Spotlight Case: The Case of the Woman With Blurry Vision for 1 Year and Difficulty With Low-Contrast Targets

  • author profile pictureShlomit Schaal, MD, PhD
  • author profile pictureDouglas Sigford, MD

Case history

A 57-year-old Caucasian woman presented with a complaint of blurry vision in both eyes for 1 year. She also noted more difficulty with low-contrast targets as well as photopsias OU; onset was insidious. Her medical history was significant for hypothyroidism of unknown etiology, for which she was taking levothyroxine, and for mitral valve prolapse. She noted submandibular lymphadenopathy around the time her symptoms began. She had no ocular history, and her family history was significant only for macular degeneration in a maternal grandfather.

Best-corrected visual acuity (BCVA) measured 20/30-2 OD (-1.25+1.50x180) and 20/50+2 OS (-0.25 sphere). Pupils, extraocular movements, gross confrontational visual fields, and intraocular pressures were normal. Her anterior segment was unremarkable, and she had no vitritis OU. Dilated funduscopy revealed small (

OCT showed a diffuse, granular disruption of the ellipsoid region of the photoreceptor outer segments OU. This was more prominent subfoveally, where there was a loss of distinction between the external limiting membrane (ELM) and the ellipsoid region (Figure 2). Fundus autofluorescence (FAF) also showed a granular pattern of disruption (Figure 3).

Figure 1: Color fundus photographs of both eyes show small (

Figure 2: SD-OCT images of both eyes show granular disruption of the ellipsoid region of the photoreceptors as well as some loss of differentiation of the photoreceptor structures near the fovea (white arrowheads).

Figure 3: Fundus blue autofluorescence pictures show a mild increase in the normal granular variation of hypoautofluorescence.

Fluorescein angiography (FA) and indocyanine green angiography (ICGA) showed early hypofluorescent patches, which were much more prominent in ICG. These hypofluorescent areas on ICG remained present throughout the angiogram in both the right and left eyes (Figures 4 and 5). Full-field electroretinography (ERG) showed decreased b-waves OU (Figure 6), and multifocal ERG showed decreased first-order response amplitudes (Figure 7) as well as prolonged implicit times OU (Figure 8).

Figure 4: Left eye—The late-AV phase of the FA (left) and ICGA (right) demonstrate hypofluorescent spots that are much more apparent in the ICGA.

Figure 5: Right eye—The late-venous phase of the FA (left) and ICGA (right) show similar hypofluorescent spots, again much more prominent using ICGA.

 
What’s your diagnosis?

The patient was diagnosed with probable subacute multiple evanescent white dot syndrome (MEWDS). Although the presentation was not that of classic MEWDS, the patient's condition may be an alternate phenotype of the same disease or a related entity within the disease spectrum.

Figure 6: Full-field ERG shows decreased b-wave amplitudes OU to stimuli presented in both the dark-adapted rod ERG and photopic cone ERG.

Figure 7: Multifocal ERG with decreased first-order responses OU indicates severe cone photoreceptor dysfunction in the fovea, parafovea, and periphery.

Figure 8: Multifocal ERG also showed prolonged P1 implicit times OU.

Discussion

MEWDS is one of the so-called white dot syndromes and is typically seen in women ages 20-50.[1] This condition, first described in 1984 by Jampol et al, is characterized by multiple small, grey/white lesions at the level of the retinal pigment epithelium (RPE) and outer retina.[2] Additionally, the fovea typically has a granular appearance that may persist after the acute disease process has passed.

MEWDS is more commonly seen in moderate myopes, and half of the patients may have a viral prodrome.[1] Symptoms include blurred vision, photopsias, and central or paracentral scotomas.[2] The prognosis is good, with most patients recovering vision within 3 months.[2] Recurrence is possible but is very uncommon.[3]

The classic FA feature of MEWDS is a wreath-like arrangement of punctate hyperfluorescence surrounding the fovea, while ICGA shows more numerous hypofluorescent spots than on FA.[2] Spectral-domain OCT (SD-OCT) may demonstrate a disruption of the ellipsoid region of the photoreceptors and RPE perturbation.[4]

The etiology of MEWDS remains unclear. It has been proposed that autoantigens targeting the photoreceptors, RPE, or choroid may develop as a result of antibody production against an unknown pathogen.[5,6]

Because there is overlap in the clinical features of MEWDS, acute idiopathic blind spot enlargement syndrome (AIBSE), acute zonal occult outer retinopathy (AZOOR), and multifocal choroiditis (MFC), these entities are sometimes thought to belong to a spectrum of disorders affecting the choroid, RPE, and outer retina.[7] They may show up in the same patient separately[8,9,10] or simultaneously,[11] which raises questions about the etiology and disease spectrum. The patient in this case showed ICG and OCT findings compatible with a bilateral choroidal and outer retina dysfunction which likely fits into this spectrum of diseases.
 

Take-home points

1. While MEWDS is typically a unilateral disease, it may present bilaterally or recur.

2. MEWDS may have a normal or near-normal FA, but ICG defects may persist. OCT changes, including outer retina disturbances, may also persist.

3. MEWDS is of unknown etiology, but it is thought to represent a phenotype within a spectrum of similar related diseases characterized by variable damage to the choroid, RPE, and outer retina.
 

References

1. Crawford CM, Igboeli O. A review of the inflammatory chorioretinopathies: the white dot syndromes [published online October 31, 2013]. ISRN Inflamm. doi:10.1155/2013/783190.

2. Jampol LM, Sieving PA, Pugh D, Fishman GA, Gilbert H. Multiple evanescent white dot syndrome. I. clinical findings. Arch Ophthalmol. 1984;102(5):671-674. doi:10.1001/archopht.1984.01040030527008.

3. Tsai L, Jampol LM, Pollock SC, Olk J. Chronic recurrent multiple evanescent white dot syndrome. Retina. 1994;14(2):160-163.

4. Silva RA, Albini TA, Flynn HW Jr. Multiple evanescent white dot syndromes [published online November 26, 2011]. J Ophthalmic Inflamm Infect. 2012;2(2):109-111. doi:10.1007/s12348-011-0051-9.

5. Jampol LM, Becker KG. White spot syndromes of the retina: a hypothesis based on the common genetic hypothesis of autoimmune/inflammatory disease. Am J Ophthalmol. 2003;135(3):376-379.

6. Heckenlively JR, Ferreyra HA. Autoimmune retinopathy: a review and summary [published online April 12, 2008]. Semin Immunopathol. 2008;30(2):127-134. doi:10.1007/s00281-008-0114-7.

7. Gass JD, Agarwal A, Scott IU. Acute zonal occult outer retinopathy: a long-term follow-up study. Am J Ophthalmol. 2002;134(3):329-339.

8. Bryan RG, Freund KB, Yannuzzi LA, Spaide RF, Huang SJ, Costa DL. Multiple evanescent white dot syndrome in patients with multifocal choroiditis. Retina. 2002;22(3):317-322.

9. Callanan D, Gass JD. Multifocal choroiditis and choroidal neovascularization associated with the multiple evanescent white dot and acute idiopathic blind spot enlargement syndrome. Ophthalmol. 1992;99(11):1678-1685.

10. Fine HF, Spaide RF, Ryan EH Jr, Matsumoto Y, Yannuzzi LA. Acute zonal occult outer retinopathy in patients with multiple evanescent white dot syndrome. Arch Ophthalmol. 2009;127(1):66-70. doi:10.1001/archophthalmol.2008.530.

11. Schaal S, Schiff WM, Kaplan HJ, Tezel TH. Simultaneous appearance of multiple evanescent white dot syndrome and multifocal choroiditis indicate a common causal relationship. Ocul Immunol Inflamm. 2009;17(5):325-327. doi:10.3109/09273940903043923.

Fincancial disclosures

Dr. Schaal - None.

Dr. Sigford - None.

Dr. Hau - SEQUENOM: Speaker, Honoraria; THROMBOGENICS, INC: Other, Honoraria.

Dr. Choudhry
- None.

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