Cases reported "Optic Atrophy"

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1/20. Cerebral arteriovenous malformation presenting as visual deterioration in a child.

    A rare case of visual loss as the presenting feature of a central arteriovenous malformation involving the vein of Galen is reported. A 5-year-old girl with a history of deteriorating vision for the past 6 months was examined. Ocular examination showed a left hemianopia, left optic atrophy, and dilated vessels of the right optic disc. MRI revealed a massive deep-seated central arteriovenous malformation involving the vein of Galen. The mechanism of visual loss is likely to be a combination of ischaemic optic atrophy associated with a steal phenomenon and direct compression of the right optic radiation.
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2/20. Evolution of descending optic atrophy. A case report.

    Fundus changes following severe trauma to the intracranial optic nerve were followed by means of serial fundus photography. The eye was completely blind. Little change was seen during the first 4 weeks. The retinal nerve fibre layer disappeared gradually during weeks 4 to 8. At the same time the retinal vessels turned narrow, and vascular pseudo-sheathing appeared close to the optic disc.Disc pallor was not maximal until the 12th week, when the peripapillary retina also had acquired a mottled appearance.
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3/20. Atypical MELAS associated with mitochondrial tRNA(Lys) gene A8296G mutation.

    We report on a unique patient with mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) presenting optic atrophy, cardiomyopathy, and bilateral striatal necrosis before stoke-like episodes became apparent. Skeletal muscle total mitochondrial dna analysis identified a heteroplasmic A to G point mutation in the tRNA(Lys) gene at position 8296. Skeletal muscle pathology revealed typical MELAS findings, including ragged-red fibers cytochrome c oxidase positive strongly succinate dehydrogenase-reactive blood vessels. Recent reports describe the 8296 mutation identified in patients with diabetes mellitus or myoclonus epilepsy with ragged-red fibers, not MELAS. We conclude that the 8296 mutation is likely to be pathogenic and that it may be not only a mutation responsible for diabetes mellitus or myoclonus epilepsy with ragged-red fibers but also for MELAS.
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4/20. Scleroderma, stroke, optic neuropathy: a rare association.

    A known case of scleroderma presented with right hemiparesis, focal seizures, optic atrophy and gangrene of digits. There was no evidence of peripheral nerve or muscle involvement. MRI showed multifocal infarcts in both cerebral hemispheres. MR angiography revealed poor flow in bilateral carotid arteries with collateralization from posterior circulation. She improved with phenytoin, nifedipine, antibiotics and immunosuppressants. The rarity of central nervous system affliction in scleroderma and large vessel vasculitis is discussed along with review of literature.
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5/20. epiretinal membrane formation is a late manifestation of shaken baby syndrome.

    shaken baby syndrome is a constellation of injuries resulting from the intentional shaking type movement of a child who is usually younger than 3-years-old. This rapid acceleration-deceleration movement of the head is responsible for lesions attributed to shearing forces placed on the vitreoretinal structures and meningeal vessels across the dura. The ophthalmic findings include intraocular hemorrhages, perimacular retinal folds, and peripheral retinoschisis in the presence of intracranial injuries such as subdural hematomas without obvious external signs of head trauma. We describe a case of late development of an epiretinal membrane in a child with a history of shaken baby syndrome and propose a differential diagnosis list for epiretinal membrane formation in the pediatric age group.
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6/20. Foster Kennedy syndrome and optociliary shunt vessels in a patient with an olfactory groove meningioma.

    A 48-year-old woman complained of acute loss of vision in her right eye. Ophthalmoscopically, the right optic disk appeared pale, and abnormally dilated vessels were noted on the disk. The left optic disk was reddish and swollen. fluorescein angiography revealed abnormal vessels on the right optic disk that might be venous. The patient had right-sided anosmia. Computed tomography and magnetic resonance imaging showed a lesion in the frontal lobe that was deviated to the right and attached to the olfactory groove. A histopathologic study of the excised specimen disclosed a meningioma. We believe that this patient with olfactory groove meningioma represents a rare case of Foster Kennedy syndrome and optociliary shunt vessels.
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7/20. A Chinese family with wolfram syndrome presenting with rapidly progressing diabetic retinopathy and renal failure.

    We describe a Chinese family with three siblings, all females, presenting with the wolfram syndrome. All three cases had almost similar clinical presentation of insulin-dependent diabetes mellitus, with rapid development of severe renal and retinal complications. Two siblings died at age thirty and thirty-one years of end-stage renal failure. All three cases had visual symptoms since early childhood progressing rapidly to loss of vision. Two of the three siblings had severe diabetic retinopathy requiring laser photocoagulation. These presentations are in contrast to most reported cases of the wolfram syndrome where advanced diabetic eye complication is a rare feature. We also present several features present in one of the siblings, viz., microcephaly, microstomia, clinodactylyl, brachydactylyl, empty sella syndrome and severe hypoplasia of the right internal carotid vessels associated with mild narrowing of the left internal carotid artery which have not been previously described.
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8/20. Ocular polyarteritis nodosa. Report of a case.

    A patient with bilateral amaurosis as a complication to polyarteritis nodosa is presented. He developed affection of the central retinal arteries and the arteries supplying the optic discs followed by retinal and optic atrophy. After one month no vessels could be observed neither in the retinae nor at the optic discs. The importance of early diagnosis and aggressive immunosuppressive treatment is stressed.
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9/20. Acute ischemic atrophy of papilla disc and diabetes.

    Acute ischemic neuropathy is the acute ischemic necrosis of the optic nerve fibers occurred by the occlusion of small vessels which distribute widely to lamina cribrosa and anterior et posterior area of lamina cribrosa. Pale coloured edema and light swelling are seen generally on the optic disc, and after a few months falls in simplex nerve atrophy. I have explained my 3 cases of ischemic neuropathies. They have some things in common, and causes are considered arteriosclerosis or temporal arteritis in old people, and often takes place based on diabetes mellitus, lupus erythematosis or another many diseases etiologically.
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10/20. optic atrophy in acute intermittent porphyria.

    A 24-year-old woman developed bilateral blindness after recovery from coma secondary to acute intermittent porphyria. Gradual return of vision in the right eye with a permanent unilateral visual field defect and optic atrophy followed. We believe the pathophysiologic mechanism was spasm of the vessels supplying the optic disk leading to ischemia and infarction of the optic nerve.
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