Simultaneous bilateral posterior ischemic optic neuropathy secondary to giant cell arteritis: a case presentation and review of the literature
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This report highlights a rare case of simultaneous bilateral blindness due to posterior ischemic optic neuropathy. Typically, ophthalmic involvement in giant cell arteritis is monocular or sequential ischemia of the anterior portion of the optic nerve, and less frequently simultaneous.
An 80-year-old Saudi male came with a history of simultaneous bilateral vision loss 5 days prior to presentation. The exam showed dilated non-reactive pupils, no light perception in both eyes, and normal fundus exam. C-reactive protein and erythrocyte sedimentation rate levels were high Magnetic resonance imaging and magnetic resonance angiography of the brain showed a right posterior optic nerve lesion and absence of flow in both ophthalmic arteries respectively. A left temporal artery biopsy confirmed giant cell arteritis.
The presentation of GCA can be atypical and patients may present with simultaneous blindness. Bilateral simultaneous PION does not exclusively occur in a post surgical setting, emphasizing the importance of decreasing the threshold of suspicion of similar cases to avoid further neurological complications.
KeywordsGiant cell arteritis Blindness Headache Posterior ischemic optic neuropathy Neuro-ophthalmology
Anterior ischemic optic neuropathy
Erythrocyte sedimentation rate
Giant cell arteritis
Magnetic resonance imaging
Magnetic resonance angiography
Posterior ischemic optic neuropathy
Giant cell (temporal) arteritis (GCA) is a chronic vasculitis of large and medium-sized vessels, which can affect individuals over 50 years of age  and is the most common systemic vasculitis . Typically, patients may present with a new headache, jaw claudication and/or monocular vision loss . In comparison to anterior ischemic optic neuropathy (AION), posterior ischemic optic neuropathy (PION) is an uncommon form of optic nerve ischemia that can occur secondarily to GCA . Here, we describe an elderly patient who presented with simultaneous bilateral loss of vision due to posterior ischemic optic neuropathies and biopsy proven GCA. This case outlines the importance of rapid recognition of GCA in the setting of an uncommon presentation as early management can prevent devastating and irreversible complications.
We report a case of an 80-year old hypertensive and diabetic Saudi male referred to our center after developing sudden bilateral painless visual loss five days earlier. There was a history of a bilateral temporal headache that had started a year earlier. The headache was more prominent on the left side, mild to moderate in severity, and stabbing in nature. It used to occur on average once a week and would spontaneously resolve over several seconds. However, the frequency had increased in the months preceding visual loss, occurring almost daily. There was no report of any previous episodes of diplopia, transient visual loss, jaw claudication, myalgia, constitutional symptoms, motor or sensory symptoms.
Reports of posterior ischemic optic neuropathy secondary to giant cell arteritis
Number of patients
Number of cases
Liozon et al. 2001
147 Biopsy proven GCA
48 Ophthalmic GCA
Both described as bilateral and recovered
Hayreh et al. 1998 
170 Biopsy proven GCA
85 Ophthalmic GCA
One described as Bilateral
Garrity et al. 2017 
32 Biopsy proven ophthalmic GCA
African American cohort
Danesh-Meyer et al. 2005 
34 Biopsy proven ophthalmic GCA
One had PION on one side and AION on the other side that was described as bilateral at presentation
Liu et al. 1994 
45 biopsy proven ophthalmic GCA
One had PION on one side and AION on the other side that was simultaneous onset
Aiello et al. 1993 
245 Ophthalmic diagnosis
204 biopsy proven
Chaudhry et al. 2007 
102 Suspected ophthalmic GCA
7 biopsy proven
Sadda et al. 2001
72 PION patients
None simultaneous due to arteritis
Hayreh 2004 
43 PION patients
The current patient presented with a history of headache for one year that culminated in bilateral visual loss from optic nerve ischemia. The differential diagnosis for a presentation of acute optic neuropathy with headache includes arteritic ischemic optic neuropathy, infections (cat-scratch, syphilis), inflammatory (para-infectious, multiple sclerosis, systemic autoimmune, paraneoplastic, and sarcoidosis). GCA in the current scenario presented fairly typically, readily distinguishing it from other conditions. The ESR and CRP were high which suggested an inflammatory process and abnormal signals were seen on the MRI suggesting infarction.
Simultaneous and sudden complete blindness of both eyes due to PION is an unusual presentation in GCA. Bilateral visual loss is usually due to the sequential onset of AION, CRAO, or PION in any combination [4, 15]. One cohort that looked at a small subgroup of simultaneous onset bilateral ischemic optic neuropathies found examination findings of different ages in each eye, raising the possibility that patients may not become aware of visual loss until it involves the other eye . Thus, an important consideration in the current case is defining how simultaneous was the onset. Our patient witnessed the visual loss occur while he was awake, and was found to have symmetrical exam findings when assessed at the referring center on the day of onset, and again at our center 5 days later with no clear disc abnormalities. There was diffusion restriction involving the right optic nerve with absence of both ophthalmic arteries by MRA. It was not surprising to find asymmetrical findings on MRI as PION changes may not always seen on MRI , and our patient did not have imaging done until one week after the event reducing the possibility of witnessing acute changes. Asymmetry in the observed MRI signals of bilateral simultaneous onset post surgical PION have also been described .
In this report we presented a patient who was clinically and histologically diagnosed as GCA. IV steroids were started upon his arrival five days from symptom onset, with dramatic improvement in headache, however, no improvement in vision occurred as is the case with optic nerve ischemia secondary to GCA . Given the yearlong history of headache, this case emphasizes the importance of early recognition of headache disorders among elderly; the threshold of suspecting GCA in elderly patients should be very low. This case highlights a rarely identified ocular presentation of GCA, it is important to identify this early in order to start treatment to avoid devastating and potentially permanent ocular or systemic complication. When bilateral PION occurs simultaneously in a non-surgical setting in an older individual, then the possibility of GCA as an etiology should be considered high.
The authors thank the patient and his son for their collaboration.
No targeted funding reported.
Availability of data and materials
The datasets used and/or analysed during the current study are available from the corresponding author on reasonable request.
AA and TM acquisition of data, analysis and interpretation, manuscript concept and design; YM critical revision of the manuscript for important intellectual content and study supervision. SH and FH analysis and interpretation of data. All authors read and approved the final manuscript.
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The authors declare that they have no competing interests.
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- 6.Vodopivec I, Rizzo JF. Ophthalmic manifestations of giant cell arteritis. Rheumatol (United Kingdom). 2018;57(May):ii63-ii72.Google Scholar
- 8.Garrity ST, Pistilli M, Vaphiades MS, Richards NQ, Subramanian PS, Rosa PR, et al. Ophthalmic presentation of giant cell arteritis in African-Americans. Eye 2017;31(1):113–8. Available from: https://doi.org/10.1038/eye.2016.199
- 9.Brekke LK, Diamantopoulos AP, Fevang B-T, Aβmus J, Esperø E, Gjesdal CG. Incidence of giant cell arteritis in Western Norway 1972–2012: a retrospective cohort study. Arthritis Res Ther. 2017;19(1):278 Available from: https://arthritis-research.biomedcentral.com/articles/10.1186/s13075-017-1479-6.CrossRefGoogle Scholar
- 23.Aiello PD, Trautmann JC, McPhee TJ, Kunselman AR, Hunder GG. Visual Prognosis in Giant Cell Arteritis. Ophthalmology [Internet]. 1993;100(4):550–555. Available from: https://doi.org/10.1016/S0161-6420(93)31608-8.
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