed the case herein reported to be the first case of primary diffuse leptomeningeal gliomatosis in which direct optic nerve infiltration was demonstrated during the course of the disease. Results: Immunohistochemistry demonstrated expression of CD56 and glial fibrillary acidic protein, and an elevated level of Ki-67; all the other markers were negative. Conclusions: According to a comprehensive literature review, we report the first case of PDLG that presented with bilateral proptosis and direct involvement of the optic nerve during the course of the disease. These new findings may explain an alternative mechanism of visual loss and proptosis in PDLG. We emphasize the importance of close collaboration between neurologists and ophthalmologists in all cases of visual symptoms associated with a neurologic condition. In case of optic nerve involvement, ophthalmologists could provide an easier route to achieve tissue specimen early in the course of this rare and fatal disease
dc.format
application/pdf
dc.language.iso
eng
dc.publisher
Lippincott Williams
dc.rights
info:eu-repo/semantics/restrictedAccess
dc.rights.uri
https://creativecommons.org/licenses/by-nc-sa/2.5/ar/
dc.subject
Gliomatosis
dc.subject
Children
dc.subject.classification
Oftalmología
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Medicina Clínica
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CIENCIAS MÉDICAS Y DE LA SALUD
dc.title
Primary Diffuse Leptomeningeal Gliomatosis in Children: A Clinical Pathologic Correlation
dc.type
info:eu-repo/semantics/article
dc.type
info:ar-repo/semantics/artículo
dc.type
info:eu-repo/semantics/publishedVersion
dc.date.updated
2017-01-30T16:03:26Z
dc.journal.volume
29
dc.journal.number
2
dc.journal.pagination
93-97
dc.journal.pais
Estados Unidos
dc.journal.ciudad
Filadelfia
dc.description.fil
Fil: Bernardini, Francesco P.. Ospedale Gaslini. Department of Ophthalmology; Italia
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Fil: Croxatto, Juan Oscar. Consejo Nacional de Investigaciones Científicas y Técnicas; Argentina. Ospedale Gaslini. Department of Neuroradiology; Italia. Fundación Oftalmológica Argentina "J. Malbrán". Departamento de Patologia Ocular y Ultraestructura; Argentina
dc.description.fil
Fil: Nozza, Paolo. Ospedale Gaslini. Department of Pathology; Italia
dc.description.fil
Fil: Rossi, Andrea. Ospedale Gaslini. Department of Neuroradiology; Italia. Fundación Oftalmológica Argentina "J. Malbrán". Departamento de Patologia Ocular y Ultraestructura; Argentina
dc.description.fil
Fil: Capris, Paolo. Ospedale Gaslini. Department of Ophthalmology; Italia
dc.journal.title
Ophthalmic Plastic And Reconstructive Surgery.
dc.relation.alternativeid
info:eu-repo/semantics/altIdentifier/url/http://journals.lww.com/op-rs/pages/articleviewer.aspx?year=2013&issue=03000&article=00004&type=abstract
dc.relation.alternativeid
info:eu-repo/semantics/altIdentifier/doi/http://dx.doi.org/10.1097/IOP.0b013e318279fe23


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