Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0017638 (glioma)
 30,880 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A case of ganglioglioma of the optic pathway associated with congenital exophthalmos and strabismus is presented. Since the tumor extended from the right optic nerve to the right geniculate body, it was diagnosed as an optic glioma before operation. However, optic nerve biopsy showed that the lesion was a ganglioglioma. Although a literature review yielded two previous cases of ganglioglioma of the optic pathway, this is the first case in which the tumor involved the whole optic pathway.
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PMID:Ganglioglioma of the optic pathway. A case report. 172 78

Gliomas of the optic nerve are rare, comprising only 1% of intracranial neoplasms in adults and 5% in children. This is the first recorded case of a ganglioglioma of the optic nerve. A 2-year-old White boy presented with a convergent squint and blindness of the left eye. At operation a pale, firm, fusiform swelling was found. The cytological and histopathological features are described.
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PMID:Ganglioglioma of the optic nerve. A case report. 685 3

A small number of children who develop disconjugate nystagmus, torticollis, and head titubation (spasmus nutans) have been found to have optic chiasm or third ventricle gliomas. However, the prevalence of glioma or other developmental abnormalities in this disorder is unknown because no large series of spasmus nutans cases has previously been reported. A reviewer of the records of 67 consecutive children initially diagnosed with spasmus nutans and followed for an average of 3.3 years at the St Louis Children's Hospital revealed the following: 61% had a history of prematurity, developmental delay, or other systemic abnormality; strabismus, most commonly infantile esotropia, developed in 55%; 43% had neuroimaging studies; and 0% had evidence of a glioma or showed signs of tumor on follow-up examinations. From this consecutive patient series, we estimate the prevalence of tumor in spasmus nutans to be less than 1.4%. Without other evidence of an intracranial mass lesion, neuroimaging of infants initially diagnosed with spasmus nutans may not be immediately warranted.
J Pediatr Ophthalmol Strabismus
PMID:Prevalence of intracranial lesions in children initially diagnosed with disconjugate nystagmus (spasmus nutans) 896 30

Eighteen patients with a glioma of the chiasma were managed between 1980 and 1994. A retrospective analysis of this series showed that the most frequent functional ophthalmological signs were visual loss, nystagmus, or strabismus. Funduscopic examination was abnormal in 65%, mostly with optic atrophy. Other exams (visual field and electrophysiology) were inadequate for diagnosis. Our series demonstrated that early diagnosis of involvement of the chiasma could improve functional prognosis as rapid management can improve prognosis.
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PMID:[Clinical features of gliomas of the chiasma. A retrospective study of a series of 18 patients]. 1099 65

A 4-month-old infant came to the department of Ophthalmology, King Chulalongkorn Memorial Hospital with right eye proptosis, strabismus, and no vision. She was diagnosed as optic nerve and chiasm glioma. The subtotal removal of the tumor was performed and followed by chemotherapy, with a satisfactory result. Because of the large size of the tumor and the presenting symptoms and signs since the patient was only 2 month old, we believe that this tumor originated in the intrauterine period. To our knowledge, this reported case is the youngest patient with optic nerve and chiasm astrocytoma in Thailand.
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PMID:Optic nerve glioma in infancy: a case report of the youngest patient in Thailand. 1152 26

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 cafiota 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 dendate 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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PMID:A case of neurofibromatosis 1 presenting with optic pathway glioma with an early onset and an aggressive course. 1829 34

The authors report a case of acute onset of comitant esotropia with diplopia in a 5-year-old boy with a diffuse pontine glioma. On first presentation, the angle of esodeviation was 30 prism diopters at distance fixation and 25 prism diopters at near fixation. Neurological symptoms appeared 10 weeks after the first visit.
J Pediatr Ophthalmol Strabismus 2012 Dec 04
PMID:Acute-onset esotropia: should we look inside? 2320 78

Congenital midline nasal masses are rare anomalies that occur in about 1 in 20,000 to 40,000 live births. Nasal gliomas are thought to be collections of heterotopic tissue of neurogenic origin, which have lost their intracranial connection. It rarely cause ocular distortion and deformity in the medial orbital wall. We describe here a case of a 12-month-old baby girl diagnosed as extranasal glioma at the lateral nasal wall and medial orbital wall presenting with strabismus and subsequently treated in our service and perform a literature review.
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PMID:Nasal glioma presenting with strabismus. 2448 10

We present a case of a 3-year-old girl with a positive family history of neurofibromatosis type-1 (NF1) presented with best corrected visual acuity of 20/40 in the right eye and <20/400 in the left eye. External ocular examination revealed left eye proptosis of 3 mm, grade II left relative afferent pupillary defect and full range of ocular motility with no strabismus. Slit lamp examination revealed iris lisch nodules bilaterally. Dilated fundus examination of the right eye was normal. Left eye disclosed a large mass extending from the optic nerve head, with associated subretinal fluid. There was neovascularization at the optic disc as well as a superior retinal hemorrhage. Computed tomography of brain/orbits showed an enlarged left optic nerve with a large mass at the optic nerve head, with no evidence of calcification. In addition, a large left optic pathway glioma (OPG), multiple hamartomas within the brain and a smaller low-grade right OPG was also reported. The remarkable feature of our case is the rare intraocular optic nerve involvement of the OPG. Early and regular ophthalmological assessment of all NF1 suspect/confirmed cases is of paramount importance in order to detect OPG early, resulting in timely intervention and salvage of vision.
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PMID:Rare case of optic pathway glioma with extensive intra-ocular involvement in a child with neurofibromatosis type 1. 2562 86