Cases reported "Optic Nerve Diseases"

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1/42. Bilateral blindness and lumbosacral myelopathy associated with high-dose carmustine and cisplatin therapy.

    PURPOSE: To report the early ocular pathologic findings associated with high-dose carmustine and cisplatin therapy. methods: A patient with metastatic breast carcinoma developed an acute onset of branch retinal artery occlusion, bilateral blindness, and a myelopathy involving the lower extremities after high-dose chemotherapy and bone marrow transplant. RESULTS: Histopathologic examination of the eye and optic nerves at autopsy disclosed nerve fiber layer infarction secondary to right inferior temporal retinal artery thrombosis. Patchy necrosis of both optic nerves, medulla oblongata, and spinal cord was associated with focal small-vessel thrombosis. CONCLUSIONS: The syndrome of retinal vascular occlusion, optic neuropathy, and myelopathy is associated with the high-dose chemotherapeutic agents carmustine and cisplatin. The distribution of necrosis suggests an ischemic event rather than direct neurotoxic effects.
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2/42. optic disk pallor and retinal atrophy in sotos syndrome (cerebral gigantism).

    PURPOSE: To report a case of sotos syndrome (cerebral gigantism) with megalophthalmos, optic disk pallor, and retinal atrophy. methods: Case report. RESULTS: A 22-year-old man was diagnosed with sotos syndrome with optic atrophy. His corneal diameters were 13 mm horizontally, and his optic disks were pale. His retinal vessels were attenuate in diameter, and small white spots were found on the retina. The results of a Goldmann visual field test were normal. His visually evoked potential to flash stimuli showed extended latent times and normal amplitudes. Keratometry was 40.5 diopters (8.33 mm) in both eyes. The axial length was 25.9 mm in the right eye and 25.4 mm in the left eye. CONCLUSION: sotos syndrome may be associated with optic disk pallor and retinal atrophy.
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3/42. Ophthalmic manifestations of neonatal onset multisystem inflammatory disease.

    PURPOSE: To report the ophthalmic manifestations of neonatal onset multisystem inflammatory disease, a recently recognized, rare systemic disorder characterized by the triad of arthropathy, rash, and abnormal central nervous system development. METHOD: Case report. RESULTS: A 2-year-old female with neonatal onset multisystem inflammatory disease presented with visual acuity of fix and follow with each eye, bilateral optic nerve head pallor and gliosis, as well as marked sheathing of the peripapillary vessels. No vitreous inflammation or macular edema was found. visual acuity was stable from the neonatal period through the 3 months of follow-up after the changes involving the optic nerve heads and peripapillary vessels were observed (a total of 33 months). CONCLUSIONS: This report provides a description of the ocular manifestations of neonatal onset multisystem inflammatory disease and supports earlier suggestions that swelling of the optic nerve heads can occur. In this case, optic nerve head pallor may have been a sequela of such swelling. The pathogenesis of neonatal onset multisystem inflammatory disease is unknown.
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4/42. Neuro-ophthalmological manifestations of systemic lupus erythematosus in Asian patients.

    PURPOSE: To report eight patients with diagnosed systemic lupus erythematosus (SLE) who presented with a variety of neuro-ophthalmological complications and outcomes. methods: A review of the follow up, subsequent management and outcomes of the neuro-ophthalmological complications of eight patients with SLE. RESULTS: The patients presented with a myriad of symptoms of varying severities. The most common manifestations were that of optic neuropathy and eye movement abnormalities.The outcome was variable ranging from complete recovery to optic atrophy with navigational visual acuity. Treatment was often empirical, although early treatment with corticosteroids has been tried with varying success, CONCLUSIONS: The pathophysiology in the neuro-ophthalmological manifestations of SLE is thought be due to vaso-occlusive disease of the small vessels of the nerve and brain, secondary to an autoimmune process. Ophthalmologists may need to consider the diagnosis of SLE in young women who present with a recent onset of neuro-ophthalmological symptoms and signs.
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5/42. Splitting and penetration of the optic nerve by an aneurysm arising from the anterior wall of internal carotid artery: case report.

    Aneurysms arising from the anterior wall of the internal carotid artery (ICA) are uncommon. There have not been any reports demonstrating the anatomical relation of such aneurysms to the optic nerve. An aneurysm arising from the anterior wall of the ICA splitting and penetrating the optic nerve is reported. A 73 year old woman presented with severe headache due to subarachnoid haemorrhage. She had never experienced a visual disturbance. At surgery, the aneurysm was shown to arise from the anterior wall of the left internal carotid artery and to split and penetrate the left optic nerve. The aneurysm was not related to arterial bifurcation of any branches and was safely neck clipped. Given the evidence of a split and penetration of the optic nerve, the pathogenesis of such an aneurysm may be due to the persistence of an embryonic vessel.
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6/42. Clinicopathologic case reports: optic nerve head metastasis.

    A case of tumor metastasis from carcinoma of the lung directly to the cribriform plate is described. Retina and optic nerve were involved, but there was no evidence of tumor invasion of the choroid. It is postulated that the original tumor embolus lodged in a cribriform branch of the arterial circle of Zinn or in the central retinal vessels.
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7/42. optic nerve arteriovenous malformation causing optic apoplexy: case report.

    OBJECTIVE AND IMPORTANCE: vascular malformations in the optic pathway are rare. Only one case of pathologically confirmed arteriovenous malformation (AVM) of the optic nerve has been reported previously. We document the case of a patient with an optic nerve AVM who presented with optic apoplexy that was diagnosed with the use of magnetic resonance imaging. CLINICAL PRESENTATION: A 15-year-old girl developed left visual disturbance of sudden onset while playing badminton. A magnetic resonance imaging scan disclosed left optic nerve swelling and intraoptical hemorrhage, although an angiogram did not reveal abnormal vessels. INTERVENTION: The patient underwent total removal of the hematoma and tangles of the abnormal vessels in the left optic nerve, which was diagnosed pathologically as an AVM. The patient recovered visual acuity, but the left visual field defect remained unchanged. CONCLUSION: Along with cavernous malformations and optic gliomas, AVMs can be a rare cause of optic nerve apoplexy. T2-weighted magnetic resonance imaging is useful in rendering the diagnosis of an optic nerve AVM, observed as a mass lesion consisting of serpiginous, tangled, low-intensity bands. Early surgical treatment is recommended to obtain a rapid recovery.
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8/42. Junctional scotoma in giant cerebral aneurysm.

    A brain lesion located at the lateral side of the sella turcica can produce a junctional scotoma by compressing the ipsilateral optic nerve and the contralateral inferonasal nerve fiber. This study reports a female patient with a junctional scotoma caused by a cerebral aneurysm. At the initial visit, she complained of visual disturbance in both eyes and the right optic disc was atrophied. The visual field showed right blindness and left superotemporal quadrantopsia. A brain CT indicated an approximately 3 cm sized brain mass located superolateral to the sella turcica. The brain MRI showed the lesion to be more like an aneurysm than a pituitary adenoma. Therefore, 4 vessels angiography was done, and this lesion was confirmed to be a sellar variant of an aneurysm located at the right carotid siphon. Like a tumor of the optic chiasm, a cerebral aneurysm can cause visual disturbance and visual field defects. Therefore, an early differential diagnosis is important because the prognosis and treatment of an aneurysm differ.
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9/42. Progressive visual loss due to a muslinoma--report of a case and review of the literature.

    Intracranial internal carotid artery (ICA) aneurysms are frequently treated either by microsurgical clipping of the aneurysm neck, by endovascular coiling of the aneurysm sac or by balloon occlusion of the parent vessel. For some broad-based aneurysms that may not be suitable for any of these options, microsurgical wrapping of the aneurysm wall with muslin or gauze rarely is applied. We report the case of a patient who suffered from a minor stroke because of arterio-arterial embolism from an intracranial ICA aneurysm. The aneurysm was treated by wrapping muslin material. After 12 months, he experienced progressive visual loss. Cranial magnetic resonance testing magnetic resonance tomography (MRT) revealed a granulomatous inflammation surrounding the site of the aneurysm and affecting optochiasmatic structures. A muslin-induced optic neuropathy is a rare but serious complication of a chronic inflammatory reaction in response to muslin or gauze used in intracranial aneurysm wrapping. If the foreign body inflammatory reaction to muslin or gauze leads to a mass formation, the term muslinoma or gauzoma is applied. Various treatment strategies include surgery, steroids and cyclophosphamide, but recovery of the vision is not predictable. As a consequence, muslin or cotton gauze should only be applied with great caution in neurovascular surgery.
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10/42. optic nerve and spinal cord manifestations of malignant atrophic papulosis (Degos disease).

    A fatal case of malignant atrophic papulosis (Degos disease) with optic nerve and spinal cord involvement is described. magnetic resonance imaging (MRI) of the optic nerve showed abnormal signal enhancement on fat suppressed T1 weighted images after intravenous meglumine gadopentetate infusion. On T2 weighted sagittal images, a sawtooth pattern was observed over seven vertebral segments of the spinal cord. On necropsy, a severe loss of myelinated nerve fibres in the left optic nerve was seen, with thrombotic obstruction of the central retinal artery. Spongy degeneration was observed in all levels of the spinal cord, with patchy and motheaten patterns caused by thromboses and endothelial proliferation in subarachnoid vessels. Findings on MRI were consistent with findings on pathological examination.
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