Paracentral Acute Middle Maculopathy as a Manifestation of Giant Cell Arteritis

Clinical Correspondence Section Editors: Robert Avery, DO Karl C. Golnik, MD Caroline Froment, MD, PhD An-Gour Wang, MD Paracentral Acute Middle Maculopathy as the Initial Presentation of Giant Cell Arteritis Heather Broyles, DO, Joseph Chacko, MD, John Chancellor, MD, MS, Frank LoRusso, MD, Paul H. Phillips, MD, Azin Mashayekhi, MD, Sami Uwaydat, MD P aracentral acute middle maculopathy (PAMM) was first described as a novel variant of acute macular neuroretinopathy affecting the middle layers of the macula (1). Spectral-domain optical coherence tomography (SD-OCT) shows hyper-reflective band–like lesions limited to the level of the inner nuclear layer. Recent studies of OCT angiography (OCT-A) suggest that PAMM is associated with vascular ischemia involving the deep capillary plexus (2,3). En face OCT shows placoid or globular hyper-reflective bands at the level of the deep vascular complex. Patients typically present with acute onset of one or more paracentral scotoma(s) in one or both eyes. Although PAMM can occur as an isolated phenomenon, it is often the result of retinal vascular pathology. Thus, PAMM lesions can occur secondary to local retinal ischemic injury, such as retinal vascular occlusion, or as a manifestation of a systemic disease with vascular risk factors such as diabetic retinopathy (3). Giant cell arteritis (GCA) is a form of systemic vasculitis in persons older than 50 that can lead to irreversible vision loss by causing occlusion of the central retinal artery or short posterior ciliary arteries. Patients with GCA-related retinal artery occlusion have severe retinal ischemia with diffuse hyper-reflectivity on OCT reflecting middle and inner retinal infarction. We report a patient who presented with isolated PAMM and was later diagnosed with GCA. To the best of our knowledge, the association between GCA and isolated PAMM reflecting focal retinal ischemia limited to the inner nuclear layer has not been described in the literature. CASE REPORT Her medical history was unremarkable. She denied hypertension, diabetes, and heart disease. On initial examination, her best-corrected visual acuity was 20/30 in the right eye, 20/20 in the left eye, with a paracentral defect by confrontational visual fields in the right eye. Intraocular pressures were 9 and 11 in the right and left eye, respectively. Pupils were equal, briskly reactive, with no relative afferent pupillary defect. Examination of the right eye revealed a normal anterior segment and a small cotton wool spot inferonasally (Fig. 1A). Examination of the unaffected left eye was normal. Fluorescein angiography of the right eye showed delayed arterial filling but normal arteriovenous filling times (Fig. 1B–D). SD-OCT (Cirrus 5000, Zeiss, MN) of the macula showed multiple hyper-reflective band–like lesions limited to the level of the inner nuclear layer, with gray lesions in the parafoveal position on nearinfrared reflectance imaging (Fig. 2A–D). OCT-A (Spectralis; Heidelberg Engineering, Franklin, Massachusetts) revealed focal capillary nonperfusion at the level of the deep capillary plexus (Fig. 2E, F). Fluorescein angiography, SDOCT, and OCT-A of the left eye were normal. MRI of the brain, EKG, and echocardiogram were normal. The erythrocyte sedimentation rate and C-reactive protein were significantly elevated at 91 mm/hr and 63 mg/L, respectively. On further questioning, the patient reported left-sided temporal headaches for the past 3 weeks. She denied jaw claudication and arthralgias. The patient was started on IV methylprednisolone [Solumedrol] 250 mg 4 times daily, and discharged home on 60 mg oral prednisone daily. The pathology results of temporal artery biopsy performed 4 days after presentation were consistent with active temporal arteritis (Fig. 1E, F). A 75-year-old woman presented with a 5 day history of a paracentral scotoma in her right eye. Department of Ophthalmology (HB, J. Chacko, J. Chancellor, PHP, SU), Harvey and Bernice Jones Eye Institute, University of Arkansas for Medical Sciences, Little Rock, Arkansas; Department of Ophthalmology (FL), Central Arkansas Veterans Healthcare System, Little Rock, Arkansas; and Department of Pathology (AM), University of Arkansas for Medical Sciences, Little Rock, Arkansas. The authors report no conflicts of interest. Address correspondence to Paul H. Phillips, MD, Department of Ophthalmology, Jones Eye Institute, University of Arkansas for Medical Sciences, 4301 W. Markham Street, Slot # 523, Little Rock, AR 72205; E-mail: [e-mail] Broyles et al: J Neuro-Ophthalmol 2021; 41: e157-e159 DISCUSSION The most common ophthalmologic manifestations of GCA are anterior ischemic optic neuropathy and central retinal artery occlusion (4). These most often result in profound, irreversible loss of vision in the involved eye, and should prompt urgent confirmation of the diagnosis and initiation of steroid therapy to protect the vision of the fellow eye. SD-OCT shows diffuse hyper-reflectivity of the inner and middle retina representing severe ischemic injury (5). e157 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Clinical Correspondence FIG. 1. A. Color fundus photograph of the right eye with arrow highlighting a single cotton wool spot. B–D. Fluorescein angiography of the right eye demonstrates delayed filling of major retinal arteries at 30.5 seconds. However, normal arteriovenous transit times between 30.5 and 48.1 seconds show no evidence of retinal arterial occlusion. E. H&E stain of temporal artery biopsy demonstrating diffuse lymphohistiocytic inflammation. F. H&E stain of temporal artery biopsy with arrow highlighting multinucleated giant cell. FIG. 2. A. SD-OCT of the right eye demonstrating placoid, hyper-reflective bands at the level of the inner nuclear layer (arrow) that spare the outer retina. B. Corresponding SD-OCT of the left eye demonstrating normal appearance of retinal layers. C. En face OCT of the right eye at the level of the deep vascular complex demonstrating placoid appearance of hyper-reflective bands. D. Corresponding en face structural OCT of the left eye demonstrating normal appearance. E. OCT-A of the right eye at the level of the deep vascular complex showing focal vascular attenuation with pruning of intermediate and deep capillary plexus. F. Corresponding OCT-A of the left eye demonstrating normal appearance of retinal vasculature. OCT-A, OCT angiography; SD-OCT, Spectral domain optical coherence tomography. PAMM involves ischemia of the deep capillary plexus resulting in selective infarction limited to the inner nuclear layer and might be the only finding of an underlying retinal vasculopathic or systemic disease. A recent study showed exclusive development of PAMM in groups of patients with an isolated nonarteritic cilioretinal artery occlusion (CILRAO) and CILRAO secondary to central retinal vein occlusion (5). In the 2 patients with CILRAO associated with GCA, diffuse hyper-reflectivity of multiple retinal layers indicative of more severe ischemic injury, not consistent with PAMM, was noted (5). Our patient had lesions diagnostic of PAMM with SDOCT findings of hyper-reflective lesions limited to the level of the inner nuclear layer and en face OCT demonstrating placoid hyper-reflective bands at the level of the deep vascular complex. Given that our patient did not have a e158 cilioretinal artery, the etiology of PAMM seems to be directly related to deep capillary plexus ischemia from GCA. Our case shows that patients with GCA may present with only mild visual loss from PAMM with ischemic injury limited to the inner nuclear layer. PAMM may be a subtle or early ocular sign of GCA that would prove invaluable to facilitate earlier diagnosis and prevent severe loss of vision. Isolated findings of PAMM in a patient within the appropriate age group should prompt an evaluation for GCA. STATEMENT OF AUTHORSHIP Category 1: a. Conception and design: H. Broyles, J. Chacko, J. Chancellor, F. LoRusso, P. H. Phillips, A. Mashayekhi, and S. Uwaydat; b. Acquisition of data: H. Broyles, J. Chacko, J. Chancellor, F. LoRusso, P. H. Phillips, A. Mashayekhi, and S. Uwaydat; c. Broyles et al: J Neuro-Ophthalmol 2021; 41: e157-e159 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Clinical Correspondence Analysis and interpretation of data: H. Broyles, J. Chacko, J. Chancellor, F. LoRusso, P. H. Phillips, A. Mashayekhi, and S. Uwaydat. Category 2: a. Drafting the manuscript: H. Broyles, J. Chacko, J. Chancellor, F. LoRusso, P. H. Phillips, A. Mashayekhi, and S. Uwaydat; b. Revising it for intellectual content: H. Broyles, J. Chacko, J. Chancellor, F. LoRusso, P. H. Phillips, A. Mashayekhi, and S. Uwaydat. Category 3: a. Final approval of the completed manuscript: H. Broyles, J. Chacko, J. Chancellor, F. LoRusso, P. H. Phillips, A. Mashayekhi, and S. Uwaydat. 2. 3. 4. 5. REFERENCES 1. 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