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06-14-2012, 06:15 PM
Multiple Evanescent White Dot Syndrome (MEWDS):

24 year-old female with one week duration of central scotoma, OD

Sudeep Pramanik, MD, MBA, Hilary A. Beaver, MD
April 8, 2005, updated April 9, 2007
Chief Complaint: One week duration of central scotoma, OD.
History of Presenting Illness: The patient is an adult white female in her mid-twenties who experienced sudden awareness of a painless, central scotoma in her right eye while reading one week ago. There has been no pain or progression in the last week. There was no known trigger. She reports normal color vision. She denies headache. She denies preceeding viral illness.
Medical History: 1) An MRI six years previously showed enlargement of the pituitary gland with no focal adenoma. Endocrine evaluation showed normal pituitary function. 2) Motor vehicle accident, 1 year previously. No loss of consciousness. Computerized tomography (CT) without contrast was normal at that time. Other history was non-contributory.
Review of systems was otherwise negative except as noted.
Medications: Benzidine for acne. No steroid use.
EXAM OCULAR

Visual Acuity: OD—20/50; OS—20/20
Manifest Refraction: Improved vision to 20/40, OD.
Critical Flicker Fusion: 21.4 +/- 3.8 Hz OD, and 32.6 +/-0.6 Hz OS
Extraocular motility: Full
Pupils: equally reactive, 0.3 log unit afferent pupillary defect (APD), OD.
Intra-ocular pressure: 11 mmHg, OD; 10 mmHg, OS.
External and anterior segment examination: Normal, OU. No cell nor flare.
Dilated fundus exam (DFE): OD—Orange, granular retinal pigment epithelium (RPE) changes in the central macula and multifocal white dots in the outer retina (see Figure 1); OS—Normal fundus (see Figure 2).
Figure 1. Stereo fundus photos, OD. Note the orange, granular changes in the central RPE and multifocal white dot changes. http://webeye.ophth.uiowa.edu/eyeforum/cases-i/case37/MEWDS-fundusOD1.JPGhttp://webeye.ophth.uiowa.edu/eyeforum/cases-i/case37/MEWDS-fundusOD2.JPG
Figure 2. Stereo fundus photos, OS. Essentially normal fundus. http://webeye.ophth.uiowa.edu/eyeforum/cases-i/case37/MEWDSfundusOS1.JPG
http://webeye.ophth.uiowa.edu/eyeforum/cases-i/case37/MEWDSfundusOS2.JPG
Goldman visual field (GVF) testing was performed and revealed a slightly enlarged blind spot and mildly reduced paracentral visual field overall, OD (see Figure 3).
Figure 3.http://webeye.ophth.uiowa.edu/eyeforum/cases-i/case37/MEWDS-GVF-OS.jpghttp://webeye.ophth.uiowa.edu/eyeforum/cases-i/case37/MEWDS-GVF-OD.jpg3A: GVF, OS, is essentially normal. 3B: GVF, OD. Enlarged blind spot with reduction of the I1e isopter centrally.
Discussion

MEWDS (Multiple Evanescent White Dot Syndrome) is a rare, unilateral, self-limiting inflammatory disease that afflicts young women more than men in a 4:1 ratio. Despite the lack of treatment, the prognosis is excellent. Our patient is the typical age and gender. She had no preceeding viral illness, which can be present in approximately 1/3 of MEWDS patients. Visual field testing reveals enlargement of the blind spot, central, and/or paracentral scotomas. Fluorescein angiography of active lesions may be normal early on and demonstrate a "wreath-like" late hyperfluorescence of the white dots. ERG during the active phase may show a reduced a-wave, consistent with inflammation at the level of the photoreceptors. Typically, the visual acuity recovers, but the granularity of the foveal RPE remains.
Diagnosis: MEWDS (Multiple Evanescent White Dot Syndrome)

EPIDEMIOLOGY


Affects young women 20-45 years of age
Typically unilateral (bilateral cases documented)
No known racial or hereditary associations
OCULAR SIGNS


Visual acuity 20/40-20/400
Orange-yellow granularity to the RPE underlying the fovea
Small, transient white dots at the level of the RPE
Small relative afferent pupillary defect possible
Mild vitreous cells
Mild optic disc edema/hyperemia possible
SYMPTOMS


Sudden, painless, monocular decline in central acuity
Photopsias
Dyschromatopsia
Central/paracentral scotomas
Preceeding viral illness in about 1/3 of cases
TREATMENT


None—resolves spontaneously
Most patients achieve normal acuity and fields in weeks to months
Photopsia, scotomas, dyschromatopsia may rarely persist
Recurrence is unusual
No systemic workup is necessary

Differential Diagnoses


Acute Idiopathic Blind Spot Enlargement Syndrome (some feel this may be the same disease)
Acute Posterior Multifocal Placoid Pigment Epigheliopathy
Acute Retinal Pigment Epithelitis
Birdshot Retinochoroidopathy
Multifocal Choroiditis
Sarcoid
Diffuse Unilateral Subacute Neuroretinitis
References


Aaberg TM. Multiple evanescent white dot syndrom., Arch Ophthalmol 1998;106:1162-1163.
Jampol LM, Wiredu A. MEWDS, MFC, PIC, AMN, AIBSE, and AZOOR: one disease or many? Retina 1995;15: 373-378.
Quillen DA, Blodi BA. Ch 9. Inflammatory Diseases. In: Clinical Retina. Chicago: AMA Press. 2002.