Bilateral amaurosis in 11 patients with giant cell arteritis confirmed by arterial biopsy]

BACKGROUND: Even in the times of corticosteroids giant cell arteritis may lead to complete bilateral blindness. Aim: To assess the frequency of complete irreversible blindness in giant cell arteritis. PATIENTS AND METHODS: Among all 218 patients with the diagnosis of giant cell arteritis confirmed by arterial biopsy between 1980 and 2000, clinical data of patients with bilateral amaurosis were further investigated. The main interest was focussed on the kind of ocular manifestation, the interval between first symptoms and therapy and the interval between involvement of the first and second eye. RESULTS: In 11 patients (9 women, 2 men, mean age: 79 years) giant cell arteritis led to complete bilateral blindness. Morphological ocular changes were anterior ischemic optic neuropathy (15 eyes), optic atrophy (4 eyes), posterior ischemic optic neuropathy (2 eyes), and central artery occlusion (1 eye). The median interval between involvement of the first eye and initiation of therapy was 4 days (1/2 day to 8 weeks). The median interval between visual loss in the first and second eye measured 4 days (simultaneously to 30 days). In 2 patients visual loss occurred 1 and 2 days after initiation of treatment (500 mg methylprednisolone/daily), respectively. Treatment with corticosteroids (100 - 1000 mg) did not result in visual improvement in any patient.CONCLUSIONS: Complete bilateral blindness occurred in 5 % of patients with giant cell arteritis, up to 2 days after initiation of treatment with corticosteroids. This number can only be further reduced by immediate therapy after clinical suspicion of giant cell arteritis.     

A new approach towards giant cell arteritis

Giant cell arteritis is a rare systemic vasculitis affecting large- and medium-sized arteries. Focal arteries lesions, include mononuclear cells infiltration of the vessel wall with giant cell formation. It is a disease of elderly persons and can result in a wide variety of systemic, neurological and ophthalmic complications, due to ischemia. The incidence of visual loss and ocular involvement varies between 14-88%, but one of the most common and severe complications is anterior ischemic optic neuropathy. The other ocular ischemic lesions include: central retinal artery occlusion, choroidal ischemia, diplopia, ocular motor paresis, anterior uveitis, cataract, ocular hypotony, corneal oedema and ulcerations, episcleritis and anterior scleritis, orbital cellulitis and pseudotumor. Because giant cell arteritis is potentially blinding disease, early diagnosis and immediate treatment with high dose corticosteroids may prevent further damage to the affected eye and prevent visual loss in the opposite eye. The purpose of this review is to revise established knowledge and to highlight the recent developments in diagnosis and management of giant cell arteritis.     

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.     

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.     

Giant cell arteritis--case report

Giant cell arteritis is a systemic disease of unknown origin. Vasculitis involves large and medium-sized vessels. Frequent clinical manifestations include characteristic headache in the temporal area, jaw or tongue claudication, apathy, fatigue, weight loss. The incidence of ocular involvement is reported in up to 70% patients. The most common and serious ophthalmic presentation is arteritic anterior ischemic optic neuropathy, which can lead to irreversible visual loss. Only early and aggressive steroid therapy may prevent this dangerous complication. The authors presented a case of a 68-years-old woman with giant cell arteritis. The main visual manifestation of this disease was anterior ischemic optic neuropathy.     

Reversible amantadine-induced corneal edema in an adolescent

PURPOSE: To document reversible corneal edema caused by amantadine in a pediatric patient. METHODS: A 14-year-old boy with a neurologic tremor was referred for bilateral visual loss. Our examination disclosed bilateral corneal edema without ocular inflammation. Pachymetry confirmed significantly increased corneal thickness above 900 microm. RESULTS: Review of the patient's medical information revealed recent institution of amantadine as a means to control the patient's tremor. On cessation of this agent, rapid resolution of corneal edema and recovery of visual acuity occurred. Repeat pachymetry measurement revealed normal corneal thickness. CONCLUSION: In cases of corneal edema and in the absence of any identifiable ocular causes, a review of toxic effects of systemic medication should be undertaken. Amantadine can cause corneal decompensation and needs to be considered as part of the differential diagnosis of corneal edema.     

Bilateral nonarteritic anterior ischemic neuropathy following acute angle-closure glaucoma in a patient with iridoschisis: case report

A 55-year-old woman was referred to our clinic because of a one-week history of visual loss and raised intraocular pressure in the left eye followed 4 days later by visual loss in the right eye. Slit-lamp examination showed bilateral conjunctival hyperemia, slight diffuse corneal edema, shallow anterior chamber and fixed and dilated pupil in both eyes. Splitting of the anterior layers of the iris with fibrillar degeneration extending for approximately one quadrant inferiorly was presented in each eye. Fundus examination showed optic disc edema with no vascular tortuosity and no cup in both eyes. The condition was treated as bilateral acute angle-closure glaucoma in a patient with irisdoschisis. After medical treatment and improvement of visual acuity, perimetry revealed a significant visual field defect especially in left eye; this case represents a rare concurrence of acute angle-closure glaucoma and bilateral nonarteritic ischemic optic neuropathy. Although most cases of elevated intraocular pressure, including acute angle-closure glaucoma, do not result in optic disc edema and irreversible vision loss, variations in the vascular supply of the nerve optic head along with others ocular systemic risk factors, may predispose certain individuals to nonarteritic ischemic optic neuropathy during periods of elevated intraocular pressure.     

Akuter Visusverlust mit beidseitigem Hornhautödem

An 84-year-old woman presented with bilateral visual loss that had appeared 3 days previously. Split lamp examination showed bilateral corneal edema with normal intraocular pressure. The patient complained of headache and vomiting, and finally collapsed. Elevated levels of inflammation markers led to the suspicion of an inflammatory disease. After investigation for internal or neurological diseases, a biopsy of the temporal artery was performed. Giant cell arteritis (Horton’s disease) was found, and steroid therapy was begun. The patient‘s general condition then improved.     

Giant cell arteritis mimicking idiopathic orbital inflammatory disease

PURPOSE: To report an unusual presentation of giant cell arteritis, referred from primary care, mimicking orbital apex syndrome. CASE REPORT: A 72 year old woman was referred with a two week history of pyrexia, dull right eye ache, 2mm of right proptosis, mild conjunctival chemosis and restriction of right eye movements. RESULTS: An erythrocyte sedimentation rate (ESR) was 90 and fluorescein angiography showed almost complete choroidal non-perfusion suggestive of giant cell arteritis. Temporal artery biopsy confirmed the diagnosis. CONCLUSIONS: Giant cell arteritis (GCA) typically presents with anterior ischemic optic neuropathy (AION), choroidal ischemia, central retinal artery occlusion, infrequently manifesting as an ocular motility problem, but has rarely been known to mimick idiopathic orbital inflammatory disease. Prompt recognition and therapy can minimize the chance of ipsilateral ocular involvement and protect the fellow eye.     

鼻咽癌长期放射治疗后双眼缺血综合征1例

目的：报道1例因接受放射治疗后出现双侧颈总动脉完全闭塞和双眼缺血综合征(ocular ischemic syndrome,OIS)的患者。 方法：病例报告。1例57岁男性患者主诉左眼一过性黑矇伴有眼部和眶周疼痛6mo,诊断为双侧OIS,依次给予双侧全视网膜光凝(panretinal photocoagulation,PRP)、白内障手术、颈动脉内膜剥脱术和Ahmed青光眼阀植入术。 结果：经过PRP和白内障超声乳化手术治疗,双侧眼部病情稳定。但患者行双侧颈动脉内膜剥脱术后出现眼压升高,视力降至指数。 结论：鼻咽癌放射治疗后出现的双侧颈总动脉完全闭塞和双眼OIS的治疗比较困难,预后不佳。     

Tempo(ral) was the heart of the matter

A 71-year-old woman was admitted with fever, headache, and weight loss associated with elevated inflammatory markers. She developed acute bilateral ophthalmoplegia and asymmetrical ptosis, rapidly followed by anterior ischemic optic neuropathy. Although the first temporal artery biopsy was negative, contralateral temporal artery biopsy revealed features consistent with giant cell arteritis. Even while under steroid therapy, she died a few days later from myocardial infarction. Acute bilateral complete ophthalmoplegia is a rare presentation of a limited number of possible diseases. Among these, giant cell arteritis should be suspected in the appropriate clinical scenario.     

Blind runner

Bilateral ocular ischemic syndrome and ischemic optic neuropathy have rarely been reported as initial manifestations of Takayasu arteritis (TA). Appearance of ocular symptoms in TA is related to the extent and severity of involvement of the aorta and its major branches. We report a case of bilateral ocular ischemic syndrome with unilateral ischemic optic neuropathy secondary to TA in a 42-year-old Pakistani man who had severe ocular and cerebral ischemia.     

Bilateral internuclear ophthalmoplegia—an unusual initial presenting sign of giant cell arteritis

A 63-year-old man presented six days after the sudden onset of horizontal double vision. His left eye became divergent two days later. On initial examination he had bilateral internuclear ophthalmoplegia with weakness of adduction and abducting nystagmus. Convergence was weak but there were no other neuro-ophthalmic signs. Constitutional signs included confusion and unsteadiness on his feet. A provisional diagnosis of arteritis was made. His ESR was 92 mm/h and a superficial temporal artery biopsy confirmed the diagnosis of giant cell arteritis. After two weeks or oral prednisolone his eye movements returned to normal. There have been no further relapses.
This would appear to be a unique presentation of giant cell arteritis. The causes of internuclear ophthalmoplegia are discussed along with a review of the ocular and neuro-ophthalmic signs of giant cell arteritis.     

Anterior ischemic optic neuropathy associated with giant cell arteritis. Case report

Giant cell arteritis is a systemic vasculitis that affects large- and medium sized arteries. The most common ophthalmic manifestation of this disease is anterior ischemic optic neuropathy, leading to acute, painless visual loss in one or both eyes. It is caused by ischemia of the optic nerve head, which is mainly supplied by the short posterior ciliary arteries. Early diagnosis is the key to correct management and prevention of visual loss in the second eye. The treatment of choice for arteritic ischemic optic neuropathy is high dose of systemic corticosteroids. Only such treatment may prevent blindness. The authors presented a case of a 62 years man with anterior ischemic neuropathy in one eye, which was diagnosed as arteritic form caused by giant cell arteritis. The correct diagnosis was based on typical clinical signs of ischemic changes in the optic nerve head and diagnostic criteria for giant cell arteritis, advocated by American College of Rheumatologists.     

Bilateral internuclear ophthalmoplegia--an unusual initial presenting sign of giant cell arteritis

A 63-year-old man presented six days after the sudden onset of horizontal double vision. His left eye became divergent two days later. On initial examination he had bilateral internuclear ophthalmoplegia with weakness of adduction and abducting nystagmus. Convergence was weak but there were no other neuro-ophthalmic signs. Constitutional signs included confusion and unsteadiness on his feet. A provisional diagnosis of arteritis was made. His ESR was 92 mm/h and a superficial temporal artery biopsy confirmed the diagnosis of giant cell arteritis. After two weeks or oral prednisolone his eye movements returned to normal. There have been no further relapses. This would appear to be a unique presentation of giant cell arteritis. The causes of internuclear ophthalmoplegia are discussed along with a review of the ocular and neuro-ophthalmic signs of giant cell arteritis.     

Delayed choroidal perfusion in giant cell arteritis.

The fundus fluorescein angiograms of 13 patients with visual disturbance due to biopsy-proven giant cell arteritis (11 with anterior ischemic optic neuropathy (AION); 2 with visual obscurations only) were compared with the fluorescein angiograms from 33 patients with acute nonarteritic AION and 23 age-matched normal eyes. In all 13 patients with giant cell arteritis, the fluorescein angiograms showed a significant delay of choroidal filling time (mean 69 seconds) in comparison with either normal subjects (mean 5.8 seconds) or patients with nonarteritic AION (mean 5.5 seconds). In patients presenting with acute AION, the finding of delayed choroidal filling on fluorescein angiography should raise the index of suspicion of giant cell arteritis and lead to prompt investigation and treatment.     

[In Press] The impact of combined oral contraceptives on ocular tissues: a review of ocular effects

This paper was aimed to review the action and adverse effects of Combined Oral Contraceptives on ocular tissues. Over 100 million women around the world use common contraceptive methods, including intrauterine devices, combined estrogen and progestin oral contraceptives (COCs), as well as progestin only preparations (oral contraceptives, implants or injections). COCs are widely used for contraception, but they are also indicated in menorrhagia, endometriosis, acne and hirsutism, fibroid uterus and premenstrual syndrome. However, they have been associated with high rates of cardiovascular events, venous thromboembolic disease, ischemic strokes and breast cancer. The incidence of COCs-related ocular complications was estimated to be 1 in 230,000, including dry eye symptoms, corneal edema, lens opacities and retinal neuro-ophthalmologic or vascular complications. We may infer that the serious ocular complications of COCs can be prevented by eliminating the estrogen dosage and choosing third-generation progestins. In any case, doctors should take into consideration the systemic and ocular history of the patients before selecting any method of contraception.     

Ocular manifestations of immunologic and rheumatologic inflammatory disorders

Our understanding of ocular manifestations in immunologic and rheumatologic inflammatory disorders has continued to expand over this past year. Studies that support the proposed immunologic pathogenesis of Vogt-Koyanagi-Harada disease are discussed. An article on the course of Beh?et's disease is reviewed as well as another article discussing severe visual loss in patients with a familial occurrence of Beh?et's disease. The prognostic factors for visual outcome in sarcoid uveitis are presented. An unusual case of giant cell arteritis and the most significant symptoms and diagnostic clues in helping make the diagnosis of giant cell arteritis are discussed.     

Marginal Corneal Ulceration (Limbal Guttering) as a Presenting Sign of Temporal Arteritis

A 68-year-old white woman was seen because of pain in each eye associated with the development of a marginal corneal ulceration (limbal guttering) bilaterally. Subsequently, the patient developed swelling over the bridge of the nose, painful necrotic lesions of the fingernail beds, scalp, on one knee, and conjunctival ulceration with scleral thinning. Medical examination revealed a markedly elevated erythrocyte sedimentation rate. A temporal artery biopsy specimen was positive for giant cell arteritis. The skin and ocular lesions resolved when the patient was given systemic corticosteroid therapy. The systemic and ocular manifestations of giant cell arteritis and the differential diagnosis and cause of limbal guttering are discussed.     

Herpes zoster vasculitis presenting as giant cell arteritis with bilateral internuclear ophthalmoplegia

PURPOSE: To present a case of herpes zoster vasculitis presenting as giant cell arteritis. DESIGN: Interventional case report. METHODS: A 77-year-old woman presented with sudden onset of diplopia associated with temple headaches and a previous history of herpes zoster ophthalmicus. A temporal artery biopsy was obtained and in-situ hybridization performed for herpes zoster DNA. RESULTS: The patient presented with a bilateral internuclear ophthalmoplegia. Initial diagnostic evaluation, including erythrocyte sedimentation rate, C-reactive protein, and temporal artery biopsy, was consistent with giant cell arteritis. However, in-situ hybridization of the temporal artery specimen was positive for herpes zoster DNA. CONCLUSIONS: Herpes zoster vasculitis may mimic giant cell arteritis and should be considered in the differential of any patient with presumed giant cell arteritis with suspicious findings, central nervous system involvement, or previous herpes zoster infection.