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Research Letters |

Recovery of Vision From No Light Perception in Giant Cell Arteritis FREE

Matthew J. Thurtell, MBBS, FRACP; Randy H. Kardon, MD, PhD
[+] Author Affiliations

Author Affiliations: Departments of Neurology (Dr Thurtell) and Ophthalmology and Visual Sciences (Drs Thurtell and Kardon) and Veterans Affairs Medical Center (Drs Thurtell and Kardon), University of Iowa, Iowa City.


Arch Ophthalmol. 2012;130(8):1080-1082. doi:10.1001/archophthalmol.2012.308.
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Published online

Up to 50% of patients with giant cell arteritis (GCA) have visual symptoms early in the disease course, in most cases due to anterior ischemic optic neuropathy (AION).1 The vision loss from AION in GCA is often devastating, with the initial visual acuity being 20/200 or worse in more than 50% of patients.1,2 There is often, but not always, pallid optic disc edema and there is rarely a significant recovery, even with timely initiation of corticosteroids.26 We describe a patient with biopsy-proven GCA who had severe vision loss due to AION but had almost complete recovery of vision over subsequent weeks.

A 67-year-old diabetic woman without obvious retinopathy was evaluated 3 days following the sudden onset of vision loss in the left eye. She also reported headaches, scalp tenderness, jaw claudication, and weight loss over several weeks. She had begun treatment with oral prednisone (1 mg/kg/d) immediately after the onset of vision loss.

On examination, visual acuities were 20/20 OD and no light perception OS. There was a dense relative afferent pupillary defect in the left eye, with no direct pupillary response to light in the left eye. On funduscopic examination, there was hyperemic (nonpallid) optic disc edema in the left eye with a peripapillary cotton-wool spot, nerve fiber layer hemorrhages, and dot-blot hemorrhages (Figure 1A). Fluorescein angiography showed severely delayed choroidal filling in the left eye (Figure 2A). The erythrocyte sedimentation rate was 42 mm/h and the C-reactive protein level was 14.0 mg/L (reference range, <5.0 mg/L; to convert to nanomoles per liter, multiply by 9.524). Temporal artery biopsy showed histopathologic changes consistent with GCA. She continued treatment with oral prednisone (1 mg/kg/d).

Place holder to copy figure label and caption
Graphic Jump Location

Figure 1. Fundus photographs and results of kinetic (Goldmann) perimetry at the initial visit (A), 1 week later (B), and 6 weeks later (C).

Place holder to copy figure label and caption
Graphic Jump Location

Figure 2. Early- and late-phase photographs from fluorescein angiography at the initial visit (A) and 6 weeks later (B).

One week later, visual acuities were 20/20 OD and 20/50 OS. There was a dense relative afferent pupillary defect and decreased optic disc edema with resolving hemorrhages in the left eye (Figure 1B). Kinetic (Goldmann) perimetry showed severe visual field constriction in the left eye (Figure 1B). Six weeks after the initial visit, visual acuities were 20/20 OD and 20/40 OS. There was a 0.9–log unit relative afferent pupillary defect and mild temporal pallor without hemorrhages in the left eye (Figure 1C). Kinetic (Goldmann) perimetry showed mild generalized depression in the left eye (Figure 1C). Fluorescein angiography showed improved early choroidal filling in the left eye (Figure 2B). Six months later, her visual function and examination findings were unchanged.

Improvement in vision rarely occurs in patients with AION due to GCA, presumably because there has been complete occlusion of the posterior ciliary arteries causing optic nerve head infarction.4 Although some series have reported improvement in up to one-third of patients,2 there has often not been an improvement in the visual field, suggesting that the apparent recovery could be an artifact of visual acuity testing (eg, learned ability to eccentrically fixate).4 In series in which visual acuity and visual field changes have been reported, improvement in both has been observed in 4% to 5% of eyes, although the improvement was not substantial in most cases.4,6 Factors that predict visual recovery remain unclear, although the chance of improvement might be higher when treatment with corticosteroids is started early.4

Our patient with biopsy-proven GCA initially had no light perception in one eye, associated with signs of AION. She was immediately treated with prednisone and subsequently experienced dramatic improvement in both visual acuity and visual field over subsequent weeks. The presence of hyperemic rather than pallid optic disc edema and the delayed rather than absent choroidal filling on fluorescein angiography suggest that there was severe inflammatory narrowing, rather than complete occlusion, of the posterior ciliary arteries or development of collaterals. We propose that this unusual extent of vision recovery occurred because there was reversible ischemia rather than infarction of the optic nerve head. We suggest that hyperemic optic disc edema and delayed choroidal filling without posterior ciliary artery occlusion could predict a chance of improvement in patients with AION due to GCA.

Correspondence: Dr Thurtell, Department of Ophthalmology and Visual Sciences, University of Iowa, 200 Hawkins Dr, PFP, Iowa City, IA 52242 (mj.thurtell@gmail.com).

Financial Disclosure: None reported.

Additional Contributions: Sohan S. Hayreh, MD, PhD, provided helpful discussions.

Hayreh SS, Podhajsky PA, Zimmerman B. Ocular manifestations of giant cell arteritis.  Am J Ophthalmol. 1998;125(4):509-520
PubMed   |  Link to Article
Liu GT, Glaser JS, Schatz NJ, Smith JL. Visual morbidity in giant cell arteritis: clinical characteristics and prognosis for vision.  Ophthalmology. 1994;101(11):1779-1785
PubMed
Aiello PD, Trautmann JC, McPhee TJ, Kunselman AR, Hunder GG. Visual prognosis in giant cell arteritis.  Ophthalmology. 1993;100(4):550-555
PubMed
Hayreh SS, Zimmerman B, Kardon RH. Visual improvement with corticosteroid therapy in giant cell arteritis: report of a large study and review of literature.  Acta Ophthalmol Scand. 2002;80(4):355-367
PubMed   |  Link to Article
Foroozan R, Deramo VA, Buono LM,  et al.  Recovery of visual function in patients with biopsy-proven giant cell arteritis.  Ophthalmology. 2003;110(3):539-542
PubMed   |  Link to Article
Danesh-Meyer H, Savino PJ, Gamble GG. Poor prognosis of visual outcome after visual loss from giant cell arteritis.  Ophthalmology. 2005;112(6):1098-1103
PubMed   |  Link to Article

Figures

Place holder to copy figure label and caption
Graphic Jump Location

Figure 1. Fundus photographs and results of kinetic (Goldmann) perimetry at the initial visit (A), 1 week later (B), and 6 weeks later (C).

Place holder to copy figure label and caption
Graphic Jump Location

Figure 2. Early- and late-phase photographs from fluorescein angiography at the initial visit (A) and 6 weeks later (B).

Tables

References

Hayreh SS, Podhajsky PA, Zimmerman B. Ocular manifestations of giant cell arteritis.  Am J Ophthalmol. 1998;125(4):509-520
PubMed   |  Link to Article
Liu GT, Glaser JS, Schatz NJ, Smith JL. Visual morbidity in giant cell arteritis: clinical characteristics and prognosis for vision.  Ophthalmology. 1994;101(11):1779-1785
PubMed
Aiello PD, Trautmann JC, McPhee TJ, Kunselman AR, Hunder GG. Visual prognosis in giant cell arteritis.  Ophthalmology. 1993;100(4):550-555
PubMed
Hayreh SS, Zimmerman B, Kardon RH. Visual improvement with corticosteroid therapy in giant cell arteritis: report of a large study and review of literature.  Acta Ophthalmol Scand. 2002;80(4):355-367
PubMed   |  Link to Article
Foroozan R, Deramo VA, Buono LM,  et al.  Recovery of visual function in patients with biopsy-proven giant cell arteritis.  Ophthalmology. 2003;110(3):539-542
PubMed   |  Link to Article
Danesh-Meyer H, Savino PJ, Gamble GG. Poor prognosis of visual outcome after visual loss from giant cell arteritis.  Ophthalmology. 2005;112(6):1098-1103
PubMed   |  Link to Article

Correspondence

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