【摘要】 一位67岁男性患者出现右边颞侧头痛和右眼视力突发性无痛性丧失，出现这些症状时还伴有全部眼肌麻痹和视盘水肿。ESR和C反应蛋白升高。核磁共振检查证实有视神经鞘膜炎的特征。右颞侧动脉的活组织切片检查正常。眼外肌运动随着全身激素的应用而好转。不幸的是此后患者右眼发展为中央视网膜动脉阻塞。全眼肌麻痹是巨细胞动脉炎的一种罕见表现，为了得到满意的治疗结果并且阻止对侧眼盲，疾病开始时使用皮质类固醇激素是必要的。
【关键词】 巨细胞动脉炎；视神经鞘膜炎；视网膜中央动脉阻塞
INTRODUCTION
Giant cell arteritis (GCA) is also known as temporal arteritis. It is a granulomatous arteritis of unknown etiology and occurs mainly in patients over 50 years of age[1]. Visual loss, headache and jaw claudication are some of the presentation in GCA [2]. Visual loss from GCA is rare in SouthEast Asia but common in Caucasians[2,3]. We report a rare manifestation of patient with GCA, presenting with total ophthalmoplegia, optic perineuritis and central retinal artery occlusion in the right eye.
CASE REPORT
A 67yearold Chinese man complained sudden onset of painless loss of vision in the right eye for three days duration associated with minimal eye discomfort. He noticed diminution in vision after waking up in the morning. It was associated with limitation of movements of the right eye. Patient had several attacks of severe and localised right sided temporal headache prior to presentation. However there was no transient, repeated blurred vision or diplopia. There was no history of fever, intermittent claudication, joint pain or any neurological deficit. His premorbid vision was good and equal in both eyes. Patient was diagnosed to have diabetes mellitus and hypertension for the past 10 years on regular follow up. There was also no history loss of weight, malaise or depression. General examination revealed healthy, medium built man with good orientation. Patient was afebrile and vital signs were stable. Visual acuity in right eye was hand movement with positive relative afferent pupillary defect. There was a total restriction of extraocular movement in all gazes in the right eye (Figure 1A). Anterior segment was normal. Fundoscopy showed slight hyperaemic and swollen disc with normal vessels. The left eye was normal with visual acuity of 6/9. He had prominent pulsatile temporal artery bilaterally (Figure 2) associated with scalp tenderness. Examination of carotid artery revealed a good volume pulse with no carotid bruit. There was no neurological deficit. Other systemic examinations were unremarkable.
Magnetic resonance imaging (MRI) of brain and orbit demonstrated thickening of the right optic nerve on axial view with doughnut sign on coronal view suggestive of optic perineuritis (Figure 3). Extraocular muscles were normal in both eyes with no mass seen in the orbit. The brain and the left optic nerve were normal. Erythrocyte sedimentation rate (ESR) and Creactive protein (CRP) were elevated, 67mm/h and 42mg/L respectively. Collagen screening was negative. ECG and chest Xray were normal. A diagnosis of GCA was made clinically based on the above findings.
Patient was started on intravenous methylprednisolone 250mg qid for 3 days. On day 3 of treatment, he developed sudden deterioration of right eye vision to no perception of light. Fundoscopy revealed central retinal artery occlusion with hyperemic and swollen optic disc (Figure 4). The vision in the left eye was maintained at 6/9 with normal fundus appearance.
Figure 1Extraocular movements in the right eyeA: Total ophthalmoplegia of the right eye at presentation; B:After treatment with corticosteroids（略）
Figure 2 Prominent pulsatile temporal artery (arrow)（略）
Figure 3 MRI of orbit A: Coronal view showing doughnut sign (arrow); B: Axial view showing thickened optic nerve (arrow)（略）
The extraocular movements of the right eye were same. Oral prednisolone of 1mg/kg daily was started on day 4 and continued for another 11 days. A right temporal artery biopsy was performed only on day 15 after consented by patient. About 2.5cm length of the artery was harvested and sent for histopathological examination. It showed prominent continuous internal elastic lamina with no mononuclear inflammatory cell or giant cell (Figure 5). Patient was planned for repeat biopsy as the initial biopsy was negative. However, the second biopsy was not permitted by the patient. After a week, the ESR was reduced to 34mm/h. He was discharged after 2 weeks with maintenance dose of oral prednisolone of 1mg/kg daily. The steroid was tapered down slowly after 3 weeks with close monitoring of ESR and CRP. Six weeks later the right extraocular movements were markedly improved. Patient was able to move the eye in all direction with only slight limitation (Figure 1B). However, the visual acuity was maintained at no perception of light in the right eye. The left visual acuity was 6/9 with normal optic disc and fundus. The ESR was 17mm/h with normal CRP. Patient developed neovascularization in the iris and over the disc during subsequent followup at three months. Patient was given panretinal photocoagulation to arrest neovascularization in the right eye. He was maintained on oral prednisolone 5mg on every other day. His latest ESR and CRP were 16mm/h and 9mg/L respectively. Patient was kept under close observation for the left eye. He was asked to come immediately if there is any deterioration of vision in the left eye. He was maintained on low dose oral prednisolone with regular monitoring of ESR and CRP. His blood sugar and blood pressure were also monitored regularly.
Figure 4 Central retinal artery occlusion of the right eye with hyperaemic and swollen disc（略）
Figure 5 Superficial temporal artery with prominent continuous internal elastic lamina (HE×10)（略）
DISCUSSION
Giant cell arteritis is a systemic vasculitis and can produce bilateral visual loss [4]. The estimated annual incidence of GCA ranges between 0.49 and 23.3 cases per 100000 in the population older than 50 years of age [4]. Typically, it involves the branches of the external carotid artery and the vertebral, ophthalmic, and posterior ciliary arteries[1].
The pathogenesis of GCA is Tcell dependent and antigen driven. The inflammatory infiltrate is largely composed of CD4+ Tcells and macrophages with approximately 50% of lesions containing multinucleated giant cells clustered near the disrupted internal elastic lamina. Subsequently metalloproteinases, growth factors and angiogenic factors will be produced.
Ultimately there will be degradation of the internal elastic lamina and occlusive luminal hyperplasia [5]. This results in ischaemia and dysfunction of the organ system fed by the vessel evidenced by ophthalmoplegia, amaurosis fugax and intermittent diplopia and even complete visual loss as evidenced by central retinal artery occlusion in this case.
Visual loss, being one of the most devastating complications occurs in 50% of GCA patients and is often the only presenting symptom [3]. The common ocular manifestation is anterior ischaemic optic neuropathy whereas central retinal artery occlusion accounts for less than 10% of cases [3]. Patients with giant cell arteritis may present with unilateral and bilateral central retinal artery occlusion [6]. However only about 5% to 10% of patients with a central retinal artery occlusion have giant cell arteritis[6]. Other ocular manifestations include amaurosis fugax, diplopia, ophthalmoplegia, retrobulbar ischaemic optic neuropathy (rare), Horners syndrome and visual hallucinations [7]. Ocular motor disturbances may occur in 6%12% of patients with biopsyproven GCA, as the presenting manifestation [8]. The pathophysiological mechanisms for ophthalmoplegia are the primary affection of the eye muscles and/or cranial nerves [9]. The clinical manifestations associated with GCA are headache, scalp tenderness, polymyalgia, polyarthralgia, fever, anorexia, weight loss, jaw claudication, malaise and depression [7].
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