A case of neurofibromatosis 1 presenting with optic pathway glioma with an early onset and an aggressive course.

A case of neurofibromatosis 1 presenting with optic pathway glioma with an early onset and an aggressive course.
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DOI:
10.4103/0301-4738.39128
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发表时间:
2008-03
影响因子:
3.1
通讯作者:
Bhuttan S
Bhuttan S
中科院分区:
医学4区
文献类型:
--
作者:
Ganesh S;Gupta A;Sharma M;Bhuttan S

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与神经纤维瘤病1相关的视路胶质瘤具有典型的无痛病程。然而,涉及的视辐射是相对罕见的,并与一个更积极的过程。摘要一位三岁女童因左眼斜视及视力丧失而就诊,并伴有相对性瞳孔传入障碍及视盘苍白。她身上有很多Au lait咖啡色的斑点。视觉诱发电位提示视神经传导缺陷,脑磁共振成像提示视神经、视交叉和视束受累的视路胶质瘤。视放射和齿状核有错构瘤。视神经活检证实了毛细胞型星形细胞瘤。随后在全身麻醉下进行根治性放疗。本病例报告的目的是强调参与的光学辐射和异常积极的临床过程中,这种情况下。
Optic pathway glioma associated with neurofibromatosis 1 has a classically indolent course. However, involvement of the optic radiations is relatively rare and is associated with a more aggressive course. A three-year-old girl presented with strabismus and loss of vision in the left eye with relative afferent pupillary defect and optic disc pallor. She had multiple café au lait spots. Visually evoked potential was suggestive of an optic nerve conduction defect and magnetic resonance imaging of the brain was suggestive of an optic pathway glioma involving the optic nerves, the optic chiasma and the optic tracts. The optic radiations and the dentate nuclei had hamartomas. Optic nerve biopsy confirmed pilocytic astrocytoma. Radical radiotherapy under general anesthesia was subsequently given. This case report aims to highlight the involvement of the optic radiations and the unusually aggressive clinical course in this case.
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