Woman presents with unilateral blurred vision
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An 84-year-old woman presented to the Tufts Medical Center emergency department with a chief complaint of blurred vision in the right eye.
The woman, who had a medical history of temporal artery biopsy-proven giant cell arteritis (GCA) tapered off of prednisone and tocilizumab, endorsed a gradual, global decrease in the quality of vision in the right eye as well as slowly decreasing nasal visual field in the right eye starting a few weeks before presentation. She had been seen by her local glaucoma specialist and was told her exam was normal and stable but was recommended to obtain an erythrocyte sedimentation rate (ESR), which was normal at 13 mm/hr.
Source: Julia Watson, MD, and Yosbelkys Martin-Paez, MD
She traveled out of state and experienced acute worsening of vision approximately a week before presentation, so she called her neuro-ophthalmologist at Tufts Medical Center and was recommended to present to a local emergency department. However, she preferred to return to Boston to be evaluated at Tufts. Upon evaluation by the consulting ophthalmology resident, she reported her vision changes began at least 6 months before presentation. GCA review of systems, including double vision, headache, fevers, chills, weight loss, jaw claudication, and stiffness in shoulders or hips, was negative. She denied new floaters, flashes of light, eye redness, pain at rest or with eye movements, and light sensitivity.
Her medical history also included psoriasis, glenohumeral osteoarthritis, GERD, overactive bladder, prediabetes (last HbA1c, 6.2) and supraventricular tachycardia. Her medications included metoprolol, amlodipine, atorvastatin, omeprazole, zoledronic acid and vibegron. Her family history was notable for a cousin with GCA. She had no known drug allergies. She denied smoking or drug use. She drank alcohol socially.
Her ocular history included glaucoma, for which she was followed locally by a glaucoma specialist and was on timolol every morning in the right eye and latanoprost every evening in the right eye, posterior vitreous detachment in both eyes and dry eyes. She had a remote history of bilateral cataract surgery and YAG capsulotomies.
Examination
On exam, near best corrected visual acuity was 20/25 eccentrically in the right eye and 20/20 in the left eye. Pupils were equally round and reactive to light with 1+ right relative afferent pupillary defect. IOP measured by Tono-Pen (Reichert) was 27 mm Hg in the right eye and 19 mm Hg in the left eye. The exam was notable for dyschromatopsia, and the patient was unable to see the nasal Ishihara numbers. Confrontation visual fields were slightly limited superonasally. Extraocular movements were full with orthotropic alignment.
Anterior segment slit lamp exam was unremarkable in both eyes. On dilated fundus exam, the right optic nerve was pale and cupped with peripapillary atrophy. The left optic nerve appeared pink and sharp with 0.3 cup-to-disc ratio. The macula and peripheral retina were normal in both eyes.
Ancillary testing
OCT retinal nerve fiber layer (RNFL) in the right eye (Figure 1a) showed superior and inferior thinning. Ganglion cell analysis in the right eye (Figure 1b) showed diffuse thinning sparing nasally. Humphrey visual field (HVF) 30-2 (Figure 2b) showed dense nasal depression inferiorly and superiorly.
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Unilateral blurred vision
GCA should be on the differential for a wide range of vision changes in older adults. In an older patient with a known history of GCA, it is even more imperative to rule out the possibility of relapsing GCA manifesting as arteritic anterior ischemic optic neuropathy (AAION) or central retinal artery occlusion (CRAO). However, other differential diagnoses for an older woman presenting with progressive unilateral visual field deficits include progression of glaucomatous optic neuropathy, optic disc drusen and optic nerve sheath meningioma, among others.
There was low suspicion for GCA with ocular involvement given negative review of systems, relatively preserved visual acuity, unilateral presentation, and no findings of acute AAION or acute CRAO on thorough posterior segment exam and/or imaging. Although to the patient’s knowledge there had been no progression of her glaucoma over the preceding year, the slowly progressive optic neuropathy with cupped nerve on fundus exam and automated visual field with a pattern of depression pointed to glaucoma as the most likely cause of her recent symptoms.
Workup, management and follow-up
ESR and C-reactive protein were 15 mm/hr and 0.93 mg/dL, respectively. MRI of the brain and orbits with and without contrast (Figure 3) showed diffuse atrophy of the right optic nerve’s intraorbital segment with increased prominence of the optic nerve sheath. There was no evidence of associated optic nerve or perineural enhancement to suggest active optic neuritis. There were no intracranial or intraorbital masses or lesions.
OCT RNFL and HVF 30-2 from the initial evaluation at Tufts 6 years prior in the setting of GCA diagnosis were reviewed (Figures 4 and 5). OCT RNFL of the right eye showed superior thinning and borderline inferior thinning, and ganglion cell complex (GCC) in the right eye showed temporal thinning. HVF 30-2 of the right eye showed nasal depression. Testing in the left eye was normal.
Given the increased IOP in the right eye, dorzolamide and brimonidine were added to the patient’s regimen. One-month follow-up with Tufts glaucoma department was arranged.
Discussion
GCA is a life- and vision-threatening must-not-miss diagnosis. It is also a chronic and relapsing disease with most relapses occurring in the first 2 years after diagnosis. It requires regular surveillance and vigilance on the part of patients and a multidisciplinary team. However, as Hickam’s dictum states, patients can have as many diseases as they please. Although progression of glaucoma is not typically acute, patients may become acutely aware of their field deficits, especially as they encroach centrally. Despite the patient’s report that her glaucoma was stable per her local ophthalmologist, it is important to draw independent conclusions from the information at hand. In addition, given the high rate of fellow eye involvement in cases of unilateral open-angle glaucoma, consistent and close follow-up is essential for this patient.
- References:
- Alba MA, et al. Autoimmun Rev. 2024;doi:10.1016/j.autrev.2024.103580.
- Niziol LM, et al. JAMA Ophthalmol. 2018;doi:10.1001/jamaophthalmol.2018.3274.
- For more information:
- Edited by William W. Binotti, MD, and Julia Ernst, MD, PhD, of New England Eye Center, Tufts University School of Medicine. They can be reached at william.binotti@tuftsmedicine.org and julia.ernst@tuftsmedicine.org.
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