Cases reported "Optic Atrophy"

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1/36. Unilateral glaucomatous damage in a patient with hemifacial spasm.

    We report a patient with hemifacial spasm of the right hemiface and ipsilateral glaucomatous optic neuropathy. No risk factors were found to explain the unilateral glaucoma. Ocular pressure readings were always within normal range.
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2/36. Optic glioma with characteristic bilateral optic atrophy in a 3-year-old girl.

    We report a case of optic glioma with bilateral optic atrophy. A 3-year-old girl presented with vomiting and left hemiparesis. She had hypothalamic dysfunction, right ptosis, right monocular nystagmus, left facial palsy, left hemiparesis, and left pes adductus. neuroimaging studies showed obstructive hydrocephalus with a large suprasellar calcified tumor with a ring-like enhancement mimicking craniopharyngioma. Visual-evoked potentials showed delayed latency of N75 in the right occipital lead. The tumor, a pilocytic astrocytoma in the right optic tract and chiasma, was partially removed via a right frontotemporal craniotomy. The right optic nerve had shrunk to half the normal diameter and became twisted downwardly. intracranial pressure (ICP) increased to 40 cm H2O. The fundus had bilateral optic atrophy without disc swelling. To our knowledge, this is the first report of a lamina/dot sign of the optic disc in a small child with a brain tumor and a normal neuroretinal fiber layer. These ocular findings may result from possible interruption of the axonal flow caused by the tumor and not increased ICP.
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3/36. Bilateral optic nerve atrophy in myotonic dystrophy.

    PURPOSE: To document a case of bilateral optic atrophy in a patient with myotonic dystrophy. myotonic dystrophy is an autosomal dominant disorder, genetically resulting from an expansion of an unstable CTG repeat in the 3'-untranslated region of a protein kinase gene (DMPK) on chromosome 19q13.3. methods: Case report, clinical examination, fundus photographs, visual fields, visual evoked potentials, electroretinograms, and genetic studies of a 56-year-old woman clinically diagnosed with myotonic dystrophy. RESULTS: The patient experienced decreased vision consisting of light perception with the right eye and 20/25 with the left. Fundus examination showed bilateral pallor of the optic disks. intraocular pressure was normal. Visual field testing, visual evoked potentials, and electroretinogram were abnormal. A pathologic CTG expansion in the myotonic dystrophy gene was found. CONCLUSIONS: In a patient with myotonic dystrophy, confirmed with genetic molecular diagnosis, bilateral optic atrophy was present. optic atrophy should be considered a possible complication of myotonic dystrophy.
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4/36. Optic neuropathy in children with lyme disease.

    Involvement of the optic nerve, either because of inflammation or increased intracranial pressure, is a rare manifestation of lyme disease. Of the 4 children reported here with optic nerve abnormalities, 2 had decreased vision months after disease onset attributable to optic neuritis, and 1 had headache and diplopia early in the infection because of increased intracranial pressure associated with Lyme meningitis. In these 3 children, optic nerve involvement responded well to intravenous ceftriaxone therapy. The fourth child had headache and visual loss attributable to increased intracranial pressure and perhaps also to optic neuritis. Despite treatment with ceftriaxone and steroids, he had persistent increased intracranial pressure leading to permanent bilateral blindness. Clinicians should be aware that neuro-ophthalmologic involvement of lyme disease may have significant consequences. If increased intracranial pressure persists despite antibiotic therapy, measures must be taken quickly to reduce the pressure.
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5/36. Spontaneous regression of retinal astrocytic hamartoma in a patient with tuberous sclerosis.

    PURPOSE: To report the complete regression of a retinal astrocytic hamartoma in conjunction with tuberous sclerosis. DESIGN: Observational case report. methods: A 12-year-old boy with tuberous sclerosis, diagnosed early in life, was found to have a retinal astrocytic hamartoma in his left eye. He had bilateral optic atrophy because of long-standing increased intracranial pressure due to a cerebral giant cell astrocytoma. He was followed on a yearly basis with fundus photography and fluorescein angiography. RESULTS: At the time of initial presentation, the minimally elevated intraretinal tumor measured 2 x 1.5 mm. A year later, the same tumor measured 1.5 x 1 mm. Two years after initial presentation, the tumor totally disappeared. CONCLUSION: This patient has demonstrated that retinal astrocytic hamartoma may undergo spontaneous regression.
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6/36. Severe ocular and orbital toxicity after intracarotid injection of carboplatin for recurrent glioblastomas.

    BACKGROUND. glioblastoma is a malignant tumor that occurs in the cerebrum during adulthood. With current treatment regimens including combined surgery, radiation and chemotherapy, the average life expectancy of the patients is limited to approximately 1 year. Therefore, patients with glioblastoma sometimes have intracarotid injection of carcinostatics added to the treatment regimen. Generally, carboplatin is said to have milder side effects than cisplatin, whose ocular and orbital toxicity are well known. However, we experienced a case of severe ocular and orbital toxicity after intracarotid injection of carboplatin, which is infrequently reported. CASE. A 58-year-old man received an intracarotid injection of carboplatin for recurrent glioblastomas in his left temporal lobe. He complained of pain and visual disturbance in the ipsilateral eye 30 h after the injection. Various ocular symptoms and findings caused by carboplatin toxicity were seen. RESULTS. He was treated with intravenous administration of corticosteroids and glycerin for 6 days after the injection. Although the intraocular pressure elevation caused by secondary acute angle-closure glaucoma decreased and ocular pain diminished, inexorable papilledema and exudative retinal detachment continued for 3 weeks. Finally, 6 weeks later, diffuse chorioretinal atrophy with optic atrophy occurred and the vision in his left eye was lost. CONCLUSION. When performing intracarotid injection of carboplatin, we must be aware of its potentially blinding ocular toxicity. It is recommended that further studies and investigations are undertaken in the effort to minimize such severe side effects.
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7/36. intraocular pressure-induced interlamellar keratitis after LASIK surgery.

    PURPOSE: To describe a case of interlamellar stromal keratitis induced by increased intraocular pressure (IOP) after LASIK surgery. methods: Case report and review of the literature. RESULTS: A 53-year-old white man with a history of treated ocular hypertension underwent uncomplicated LASIK surgery. The postoperative course was complicated by markedly elevated IOP induced by topical corticosteroid drops used to treat what appeared to be diffuse lamellar keratitis. Because IOPs remained uncontrolled despite maximal therapy, topical steroids were discontinued after a total of 9 weeks. The IOP rapidly returned to normal range with complete resolution of the corneal findings. Humphrey visual field analysis, confocal scanning laser imaging of the optic nerve, and stereoscopic disc photographs all demonstrated that significant glaucomatous field loss and optic atrophy developed over this 8-week period. DISCUSSION: The IOP should be immediately evaluated in patients who present with interlamellar stromal keratitis more than 1 week after LASIK. If the IOP is elevated, corticosteroid drops should be discontinued to prevent permanent visual loss. Furthermore, if a glaucoma specialist examines a patient with a history of LASIK and unexplained visual field loss, the medical record should be reviewed to determine if the postoperative course was complicated by this diffuse lamellar keratitis-like phenomenon.
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8/36. Funduscopic appearance of papilledema with optic tract atrophy.

    In a child with a hypothalamic glioma, elevated intracranial pressure and retrograde optic tract atrophy produced distinctive patterns of papilledema and disc pallor in each eye. On the side of the tract lesion, the disc was swollen nasally and was pale temporally. In the contralateral fundus, the swollen optic disc was bisected horizontally by a pale atrophic band. These funduscopic signs exemplify a rare instance in neuro-ophthalmologic diagnosis wherein specific lateralizing and localizing information can be deduced solely from the appearance of papilledema.
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9/36. Retinal nerve fiber layer thickness in optic tract syndrome.

    BACKGROUND: Optic tract syndrome (OTS) is characterized by incongruous homonymous hemianopia and a perpendicular pattern of bilateral optic atrophy due to the optic tract lesion. However, loss of retinal nerve fiber layer thickness (RNFLT) associated with OTS has not been quantitatively assessed. CASE: A 20-year-old woman with blunt head trauma showed normal visual acuity, color vision, ocular motility, and intraocular pressure. Because of a relative afferent pupillary defect in her left eye and left-sided homonymous hemianopia, we suspected right-sided optic tract damage, although magnetic resonance imaging detected no intracranial lesion. OBSERVATIONS: Using optical coherence tomography (OCT), the RNFLT of this case was measured at 31 months after the trauma and compared with age-matched normal controls (n = 41). Nasal, temporal, superior, and inferior quadrant RNFLT was reduced by 22%, 21%, 5%, and 46% in the right eye and 76%, 64%, 25%, and 27% in the left eye, respectively. The reduction was > 3 x the standard deviation of the normal mean values in the nasal and temporal quadrants of the left eye and in the inferior quadrant of the right eye. CONCLUSIONS: OCT can determine the RNFLT reduction corresponding to the characteristic patterns of optic atrophy of OTS.
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10/36. Atypical external hydrocephalus with visual failure due to occult leptomeningeal dissemination of a pontine glioma. Case report.

    The authors report on the case of a diffuse pontine glioma in a 5-year-old boy in whom radiologically and cytologically occult leptomeningeal metastases led to the development of an atypical "external" hydrocephalus, associated with grossly elevated intracranial pressure (ICP). Initial neuroimaging demonstrated only mild communicating ventricular dilation associated with a noticeable enlargement of the subarachnoid space, particularly over the surface of the frontal lobes; these features are not usually associated with significantly elevated ICP. Possible pathophysiological mechanisms resulting in this unusual clinical presentation are discussed. Early recognition of the severity of the raised ICP despite the paucity of clinical and radiological signs may have averted the development of blindness due to optic atrophy.
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