To describe a rare presentation of Giant Cell Arteritis with bilateral temporal arteritis & optic perineuritis.

Giant cell arteritis (GCA) is an arterial vasculitis that involves the aorta, carotid, and temporal arteries. It is most common in women greater than 50 years of age and is associated with polymyalgia rheumatica. Complications include cerebral ischemia, aortic aneurysm or dissection, and vision loss. In this case, we will further discuss a rare orbital complication related to GCA.

Consent was obtained from the patient.

A 78-year-old female with a PMH of ovarian cancer, CAD, and TIA presents with 3 months of right ear fullness associated with a month of right temporal headache, blurry vision, and 5 days of binocular diplopia. Neurological exam with normal visual fields at a one-foot distance; however, endorses horizontal diplopia at greater than four feet. No RAPD was observed. All other cranial nerves were preserved, and there was no tenderness to bilateral temporalis. Labs were significant for ESR 80 and CRP 7.41. MRI brain/orbits with and without contrast demonstrated enhancement throughout orbital fat and optic nerve sheaths bilaterally, indicating optic perineuritis. MRA brain and neck and MRV brain without IV contrast were unremarkable. Ultrasound demonstrated segments of bilateral superficial temporal arteries with absent flow, suggesting  bilateral temporal artery thrombosis. Right temporal artery biopsy pathology revealed transmural inflammation/thickening, histiocytes, and giant cells. She was treated with IV methylprednisolone 250 mg every 6 hours for 3 days, which led to rapid improvement of visual symptoms followed by prednisone taper.

Optic perineuritis is a rare inflammatory process involving the optic nerve sheath. It is typically associated with autoimmune conditions and rarely vasculitic diseases. This case highlights key clinical, radiographic, and pathologic features that led to the timely diagnosis and treatment of GCA.