A Novel Case Reveals Important Consideration of the Diagnosis of Giant Cell Arteritis in Patients with Bilateral Painful Optic Perineuritis
Jordan Santos1, Faraz Behzadi1, Mozammil Alam1, Thomas Varkey1, Ghassan Ibrahim2, Trent Smith3, Alan Wang1
1Neurology, 2Pathology, 3Rheumatology, Banner University Medical Center Phoenix
Objective:

To present a novel case of giant cell arteritis (GCA) with bilateral temporal arteritis initially presenting with bilateral optic perineuritis (OPN).

Background:

GCA is a rare autoimmune vasculitis affecting medium and large-sized arteries. The classic demographic affected is Northern European females older than 50 years. Symptoms can include temple tenderness, jaw claudication, diplopia, and vision loss with severe complications such as ischemic stroke, aortic dissection, or aneurysm. If left untreated, vision loss can potentially become permanent. Thus, it is vital to recognize both typical and atypical clinical presentations so that immediate corticosteroid therapy can begin prior to diagnostic confirmation with temporal artery biopsy. Atypical presentations of GCA can include clinical manifestations such as OPN. Here we present such a case in a 57 year old female.

Design/Methods:

A consent form was obtained from the patient of interest.

Results:

A 57 year old female with past medical history of hypothyroidism presented with 2 weeks of worsening, fluctuating, subacute, central vision loss progressing from right eye to left with bilateral extraocular motility restriction and pain, jaw claudication, and pressure of the temples. Neurologic and funduscopic examination were unremarkable with no RAPD or visual field deficits. Labs were concerning for elevated ESR 70 mm/hr and CRP 26.8 mg/L. MRI of brain and orbits revealed bilateral, circumferential optic nerve sheath enhancement suggesting OPN. Temporal artery biopsy confirmed GCA with bilateral temporal arteritis. The patient was treated with IV methylprednisolone 1000 mg daily for 4 days with significant improvement in visual symptoms. She was then transitioned to a prednisone taper and upadacitinib 15 mg daily with continued symptom improvement.

Conclusions:

GCA can atypically present with bilateral OPN and associated cranial nerve dysfunction. This case emphasizes the importance of clinicians recognizing this atypical presentation so that appropriate diagnostic workup and immediate corticosteroid treatment can be initiated. 

10.1212/WNL.0000000000215399
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