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Show Journal of Neuro- Ophthalmohgy 18( 2): 124- 126, 1998. © 1998 Lippincott- Raven Publishers, Philadelphia Postural Vision Loss in Giant Cell Arteritis Marianne Diego, M. D., and Curtis E. Margo, M. D., M. P. H. Two patients with biopsy- proven giant cell arteritis experienced bilateral transient vision loss after bending over and after getting up from a supine position. One patient had no demonstrable signs of carotid, ophthalmic, or anterior ciliary vascular disease, suggesting that his episodes of transient vision loss were due to vertebrobasilar insufficiency. The other patient experienced bilateral postural vision loss in the context of impending bilateral anterior ischemic optic neuropathy. Bilateral transient postural vision loss is an uncommon manifestation of giant cell arteritis that could reflect either severe bilateral vascular compromise of the anterior circulation or severe vertebrobasilar insufficiency. In either situation, prompt evaluation and treatment is indicated to prevent the irreversible sequelae of the disease. Key Words: Giant cell arteritis- Postural hypotension- Vertebrobasilar insufficiency. Vision loss affects nearly a third of patients with giant cell arteritis ( 1). Vision loss typically occurs abruptly, without pain, and presents with 20/ 200 or worse acuity in more than half of those patients with biopsy- proven disease ( 2). Most patients present with findings of an anterior ischemic optic neuropathy ( 2). Before vision is affected, headache and jaw claudication are typically noted in people with giant cell arteritis; however, a substantia] proportion may present with minor premonitory symptoms or may be asymptomatic. Transient vision loss, or amaurosis fugax, may precede permanent vision loss in a few patients ( 3). We describe two patients with giant cell arteritis who complained of transient bilateral vision loss associated with changing body position. CASE REPORTS Case 1 A 72- year- old man was referred for evaluation of transient visual loss. His symptoms had been present for 2 weeks and consisted of several episodes of transient, bilateral vision loss lasting 2 to 15 minutes. These painless episodes were associated with changing position: going Manuscript received July 29, 1997; accepted January 8, 1998. From the Department of Ophthalmology, University of South Florida College of Medicine, Tampa, Florida, U. S. A. Address correspondence and reprint requests to Curtis E. Margo, M. D., M. P. H., Department of Ophthalmology, Watson Clinic, 1600 Lakeland Hills Boulevard, Lakeland, FL 33805, U. S. A. from a bending, head- down position, to sitting or standing. Vision loss was described as complete and total darkness. Review of systems revealed a new type of " arthritic" pain of the shoulders and neck over the last 3 months. The patient also had a 5- pound weight loss over the previous month and a persistent headache for 2 weeks, which was exacerbated by touching his forehead. Examination showed 20/ 30 best- corrected visual acuity in each eye. Pupillary reaction to light and ocular motility examinations were normal. Slit- lamp examination and dilated fundus examination were negative except for moderate arteriolar attenuation of retinal arterioles. No emboli were seen. Both superficial temporal arteries and their branches were visible, without palpable pulses, and tender to touch. Supine blood pressures were performed daily for 3 days and averaged 140/ 88 mm Hg without any significant orthostatic drop. A single transient episode of bilateral vision loss, lasting 3 minutes, occurred the first day when testing blood pressure and pulse ( before initiation of therapy). A Westergren sedimentation rate was 120 mm/ hour. Biopsy of the right superficial temporal artery showed giant cell arteritis with focal, total vascular occlusion. B- mode ultrasound studies of the carotid artery showed no clinically significant obstruction, with less than 25% occlusion of the external carotid artery on each side. Computed tomography scans of the brain and orbits were negative. Compression ophthalmodynamometry revealed systolic central artery occlusion pressures of 128 mm Hg OD and 126 mm Hg OS. Diastolic pressures were 72 mm Hg OD and 74 mm Hg OS. Concurrent brachial arm pressure was 142/ 84. The patient was treated with intravenous methylpred-nisolone, 250 mg every 6 hours, for 3 days. Shoulder and neck pain was relieved within 1 day of starting steroids. Oral prednisone 80 mg/ day was started on the fourth day and tapered slowly over the next 6 months based on sedimentation rate and symptoms. Symptoms of postural vision loss were not reported by the patient after discharge from the hospital. The patient was monitored as an outpatient for 11 months before being lost to follow-up. Case 2 A 72- year- old woman with diabetes presented with a 1- month history of diffuse temporofrontal headaches, a transient episode of lower visual field loss in the right 124 POSTURAL VISION LOSS IN GIANT CELL ARTERITIS 125 eye, and severe visual loss in the left eye the previous day. The patient had a history of treated hypertension and coronary atherosclerotic heart disease. Review of systems revealed a 6- pound weight loss over the last 2 weeks, jaw pain with chewing, occasional chills, and scalp tenderness. On examination, corrected visual acuity was 20/ 70 OD and hand motions OS. A left afferent pupillary defect was present. Ocular motility was full. Both optic discs were swollen. Confrontation and Gold-mann visual fields showed a right inferior altitudinal field defect. The superficial temporal arteries were grossly visible bilaterally but pulseless and tender. Erythrocyte sedimentation rate was 107 mm/ hour. Biopsy of the right superficial temporal artery showed giant cell arteritis with vascular occlusion. The patient was started on intravenous methylprednis-olone ( 250 mg q 6 h) immediately after the suspicion of giant cell arteritis was raised in the clinic. On the second day of therapy, the patient experienced bilateral, total vision loss after bending over to pick up her slippers. Vision returned in both eyes after 15 minutes. On careful questioning, the patient was able to discern transient complete loss of vision in the left eye from her baseline of " hand motion" vision. Arterial Doppler studies showed bilaterally patent external and internal carotid arteries and partial obstruction of the right vertebral artery. Magnetic resonance imaging of the brain showed age- related cortical atrophy. After 3 days of intravenous steroids, the patient was converted to 80 mg of oral prednisone per day and her condition monitored by regular examinations and measurement of erythrocyte sedimentation rate. Visual acuities and fields remained unchanged 1 month after initiation of steroid therapy. The patient had no more episodes of transient vision loss. DISCUSSION Postural vision loss has been associated with active giant cell arteritis. Hollenhorst ( 4), in 1967, described four patients who experienced loss of vision on sitting or standing. Coexisting atheromatous carotid artery was present in all four patients and was thought to have contributed to the postural vision loss in each patient. Wykes and associates ( 5) described a similar case in which an elderly woman complained of monocular vision loss with any abrupt change of posture. Her symptoms resolved with prednisone treatment. Our patients differ in one important way from those previously described because their transient postural visual loss was bilateral. The pattern of bilateral vision loss would indicate that the vascular obstruction must affect the visual pathway at two separate sites simultaneously, or it involves the vertebrobasilar system. Detailed pathologic study of patients dying during the active phase of giant cell arteritis have shown that the vertebrobasilar system is often severely involved ( 6). Autopsy findings in 12 patients with active giant cell arteritis before death showed all had severe involvement of the vertebral and basilar arteries ( 6). These finding contrast with the relative paucity of reports describing clinical manifestations of giant cell arteritis due to occlusive vascular disease of the posterior cerebral circulation. This discrepancy may be due to the fact that diffuse vascular lumen narrowing secondary to obliterative vasculitis has a greater hemodynamic effect on narrow-caliber arteries, such as the ophthalmic artery and the long and short posterior ciliary arteries, than on larger-caliber vessels such as the vertebral artery. Although uncommon, clinical manifestations of the vertebrobasilar system in giant cell arteritis can occur ( 7,8). Chisholm ( 7) reported a 79- year- old man who presented with light perception vision due to bilateral occipital lobe infarction from giant cell arteritis. Occipital lobe infarction with giant cell arteritis has subsequently been reported by others ( 9,10). In a series of 166 consecutive patients with biopsy- proven giant cell arteritis, four ( 2.4%) had clinical manifestations of disease attributed to involvement of the vertebrobasilar system ( 3). Although relatively rare, the lateral medullary syndrome and the syndrome of truncal ataxia- ophthalmoplegia are other potential complications of vertebrobasilar insufficiency due to giant cell arteritis ( 6,8,11). The pattern of artery involvement in giant cell arteritis reveals that clinically significant involvement of the intracranial arteries is rare, except for the first 5 mm of the intradural vertebral arteries. Strokes occasionally occur on the basis of occlusion of internal carotid, middle cerebral, or vertebral arteries ( 6). There is a positive correlation, however, between the severity of postmortem involvement of the vertebral arteritis and occipital infarction and brainstem ischemia in people who die with active giant cell arteritis ( 6). It is not surprising that the lateral medullary syndrome is seen with some regularity in giant cell arteritis because it is caused by occlusion of the posterior inferior cerebellar artery, the first intracranial branch of the vertebral artery. Postmortem studies have shown that posterior circulation ischemia in giant cell arteritis may be caused by either thrombotic occlusion of the vertebral arteries or embolization ( 6). Thrombus may also extend distally to occlude branches of the vertebral arteries. Monteiro and associates ( 8) have described two patients with giant cell arteritis and the syndrome of truncal ataxia- ophthalmoplegia, which is another manifestation of vertebral artery insufficiency. Patients with this syndrome exhibit truncal ataxia, upbeat nystagmus, and up-gaze palsy presumably due to severe obstruction of one or both vertebral arteries combined with embolization to distal basilar artery. Decrease in the collateral circulation through the circle of Willis is considered an important aggravating factor. Truncal ataxia results from infarction of the cerebellar vermis, which is frequently supplied by the posterior inferior cerebellar artery. We suspect that transient bilateral vision loss reported by our first patient may have been due to vertebrobasilar involvement of giant cell arteritis. Bilateral severe posterior ciliary artery involvement is possible, but the patient's carotid arteries were patent on ultrasonography and compression ophthalmodynamometry was negative. J Neiim- Ophlhulmol, Vol. 18, No. 2. I99H 126 M. DIEGO AND C. E. MARGO In theory, this indicates normal ophthalmic artery pressure and relative vascular patency, although normal ophthalmodynamometry results do not exclude clinically significant occlusion of the posterior ciliary arteries. The cessation of postural vision loss with corticosteroid therapy suggests that giant cell arteritis was responsible for these transient visual symptoms. The second patient also had a transient episode of bilateral postural vision loss, but localization of her disease process is more complicated. Her monocular vision loss and bilaterally swollen optic discs indicate bilateral anterior ischemic optic neuropathy. We suspect the patient's single episode of orthostatic vision loss probably reflected her precarious ocular circulation at the time of hospitalization. Transient visual obscurations from disc edema are possible, but the duration of visual loss is more consistent with ischemia. Doppler studies also revealed partial occlusion of the vertebral arteries, which makes it difficult to exclude the possibility of transient vertebrobasilar insufficiency without performing angiography. The resolution of the patient's symptoms with corticosteroids does not help to localize the site of the lesion( s). Bilateral vision loss associated with changes in posture in an elderly person can be a symptom of giant cell arteritis. The site of vascular obstruction in this setting may not always be apparent, because giant cell arteritis can affect both the anterior and posterior vascular supply of the visual pathway. Postural vision loss in giant cell arteritis should be regarded as a sign of impending vascular occlusion and needs to be evaluated and treated accordingly. REFERENCES 1. Goodman BW Jr. Temporal arteritis. Am J Med 1979; 67: 839- 52. 2. Liu CT, Glaser IS, Schatz NJ, Smith JL. Visual morbidity in giant cell arteritis: clinical characteristics and prognosis. Ophthalmology 1994; 101: 1779- 85. 3. Caselli RJ, Hunder GG, Whisnant JP. Neurologic disease in biopsy- proven giant cell ( temporal) arteritis. Neurology 1988; 38: 352- 9. 4. Hollenhorst RW. Effect of posture of retinal ischemia from temporal arteritis. Arch Ophthalmol 1967; 78: 569- 77. 5. Wykes WN, Adams GGW, Cullen JF. Temporal arteritis: visual loss associated with posture. Neuro- ophthalmology 1984; 4: 107- 9. 6. Wilkinson IMS, Russell RWR. Arteritis of the head and neck in giant cell arteritis: a pathological study of the pattern of arterial involvement. Arch Neurol 1972; 27: 378- 91. 7. Chisholm IH. Cortical blindness in cranial arteritis. Br J Ophthalmol 1975; 59: 332- 3. 8. Monteiro MLR, Coppeto JR, Greco P. Giant cell arteritis of the posterior cerebral circulation presenting with ataxia and ophthalmoplegia. Arch Ophthalmol 1984; 102: 407- 9. 9. McAlindon TE, Ferguson IT. Mononeuritis multiplex and occipital lobe infarction complicating giant cell arteritis. Br J Rheumatol 1989; 28: 257- 63. 10. Nevyas JY, Nevyas HJ. Giant cell arteritis with normal erythrocyte sedimentation rate: a management dilemma. Metah Pediatr Syst Ophthalmol 1987; 10: 18- 21. 11. 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