Our patient was a 70-year-old African-American male with noninsulin-dependent diabetes who presented with a two-month history of two episodes of episodic vertical diplopia, each episode lasting 15 to 20 minutes. He denied associated ptosis.
Our differential diagnosis included microvascular nerve palsy, compressive lesion of the orbit, myasthenia gravis, giant cell arteritis (GCA) and skew deviation. Upon further questioning, the patient reported fatigue, right-sided headache, jaw claudication, and joint pain. This led to a focused exam with the following pertinent findings:
Head: +tenderness to palpation of the scalp
Distance visual acuity with correction: 20/15 OD, 20/20 OS
Pupils: briskly reactive without afferent pupillary defect OU
Alignment: 1 PD right hypertropia in primary, up-gaze and left gaze 2 PD right exophoria in primary gaze, 1 PD right exophoria in up-gaze
Motility: Full bilaterally
Dilated fundus exam: Normal nerve, macula, vessels and periphery OU
An MRI of the brain/orbits and MRA of the head/neck were negative. The erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) were elevated to 48 mm/hr and 3.0 mg/L, respectively. The constellation of clinical symptoms including episodic diplopia, constitutional symptoms of headache, scalp tenderness, jaw claudication and fatigue with elevated inflammatory markers pointed to a diagnosis of GCA. The patient was started on high-dose systemic steroids and his symptoms improved within 72 hours. A temporal artery biopsy was performed, demonstrating diffuse mononuclear cell infiltrate with giant cells at the intima-media junction, confirming GCA.
Giant cell arteritis (GCA) is an idiopathic inflammatory disorder leading to vascular obstruction of medium- to large-sized arteries, most often in individuals over 50 years of age. (Figure 1)1,2 While the classic presentation involves arteritic anterior ischemic optic neuropathy, GCA has a wide range of ocular and extraocular manifestations, including diplopia. Incidence of GCA presenting as diplopia varies from 5.9% to 33.3%.3-5 The diagnosis is usually made clinically and confirmed by the presence of elevated ESR, CRP and platelets with a positive and temporal artery biopsy.6 Timely diagnosis is crucial as GCA can threaten both vision and life if left untreated. Treatment involves high-dose systemic steroids for several months with close monitoring of inflammatory markers and recurrence of clinical symptoms. Some studies have shown interleukin-6 blockers to be effective nonsteroidal alternatives in refractory cases.7-9
Ophthalmologists must maintain a high index of suspicion for GCA in patients over 50 years of age presenting with diplopia, taking care to obtain a thorough review of systems and order appropriate laboratory studies. OM
- Kale N, Eggenberger E. Diagnosis and management of giant cell arteritis: a review. Curr Opin Ophthalmol. 2010;21:417-422.
- Weyand C, Goronzy J. Giant-cell arteritis and polymyalgia rheumatica. N Engl J Med. 2014; 371:50-57.
- Hayreh SS, Podhajsky PA, Zimmerman B. Ocular manifestations of giant cell arteritis. Am J Ophthalmol. 1998;125:509-520.
- González-Gay MA, García-Porrúa C, Llorca J, et al. Visual manifestations of giant cell arteritis. Trends and clinical spectrum in 161 patients. Medicine (Baltimore). 2000;79:283-292.
- Haering M, Holbro A, Todorova MG, et al. Incidence and prognostic implications of diplopia in patients with giant cell arteritis. J Rheumatol. 2014;4:1562-1564.
- Kermani T, Schmidt J, Crowson C, et al. Utility of erythrocyte sedimentation rate and C-reactive protein for the diagnosis of giant cell arteritis. Semin Arthritis Rheum. 2012;41:866-871.
- Mahr AD, Jover JA, Spiera RF, et al. Adjunctive methotrexate for treatment of giant cell arteritis: an individual patient data meta-analysis. Arthritis Rheum. 2007;56:2789-2797.
- Yates M, Loke YK, Watts RA, Macgregor AJ. Prednisolone combined with adjunctive immunosuppression is not superior to prednisolone alone in terms of efficacy and safety in giant cell arteritis: meta-analysis. Clin Rheumatol. 2014;33: 227-236.
- Unizony S, Arias-Urdaneta L, Miloslavsky E et al. Tocilizumab for the treatment of large-vessel vasculitis (giant cell arteritis, Takayasu arteritis) and polymyalgia rheumatica. Arthritis Care Res (Hoboken). 2012;64:1720-1729.