Neuro-ophthalmic Manifestations of Giant Cell Arteritis: A Review

Giant cell arteritis (GCA) is the most common vasculitis of large and medium vessels affecting adults over the age of 50. Due to its acute ischemic damage through a systemic inflammatory process, GCA is a medical emergency with the risk of permanent vision loss. Therefore, early intervention is critical. Patients often present with well-documented systemic manifestations such as jaw claudication, headache, scalp tenderness, and fatigue. These patients are usually subject to blood tests for inflammatory markers and temporal artery biopsy. However, clinical manifestations vary considerably and may masquerade as neuro-ophthalmic manifestations that are not pathognomonic of GCA. We conducted a review to discuss documented neuroophthalmic manifestations and provide insight into the rare presentations to help avoid pitfalls in its diagnosis. Findings from the included articles were sorted into afferent and efferent neuroophthalmic manifestations. According to our literature review, the main afferent manifestations documented are ischemic optic neuropathy, retinal artery occlusion, choroidal infarction, ocular ischemic syndrome, orbital inflammatory syndrome, and strokes causing visual field loss. The efferent manifestations include cranial neuropathy (3rd, 4th, and 6th), extraocular muscle ischemia, and internuclear ophthalmoplegia. Other rare causes are tonic pupil from ciliary ganglion involvement, uveitis, and encephalopathy. If GCA is suspected, in addition to inquiring about systemic symptoms and doing a complete neuro-ophthalmic exam, these patients should be sent for inflammatory serological markers, as well as temporal artery biopsy (TAB). If clinical suspicion remains high, high-dose steroids should be started immediately.