Ophthalmic Surgery, Lasers and Imaging Retina

Case Report 

Recurrence of Acute Macular Neuroretinopathy

Amber Hoang, MD; Kay T. Khine, MD; Odette M. Houghton, MD

Abstract

A 31-year-old female with a 2-month history of a central scotoma was diagnosed with acute macular neuroretinopathy (AMNR). Her symptoms resolved spontaneously, only to recur 2 years later with progressively worsening visual field deficits that did not improve with a trial of oral prednisone. The authors report a case of AMNR that is distinguished from other reports by its recurrence in the same eye after complete resolution of the first episode.

[Ophthalmic Surg Lasers Imaging Retina. 2018;49:962–968.]

Abstract

A 31-year-old female with a 2-month history of a central scotoma was diagnosed with acute macular neuroretinopathy (AMNR). Her symptoms resolved spontaneously, only to recur 2 years later with progressively worsening visual field deficits that did not improve with a trial of oral prednisone. The authors report a case of AMNR that is distinguished from other reports by its recurrence in the same eye after complete resolution of the first episode.

[Ophthalmic Surg Lasers Imaging Retina. 2018;49:962–968.]

Introduction

Acute macular neuroretinopathy (AMNR) is a rare condition associated with sudden central vision loss, photopsia, and macular lesions with corresponding visual field deficits.1

AMNR can be diagnosed by visualizing the characteristic macular lesions. These lesions are not always apparent on fundus examination. Recent reports suggest that infrared-imaging and optical coherence tomography (OCT) may be superior in detecting small lesions not evident with colored or red-free photography.2–6 Since the lesions are believed to be in the outer retina, multifocal electroretinography may reveal abnormality in the bipolar7 and / or photoreceptor layer.8 Fluorescein angiography usually remains normal.1,9,10

Case Report

A 31-year-old white female with hypothyroidism and myopia presented with a 2-month history of scotoma in the right eye (OD). Her symptoms included photopsias and floaters in both eyes (OU). She denied pain, recent febrile illness, trauma or use of oral contraceptives. Visual acuity (VA) was 20/15 OD and 20/30 in the left eye (OS). There was no afferent pupil defect. Biomicroscopy was unremarkable. The maculae appeared normal (Figure 1). Infrared radiance (IR) OD revealed multiple patches of decreased reflectance (Figure 2A) corresponding to areas of outer segment / retinal pigment epithelium (OS/RPE) interface disruption, with overlying inner segment / outer segment junction attenuation (IS/OS) (Figure 2B). Autofluorescent images were normal OU. Visual field (VF) revealed an enlarged blind spot OD (Figure 6). It was felt that her findings were most consistent with AMNR and she received no treatment. Multifocal electroretinogram (mfERG) was normal.

Dilated fundus exam revealed normal-appearing vitreous and vessels of both eyes. The optic nerve in the left eye was tilted inferiorly and there was a large scleral crescent in both eyes. The macula had a normal appearance in both eyes.

Figure 1.

Dilated fundus exam revealed normal-appearing vitreous and vessels of both eyes. The optic nerve in the left eye was tilted inferiorly and there was a large scleral crescent in both eyes. The macula had a normal appearance in both eyes.

Infrared radiance (IR) and optical coherence tomography (OCT) images on initial presentation. IR in the right eye revealed multiple focal patches of oval-shaped decreased reflectance scattered circumferentially in the paramacular region (A). These lesions were more numerous in the superior macula (arrows). The OCT (B) showed normal foveal contour and inner retinal layers. There were multiple 50 μm to 100 μm focal areas of disruption of the outer segment / retinal pigment epithelium (OS/RPE) interface with overlying inner segment / outer segment (IS/OS) junction attenuation. The focal areas of OS/RPE interface disruption corresponded to the areas of reduced reflectance in the IR image (star). The IR (C) and OCT of the left eye were normal (D).

Figure 2.

Infrared radiance (IR) and optical coherence tomography (OCT) images on initial presentation. IR in the right eye revealed multiple focal patches of oval-shaped decreased reflectance scattered circumferentially in the paramacular region (A). These lesions were more numerous in the superior macula (arrows). The OCT (B) showed normal foveal contour and inner retinal layers. There were multiple 50 μm to 100 μm focal areas of disruption of the outer segment / retinal pigment epithelium (OS/RPE) interface with overlying inner segment / outer segment (IS/OS) junction attenuation. The focal areas of OS/RPE interface disruption corresponded to the areas of reduced reflectance in the IR image (star). The IR (C) and OCT of the left eye were normal (D).

Two years later, infrared radiance (IR) in the right eye (OD) revealed multiple oval-shaped focal patches of decreased reflectance scattered circumferentially in the paramacular region (A, C). These lesions were more numerous in the superior macula and were in different location from areas of decreased reflectance during her initial presentation (Figure 2). Optical coherence tomography (B, D) showed normal foveal contour and inner retinal layers. There were multiple 50 μm to 100 μm focal areas of disruption of the outer segment / retinal pigment epithelium interface with overlying inner segment / outer segment junction attenuation, corresponding to areas of reduced reflectance in the IR image (star and circle). These lesions were located in areas that were affected during her first presentation.

Figure 3.

Two years later, infrared radiance (IR) in the right eye (OD) revealed multiple oval-shaped focal patches of decreased reflectance scattered circumferentially in the paramacular region (A, C). These lesions were more numerous in the superior macula and were in different location from areas of decreased reflectance during her initial presentation (Figure 2). Optical coherence tomography (B, D) showed normal foveal contour and inner retinal layers. There were multiple 50 μm to 100 μm focal areas of disruption of the outer segment / retinal pigment epithelium interface with overlying inner segment / outer segment junction attenuation, corresponding to areas of reduced reflectance in the IR image (star and circle). These lesions were located in areas that were affected during her first presentation.

One month following the 2-year follow-up, the lesions seen on infrared radiance (IR) (A, C) were smaller in size and less numerous on IR image 3A and 3B. However, there were persistent outer segment / retinal pigment epithelium interface defects (star and circle) (B, D).

Figure 4.

One month following the 2-year follow-up, the lesions seen on infrared radiance (IR) (A, C) were smaller in size and less numerous on IR image 3A and 3B. However, there were persistent outer segment / retinal pigment epithelium interface defects (star and circle) (B, D).

Seven months following the 2-year follow-up, the infrared radiance lesions have resolved (A, B). Many of the outer segment / retinal pigment epithelium interface defects had improved (B) with residual mild disruption of the interface superiorly to the macula (star) (D).

Figure 5.

Seven months following the 2-year follow-up, the infrared radiance lesions have resolved (A, B). Many of the outer segment / retinal pigment epithelium interface defects had improved (B) with residual mild disruption of the interface superiorly to the macula (star) (D).

The 10-2 visual field revealed a subtle enlargement of the blind spot in the right eye (A) and was normal in the left eye (B).

Figure 6.

The 10-2 visual field revealed a subtle enlargement of the blind spot in the right eye (A) and was normal in the left eye (B).

Two years later, the patient returned with recurrent symptoms. She reported that the blind spot OD appeared worse compared to her initial episode. She noted that her symptoms from presentation 2 years ago had completely resolved only to recur 8 months later.

Her ocular exam was unchanged. IR and OCT revealed recurrence of the lesions OD (Figure 3). These lesions were located differently from her initial presentation (Figure 2). VF revealed a temporal scotoma OD (Figure 7). The patient was prescribed a tapering dose of oral prednisone.

Repeat visual field 2 years later revealed a moderately dense temporal scotoma in the right eye (A), whereas the left eye was normal (B).

Figure 7.

Repeat visual field 2 years later revealed a moderately dense temporal scotoma in the right eye (A), whereas the left eye was normal (B).

There was no improvement in her symptoms after 1 month. Although the lesions seen on IR were smaller and less numerous, there were persistent OS/RPE interface defects (Figure 4). The VF revealed a persistent temporal scotoma (Figure 8).

One month following the 2-year follow-up, the 10-2 visual field (VF) revealed a persistent temporal scotoma in the right eye (A). The VF was unchanged in the left eye (B).

Figure 8.

One month following the 2-year follow-up, the 10-2 visual field (VF) revealed a persistent temporal scotoma in the right eye (A). The VF was unchanged in the left eye (B).

On the last follow-up visit 7 months later, she had improvement but not resolution of her symptoms and VF defect (Figure 9). Many of the OS/RPE interface defects had improved, with residual disruption of the interface superior to the macula (Figure 5).

Seven months following the 2-year follow-up, the temporal visual field (VF) defect in the right eye persisted (A). The VF remained unchanged in the left eye (B).

Figure 9.

Seven months following the 2-year follow-up, the temporal visual field (VF) defect in the right eye persisted (A). The VF remained unchanged in the left eye (B).

Discussion

Several authors have demonstrated that one of the earliest changes in AMNR lesions on the OCT image is hyperreflectivity at the junction of the outer nuclear layer (ONL) and outer plexiform layer (OPL) or the inner nuclear layer (INL) and OPL. As the lesions resolve, they may evolve into ONL thinning associated with OS/RPE disruption or INL atrophy.2

Our patient's diagnosis was consistent with AMNR based on IR and OCT imaging. Follow-up imaging revealed resolution of the lesions on the infrared imaging. However, there was persistent disruption in the OS/RPE interface in the area of the lesions.

In our case, neither INL, ONL, nor OPL hyperreflectivity was appreciated with either episode. Most likely, our patient presented after this acute finding had resolved. It is also possible that this characteristic is more difficult to detect in smaller lesions.2 OCT images taken during our patient's initial presentation and during recurrence demonstrated focal patches of OS/IS junction disturbance with underlying OS/RPE interface disruption noted (Figure 1). Although OS/RPE interface disruption persisted, no ONL thinning was consequent to this initial episode.

Interestingly, the lesions appeared to recur in a different location for each episode that images were obtained. On OCT, there appeared to be complete resolution of the IS/OS junction and subtle residual OS/RPE interface disruption without notable thinning of the ONL during the recurrence.

Despite the resolution of the lesions detected on infrared and restoration of the IS/OS interface, the patient's symptoms persisted. This was reflected in the visual field defect, which revealed an improved, but persistent temporal field defect after 7 months of follow up (Figure 9).

AMNR has a variable prognosis. It may be self-limited without recurrence. However, persistent lesions, sometimes with progressive worsening of the scotoma, have been documented.2,7,8 Our patient reports three episodes of symptoms in the same eye. Although her symptoms improved following the first two episodes, 6-month follow-up from her third episode revealed unchanged symptoms with a persistent scotoma on VF. To the best of our knowledge, this report is the first case of recurrence that is documented with spectral-domain OCT. OCT revealed persistence of some of the first and third episode's lesions manifest as disruptions in the OS/RPE line. We were also able to demonstrate that the VF defect was persistent despite improvement in the OCT findings with restoration of the IS/OS junction.

A literature search revealed only one other report of recurrence associated with AMNR in the same eye.11 Matsuo et al. reported on two patients with recurrence.11 One patient had development of a second AMNR lesion with corresponding scotoma in the same eye 1 year later. The other patient had a similar lesion develop in the contralateral eye 2 months later. In both patients, the lesions remained stable with persistent scotomas in a 3-year and 2-year follow-up respectively.11 Unfortunately, since time-domain OCT was used to capture the recurrences, defects of the OPL, INL, IS/OS junction, or OS/RPE junction were not documented.

AMNR appears to predominantly affect women in their reproductive years. Several associations previously described include prior viral infection, use of oral contraceptives, cold medications, epinephrine, contrast media, and trauma.11–13 The mechanism of injury in AMNR is unknown. However, based on these associations immunological and ischemic etiologies have been proposed.4,12

Although our patient's gender and age are appropriate for this diagnosis, she denies any of the other factors commonly associated with AMNR. Our patient does have hypothyroidism, a positive antinuclear antibody titer, and a family history of autoimmune disorder that may point to an inflammatory etiology in our patient. The recurrence of our patient's lesions further supports the theory of an underlying inflammatory or vasculitic process.

Currently, there is no known treatment for AMNR. There is a report in which a patient had reductions of scotomas while on a 4-month trial of corticosteroids.14 Unfortunately, our patient did not enjoy any improvement following a trial of oral prednisone.

References

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Authors

From Wake Forest University, Department of Ophthalmology, Winston-Salem, North Carolina (AH); Bascom Palmer Eye Institute, University of Miami, Miami (KTK); and Mayo Clinic, Department of Ophthalmology, Scottsdale, Arizona (OMH).

The authors report no relevant financial disclosures.

Address correspondence to Odette M. Houghton, MD, Department of Ophthalmology, Mayo Clinic, 13400 East Shea Boulevard, Scottsdale, AZ 85259; email: houghton.odette@mayo.edu.

Received: April 10, 2018
Accepted: November 02, 2018

10.3928/23258160-20181203-09

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