Case of the Month

				Using the search form

If you prefer to view this article in PDF form, click here.

Amber G, Harper, BS; Melissa D. Lilly, BS, MSPA, PA-C

Amber Harper is a second-year student in the Mountain State University Physician Assistant Program, Beckley, West Virginia. Melissa Lilly is a Clinical Coordinator/Instructor in the program and a physician assistant at BARH, Beckley, West Virginia. The authors have indicated no relationships to disclose relating to the content of this article.

CASE

A 28-year-old white female presented to her primary-care provider complaining of flashes in her right eye that had started after a flulike illness and a tension headache that had lasted for 2 weeks. The flashes began in the peripheral field and evolved into a constant, shimmering gray blur. The visual defect hindered the patient's visual acuity, night vision, and ability to focus on the page when reading.

HISTORY The patient described her vision as being cloudy with holes of clarity, as if she was looking through a filter. She had no history of pain or trauma and no family history of visual defects. Cloudiness in the right eye was noted during a routine eye examination two months before this episode, but no action was taken. She was taking OTC migraine medication for tension headaches, sertraline for anxiety, budesonide intranasal for allergies, and ethinyl estradiol plus norgestimate for birth control. She reported no other medical problems.

PHYSICAL EXAMINATION The patient's visual perception was 20/40^-1 in the right eye and 20/20 in the left eye while wearing eyeglasses. In her right eye, the pupil had a decreased response to stimulation with light and the peripheral field was diminished at the temporal aspect. Fundoscopic examination revealed mild papilledema and indistinctive margins around the optic disc. The patient was referred to an ophthalmologist.

The ophthalmologic examination revealed centrocecal scotoma, or a central vision loss extending to the blind spot; decreased color vision; and loss of visual acuity. A fundoscopic examination of the right eye revealed white, flattened spots on the retinal pigment epithelium with orange granularity of the macula (see Figure 1). No abnormalities were found in the left eye. A Humphrey visual field test revealed an enlarged blind spot and reduced visual field in the right eye. Slit-lamp biomicroscopy results were negative for both eyes.

WHAT IS YOUR DIAGNOSIS?

Retinal detachment
Acute posterior multifocal placoid pigment epithiliopathy (APMPPE)
Multiple evanescent white dot syndrome (MEWDS)
Central retinal vein occlusion

DISCUSSION

The correct diagnosis is multiple evanescent white dot syndrome. The patient was referred to a retinal specialist for confirmation of the diagnosis. MEWDS is a rare disease that predominantly affects women aged 15 to 40 years.¹ It manifests as an acute, multifocal infection of the retina that is concentrated in the posterior pole of the eye with granularity of the macula.² Most patients who develop MEWDS are nearsighted and therefore have a weakened retina that is more prone to infection.³ Patients may complain of a sudden, painless, monocular visual disturbance with flashes; color vision disturbances; and centrocecal scotoma.⁴ Symptoms usually appear after a viral illness, such as an upper respiratory infection,¹ or a recent vaccination.^5,6

On examination, visual acuity ranges from 20/40 to 20/400. Multifocal white dots and photoreceptor cells can be seen on fundoscopic examination.⁷ The macula may appear orange and granular. The optic disc may have mild papilledema and undistinguishable margins.

TREATMENT MEWDS is a self-limiting disease, and no medication or treatment is needed. The disease resolves in 3 weeks to 6 months. Granularity of the fovea remains even after visual acuity resolves. In rare instances, scarring affects the visual acuity in the effected eye. MEWDS can recur, usually in the opposite eye.¹

COMMENTS The presentation of MEWDS often is puzzling and clinicians react with emergency protocol to save the patient's vision. When considering MEWDS in the differential diagnosis, the patient should be referred promptly to an ophthalmologist to confirm the diagnosis. Follow-up examinations should continue until the patient's visual acuity returns to normal.

Erich Fogg, PA-C, MMSc, department editor

REFERENCES

	1.	Jampol LM, Sieving PA, Pugh D, et al. Multiple evanescent white dot syndrome. I. Clinical findings. Arch Ophthalmol. 1984;102(5):671-674.
	2.	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.
	3.	Asano T, Kondo M, Kondo N, et al. High prevalence of myopia in Japanese patients with multiple evanescent white dot syndrome. Jpn J Ophthalmol. 2004;48(5):486-489.
	4.	Lombardo J. Multiple evanescent white dot syndrome and acute zonal occult outer retinopathies. Optom Vis Sci. 2003;80(10):673-680.
	5.	Stangos A, Zaninetti M, Petropoulos I, et al. Multiple evanescent white dot syndrome following simultaneous hepatitis-A and yellow fever vaccination. Ocul Immunol Inflamm. 2006;14(5):301-304.
	6.	Fine L, Fine A, Cunningham ET Jr. Multiple evanescent white dot syndrome following hepatitis a vaccination. Arch Ophthalmol. 2001;119(12):1856-1858.
	7.	Ikeda N, Ikeda T, Nagata M, et al. Location of lesions in multiple evanescent white dot syndrome and the cause of the hypofluorescent spots observed by indocyanine green angiography. Graefes Arch Clin Exp Ophthalmol. 2001;239(3):242-247.