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1/17. myocardial infarction and coronary artery involvement in giant cell arteritis.

    PURPOSE: To describe the pathologic findings in an unusual case of giant cell arteritis that presented initially with visual loss and rapidly culminated in myocardial infarction. CASE REPORT: After the death of the patient, a complete autopsy was performed, including bilateral enucleation. All specimens, including a temporal artery biopsy completed before the patients death, were processed for routine paraffin histology and initially stained with hematoxylin and eosin. Elastic stains were subsequently used on specimens of temporal and coronary artery. The patient presented with loss of vision in the right eye. The clinical diagnosis was anterior ischemic optic neuropathy, secondary to temporal arteritis. The temporal artery biopsy was positive. Despite high-dose corticosteroid administration, the patient progressed to neurologic impairment, and subsequently to a fatal myocardial infarction. DISCUSSION: Previous reports of temporal arteritis with coronary involvement are summarized. myocardial infarction may be a more common early complication of temporal arteritis than appreciated previously. This important complication can occur despite administration of high-dose corticosteroid therapy.
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2/17. Choroidal infarction, anterior ischemic optic neuropathy, and central retinal artery occlusion from polyarteritis nodosa.

    PURPOSE: Ocular ischemia from polyarteritis nodosa (PAN) is rare. The authors present a case of multifocal ocular infarction from PAN. methods AND RESULTS: A 70-year-old woman developed hand and foot numbness followed by intermittent blurred vision and binocular horizontal diplopia. Two weeks later, she suddenly lost vision in the right eye from a central retinal artery occlusion and then developed a left anterior ischemic optic neuropathy and bilateral triangular choroidal abnormalities consistent with infarction. Her erythrocyte sedimentation rate and c-reactive protein were elevated. Although giant cell arteritis was suspected, a multiple mononeuropathy was demonstrated by electromyogram and nerve conduction velocity studies. biopsy specimens from her sural nerve and biceps muscle showed a necrotizing vasculitis with fibrinoid necrosis, consistent with PAN. CONCLUSIONS: polyarteritis nodosa can produce ischemia of a variety of ocular structures, including the retina, choroid, and optic nerve. In our patient, all three structures were affected. To our knowledge, this is the first reported case of the triangular sign of Amalric in PAN.
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3/17. Optic disc structure and shock-induced anterior ischemic optic neuropathy.

    PURPOSE: To describe a patient who developed unilateral shock-induced anterior ischemic optic neuropathy (SIAION) after gastrointestinal hemorrhage followed by presumed idiopathic nonarteritic anterior ischemic optic neuropathy (NAION) in the fellow eye. DESIGN: Retrospective, observational case report and literature review. methods: The case history of an 80-year-old man who developed SIAION, followed by NAION in the fellow eye, was reviewed. All previously reported cases of SIAION were reviewed. MAIN OUTCOME MEASURES: Neuro-ophthalmic examination, including visual acuity, funduscopy, and automated perimetry. RESULTS: An 80-year-old man, with a history of gastrointestinal bleeding from a duodenal ulcer, was hospitalized and received four units of packed red blood cells after he was found to be severely anemic (hemoglobin 6.7 g/dl). Three days later he complained of loss of vision of the right eye. Neuro-ophthalmic examination 2 weeks later disclosed a visual acuity of counting fingers at 6 inches in the right eye and 20/40 in the left eye, with a right relative afferent pupillary defect and a superior altitudinal visual field defect. Funduscopy revealed optic disc edema with a temporal parapapillary hemorrhage in the right eye and a small optic disc, with no cup, in the left eye. A diagnosis of SIAION secondary to anemia was made. Six weeks later he developed a new inferior altitudinal visual field defect in the left eye and diffuse optic disc swelling. He had no signs or symptoms of giant cell arteritis or polymyalgia rheumatica, his hemoglobin at this time was 11.9 g/dl, and the Westergren erythrocyte sedimentation rate was 6 mm/hour. CONCLUSIONS: Our patient developed optic disc swelling of the right eye after an episode of gastrointestinal hemorrhage (SIAION). The disc swelling in the left eye occurred 8 weeks later, when his hemoglobin had increased to 11.9 g/dl. The timing of the ischemic optic neuropathies suggests that the acute anemia led to involvement of the first but not the second eye. The configuration of the optic disc may have predisposed not only to the second event (NAION) but also to the first episode (SIAION).
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4/17. Bilateral optic nerve sheath enhancement from giant cell arteritis.

    An 83-year-old man presented with acute bilateral visual loss to no light perception (NLP) OD and 20/50 OS. His fundus examination showed moderate bilateral pallid disc edema. A sedimentation rate was 60 mm/h. magnetic resonance imaging of the brain and orbits with gadolinium revealed marked bilateral enhancement of the optic nerve sheaths and adjacent orbital fat. He underwent biopsies of the optic sheath OD and bilateral temporal arteries. Histopathology of the optic nerve sheath area revealed fibroadipose tissue containing numerous arteries with intimal thickening, and mild mural inflammation consisting predominantly of lymphocyte with occasional giant cells. The bilateral temporal artery biopsies revealed focal disruption of the elastic lamina with rare giant cells. His vision had since stabilized on IV methypdnisolone therapy. The biopsies of the nerve sheath suggest that the radiologic finding of optic nerve sheath enhancement in giant cell arteritis is caused by tbe same pathophysiology, and therefore may be a manifestation of this systemic disease.
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5/17. Late ipsilateral recurrence of ischemic optic neuropathy in giant cell arteritis.

    A patient with arteriosclerosis, diabetes mellitus, and giant cell arteritis (GCA) treated continuously with low-dose prednisone developed anterior ischemic optic neuropathy (AION) at 5 and 13 months after clinical diagnosis of GCA. At the time of late recurrent AION, there were no systemic symptoms or elevations in acute phase reactants to signal active arteritis, yet temporal artery biopsy disclosed dramatic inflammation, forcing the presumption that the infarct was arteritic. Recurrent systemic symptoms and elevation of acute phase reactants are not reliable warning signs of reactivated GCA. In patients at high risk for corticosteroid complications, late biopsy may be a reasonable guide to corticosteroid weaning.
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6/17. Progressive visual loss in a patient with presumed temporal arteritis despite treatment: how to make the diagnosis.

    Giant cell (temporal) arteritis is a severe potentially fatal systemic vasculitis characterized by focal involvement of the cranial arteries resulting in ischaemic arterial occlusion. The case is presented of a 75-year-old woman with presumed giant cell arteritis and normal bilateral temporal artery biopsies. Despite a seemingly adequate course of systemic steroid therapy, the patient developed sudden catastrophic vision loss. cerebral angiography and ultrasonography were useful investigations to determine the most appropriate artery to biopsy to confirm the diagnosis of giant cell arteritis.
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7/17. Hypovolemic ischemic optic neuropathy.

    BACKGROUND: Ischemic optic neuropathy refers to an acute event of ischemia, or decreased blood flow, to the optic nerve resulting in varying degrees of vision loss and visual field defects. Typically this disease affects the elderly population who experience systemic diseases that compromise the blood flow efficiency of the optic nerve head (e.g., giant-cell arteritis, hypertension, diabetes, etc.). However, cases of blood loss to the optic nerve, secondary to traumatic injuries or surgeries, have also been shown to result in ischemic optic neuropathy, regardless of age. It seems that in these cases, the resulting anemia and hypotension play contributing roles in the development of ischemic optic neuropathy. methods: A 41-year-old black man came to us with optic nerve head pallor O.S., count-fingers vision O.S., positive afferent pupillary defect O.S., and a central scotoma O.S. after being hospitalized and treated for a stab wound to his left neck that severed his left carotid artery at the bifurcation. RESULTS: This patient had been seen in the optometry Clinic two years before the stab-wound incident. At that time, he had 20/20 vision in his left eye and no remarkable neurological deficits. His ocular presentation after the traumatic hypovolemic event was probably a direct result of the hypoperfusion to the left optic nerve head. This patient was diagnosed with a hypovolemic, or blood loss-related, ischemic optic neuropathy (O.S.). CONCLUSIONS: patients who experience large amounts of blood loss due to trauma, surgery, internal bleeding, etc. and report vision loss should be screened for possible optic nerve ischemia. As eye care providers, when we are presented with patients who have optic nerve head atrophy, we should inquire about events that may have precipitated blood loss, potentially triggering ischemic optic neuropathy.
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8/17. A case of biopsy-negative temporal arteritis--diagnostic challenges.

    A patient with systemic symptoms but no visual loss was investigated for suspected giant cell arteritis. Initial temporal artery biopsy was reported as negative; however, she returned with visual loss 2 months later, and the diagnosis of giant cell arteritis was confirmed with a subsequent biopsy. In hindsight, signs suggestive of the disease were present in the original biopsy, although the usual diagnostic features were absent.
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9/17. An occult case of giant cell arteritis presenting with combined anterior ischemic optic neuropathy and cilioretinal artery occlusion.

    A 61-year-old female presented with a moderate decrease in vision in the left eye. The patient denied any other ocular or systemic symptoms related to giant cell arteritis. visual acuity was 20/50 in the left eye with a 2 relative afferent pupillary defect and markedly abnormal color vision. Dilated fundus examination and flourescein angiography revealed optic disc edema as well as a cilioretinal artery occlusion. Erythrocyte sedimentation rate was only slightly elevated. Subsequent biopsy of the superficial temporal artery confirmed the diagnosis of giant cell arteritis. Cilioretinal arteries are anatomical variants derived from the short posterior ciliary arteries. Arteritic anterior ischemic optic neuropathy typically results from thrombotic occlusion of the short posterior ciliary arteries. Consequently, arteritic occlusion of the short posterior ciliary arteries can result in concomitant occlusion of the cilioretinal artery. This case highlights the situation where clinical symptoms were not suspicious for giant cell arteritis but the presence of an anterior ischemic optic neuropathy and a cilioretinal artery occlusion was virtually pathognomonic for giant cell arteritis.
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10/17. Ocular ichemia syndrome - a malignant course of giant cell arteritis.

    PURPOSE: To call attention to a malignant course of ocular ischemic syndrome in patients with giant cell arteritis (GCA). methods/PATIENT: A 84-year-old woman developed severe headache for about 3 (1/2) months prior to myocardial infarction and visual disturbances. RESULTS: An anterior ischemic optic neuropathy (AION) in the right eye with a distinct reduction in visual acuity was found. The retina revealed several cotton-wool spots in both eyes. Serologic examinations showed inflammatory signs. Despite treatment with prednisolone, eye pressure decreased to 2 mm Hg in the right eye and 4 mm Hg in the left eye in a few days. An ischemic iritis developed in the right eye. visual acuity worsened to detection of hand motions in the right eye and to 0.1 in the left eye. Approximately 8 (1/2) months after her initial headache, a biopsy was carried out. The patient was treated continuously with corticosteroids. histology of the superficial temporal artery indicated inflammatory cells in the vessel wall. - The patients daughter developed symptoms of GCA at the age of 54 years. CONCLUSION: An ocular ischemic syndrome points to a malignant course of the disease. A cardiac infarction can develop in GCA. A biopsy of the temporal artery can reveal inflammatory changes even after 8 (1/2) months.
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