Clinical Review & Education
JAMA Ophthalmology Clinical Challenge
More Than Just Multiple Sclerosis Neda Nikpoor, MD; Ninel Gregori, MD; Jonathan S. Chang, MD
Figure 1. Fundus photograph at presentation revealing yellow-white deep retinal lesions throughout the macula and arcades, as well as mild optic nerve edema.
A 51-year-old woman with a history of multiple sclerosis (MS) and 2 previous episodes of optic neuritis in both eyes presented with a 5-day history of photopsias and blurry vision in the left eye. The patient also noted decreased peripheral vision and pain on eye movements of the left eye. She described a recent episode of gastroenteritis prior to the onset of visual complaints. She was taking glatiramer acetate and tizanidine hydrochloride for MS. Her initial visual acuity was 20/50 in both eyes with a Quiz at 1+ relative afferent pupillary defect in the right eye. jamaophthalmology.com The patient had red desaturation in the left eye, and her color vision, which was determined by use of an Ishihara test, was decreased in both eyes. Intraocular pressure and ocular motility were normal, and the results of an external examination, an anterior segment examination of both eyes, and a posterior segment examination of the right eye were unremarkable. A fundus examination of the left eye demonstrated grade 1 disc edema and multiple small to medium yellow-white deep retinal lesions throughout the macula and along the vascular arcades (Figure 1).
jamaophthalmology.com
WHAT WOULD YOU DO NEXT?
A. Observe the patient B. Start steroid therapy C. Perform magnetic resonance imaging D. Order serum titers of IgM and IgG for Bartonella henselae
JAMA Ophthalmology July 2014 Volume 132, Number 7
Copyright 2014 American Medical Association. All rights reserved.
Downloaded From: http://archopht.jamanetwork.com/ by a J H Quillen College User on 05/30/2015
885
Clinical Review & Education JAMA Ophthalmology Clinical Challenge
Diagnosis Multiple evanescent white dot syndrome (MEWDS)
A
B
What To Do Next C. Perform magnetic resonance imaging
Discussion The patient has a history of optic neuritis and MS, with newly decreased peripheral vision and left eye pain on eye movements suggestive of a new episode of optic neuritis. Humphrey visual fields demonstrated severe constriction in both eyes due to previous episodes of optic neuritis. Magnetic resonance imaging of the brain and orbits revealed no new optic nerve lesions. Fundus autofluorescence (Figure 2A) demonstrated scattered areas of hypoautofluorescence in the areas of fundus lesions. There was no foveal granularity. Fluorescein angiography revealed faint early hyperfluorescence and increased late hyperfluorescence of the lesions, with leakage around the optic disc (Figure 2B). Optical coherence tomography revealed normal foveal contour and normal retinal pigment epithelium (RPE). Optical coherence tomography through the fundus lesions revealed hyperreflective outer retinal lesions involving the outer nuclear layer and inner segment/outer segment ellipsoid layer disruption (Figure 2C). Based on the imaging, the fundus examination, and her clinical history, along with a magnetic resonance imaging scan indicating no evidence of active optic neuritis, the diagnosis of MEWDS was made. However, owing to the pain on eye movements and mild disc edema, the neurologist suspected subacute optic neuritis in the left eye and elected to treat with a 3-day course of intravenous steroids followed by oral steroids as per the recommendations of the Optic Neuritis Treatment Trial. After 2 weeks, the patient reported visual improvement and resolution of flashes and pain in the left eye. Her visual acuity had returned to 20/30, her condition with regard to disc edema improved, and the retinal lesions were fading. At 3 months, the patient was asymptomatic, her visual acuity was stable at 20/30, and no retinal lesions were seen. Multiple evanescent white dot syndrome was first described by Jampol et al1 in 1984 as transient unilateral ocular findings of multiple white dots at the level of the RPE or deep retina and granularity of the fovea. Typically, the disease presents acutely in young myopic women as unilateral, painless decreased vision with possible photopsias or dyschromatopsia associated with a viral prodrome in one-third to one-half of patients. Blurring of the optic disc with rela-
C
Figure 2. A, Fundus autofluorescence at presentation shows scattered areas of hypoautofluorescence corresponding to the fundus lesions. B, Late fluorescein angiogram reveals increased hyperfluorescence corresponding to the retinal lesions, which indicates optic disc leakage. C, Spectral-domain optical coherence tomography through the white retinal lesions (arrowheads) reveals outer nuclear layer and ellipsoid layer disruptions.
tive afferent pupillary defect is seen occasionally. Fluorescein angiography typically reveals early hyperfluorescence and late staining of the deep retinal lesions and late leakage around the optic nerve. Indocyanine green angiography reveals late hypofluorescence of the retinal regions. Optical coherence tomography has been reported to reveal outer retinal disturbance with inner segment/outer segment ellipsoid layer disruption and RPE granularity.2 Several unanswered questions remain regarding the etiology, localization, and treatment of MEWDS. The etiology is most likely autoimmune with strong support for association with vaccines,3 HLA-B51,4 and a viral etiology or a viral trigger.5 To our knowledge, there is only one other case report of a patient with concurrent MEWDS and optic neuritis secondary to MS.6 In that case, a 30-year old woman received intravenous methylprednisolone for 3 days followed by oral steroids for 15 days, with resolution of symptoms and signs by 1 month. In summary, we describe an unusual case of MEWDS in a patient with a history of MSrelated optic neuritis and rapid recovery after treatment with systemic steroids.
ARTICLE INFORMATION
REFERENCES
Author Affiliations: Department of Ophthalmology, Bascom Palmer Eye Institute, University of Miami Miller School of Medicine, Miami, Florida (Nikpoor, Gregori, Chang); Miami Veterans Affairs Medical Center, Miami, Florida (Nikpoor, Gregori).
1. 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.
Corresponding Author: Neda Nikpoor, MD, Department of Ophthalmology, Bascom Palmer Eye Institute, University of Miami Miller School of Medicine, 900 NW 17th St, Miami, FL 33136 ([email protected]).
2. Nguyen MH, Witkin AJ, Reichel E, et al. Microstructural abnormalities in MEWDS demonstrated by ultrahigh resolution optical coherence tomography. Retina. 2007;27(4):414-418. 3. Baglivo E, Safran AB, Borruat FX. Multiple evanescent white dot syndrome after hepatitis B vaccine. Am J Ophthalmol. 1996;122(3):431-432.
4. Borruat FX, Herbort CP, Spertini F, Desarnaulds AB. HLA typing in patients with multiple evanescent white dot syndrome (MEWDS). Ocul Immunol Inflamm. 1998;6(1):39-41. 5. Gass JD. Are acute zonal occult outer retinopathy and the white spot syndromes (AZOOR complex) specific autoimmune diseases? Am J Ophthalmol. 2003;135(3):380-381. 6. Querques G, Bux AV, Forte R, Francesco P, Cristiana I, Noci ND. Multiple evanescent white dot syndrome and multiple sclerosis [in French]. J Fr Ophtalmol. 2011;34(4):252-255.
Conflict of Interest Disclosures: None reported.
886
JAMA Ophthalmology July 2014 Volume 132, Number 7
Copyright 2014 American Medical Association. All rights reserved.
Downloaded From: http://archopht.jamanetwork.com/ by a J H Quillen College User on 05/30/2015
jamaophthalmology.com
JAMA Ophthalmology Clinical Challenge
More Than Just Multiple Sclerosis Neda Nikpoor, MD; Ninel Gregori, MD; Jonathan S. Chang, MD
Figure 1. Fundus photograph at presentation revealing yellow-white deep retinal lesions throughout the macula and arcades, as well as mild optic nerve edema.
A 51-year-old woman with a history of multiple sclerosis (MS) and 2 previous episodes of optic neuritis in both eyes presented with a 5-day history of photopsias and blurry vision in the left eye. The patient also noted decreased peripheral vision and pain on eye movements of the left eye. She described a recent episode of gastroenteritis prior to the onset of visual complaints. She was taking glatiramer acetate and tizanidine hydrochloride for MS. Her initial visual acuity was 20/50 in both eyes with a Quiz at 1+ relative afferent pupillary defect in the right eye. jamaophthalmology.com The patient had red desaturation in the left eye, and her color vision, which was determined by use of an Ishihara test, was decreased in both eyes. Intraocular pressure and ocular motility were normal, and the results of an external examination, an anterior segment examination of both eyes, and a posterior segment examination of the right eye were unremarkable. A fundus examination of the left eye demonstrated grade 1 disc edema and multiple small to medium yellow-white deep retinal lesions throughout the macula and along the vascular arcades (Figure 1).
jamaophthalmology.com
WHAT WOULD YOU DO NEXT?
A. Observe the patient B. Start steroid therapy C. Perform magnetic resonance imaging D. Order serum titers of IgM and IgG for Bartonella henselae
JAMA Ophthalmology July 2014 Volume 132, Number 7
Copyright 2014 American Medical Association. All rights reserved.
Downloaded From: http://archopht.jamanetwork.com/ by a J H Quillen College User on 05/30/2015
885
Clinical Review & Education JAMA Ophthalmology Clinical Challenge
Diagnosis Multiple evanescent white dot syndrome (MEWDS)
A
B
What To Do Next C. Perform magnetic resonance imaging
Discussion The patient has a history of optic neuritis and MS, with newly decreased peripheral vision and left eye pain on eye movements suggestive of a new episode of optic neuritis. Humphrey visual fields demonstrated severe constriction in both eyes due to previous episodes of optic neuritis. Magnetic resonance imaging of the brain and orbits revealed no new optic nerve lesions. Fundus autofluorescence (Figure 2A) demonstrated scattered areas of hypoautofluorescence in the areas of fundus lesions. There was no foveal granularity. Fluorescein angiography revealed faint early hyperfluorescence and increased late hyperfluorescence of the lesions, with leakage around the optic disc (Figure 2B). Optical coherence tomography revealed normal foveal contour and normal retinal pigment epithelium (RPE). Optical coherence tomography through the fundus lesions revealed hyperreflective outer retinal lesions involving the outer nuclear layer and inner segment/outer segment ellipsoid layer disruption (Figure 2C). Based on the imaging, the fundus examination, and her clinical history, along with a magnetic resonance imaging scan indicating no evidence of active optic neuritis, the diagnosis of MEWDS was made. However, owing to the pain on eye movements and mild disc edema, the neurologist suspected subacute optic neuritis in the left eye and elected to treat with a 3-day course of intravenous steroids followed by oral steroids as per the recommendations of the Optic Neuritis Treatment Trial. After 2 weeks, the patient reported visual improvement and resolution of flashes and pain in the left eye. Her visual acuity had returned to 20/30, her condition with regard to disc edema improved, and the retinal lesions were fading. At 3 months, the patient was asymptomatic, her visual acuity was stable at 20/30, and no retinal lesions were seen. Multiple evanescent white dot syndrome was first described by Jampol et al1 in 1984 as transient unilateral ocular findings of multiple white dots at the level of the RPE or deep retina and granularity of the fovea. Typically, the disease presents acutely in young myopic women as unilateral, painless decreased vision with possible photopsias or dyschromatopsia associated with a viral prodrome in one-third to one-half of patients. Blurring of the optic disc with rela-
C
Figure 2. A, Fundus autofluorescence at presentation shows scattered areas of hypoautofluorescence corresponding to the fundus lesions. B, Late fluorescein angiogram reveals increased hyperfluorescence corresponding to the retinal lesions, which indicates optic disc leakage. C, Spectral-domain optical coherence tomography through the white retinal lesions (arrowheads) reveals outer nuclear layer and ellipsoid layer disruptions.
tive afferent pupillary defect is seen occasionally. Fluorescein angiography typically reveals early hyperfluorescence and late staining of the deep retinal lesions and late leakage around the optic nerve. Indocyanine green angiography reveals late hypofluorescence of the retinal regions. Optical coherence tomography has been reported to reveal outer retinal disturbance with inner segment/outer segment ellipsoid layer disruption and RPE granularity.2 Several unanswered questions remain regarding the etiology, localization, and treatment of MEWDS. The etiology is most likely autoimmune with strong support for association with vaccines,3 HLA-B51,4 and a viral etiology or a viral trigger.5 To our knowledge, there is only one other case report of a patient with concurrent MEWDS and optic neuritis secondary to MS.6 In that case, a 30-year old woman received intravenous methylprednisolone for 3 days followed by oral steroids for 15 days, with resolution of symptoms and signs by 1 month. In summary, we describe an unusual case of MEWDS in a patient with a history of MSrelated optic neuritis and rapid recovery after treatment with systemic steroids.
ARTICLE INFORMATION
REFERENCES
Author Affiliations: Department of Ophthalmology, Bascom Palmer Eye Institute, University of Miami Miller School of Medicine, Miami, Florida (Nikpoor, Gregori, Chang); Miami Veterans Affairs Medical Center, Miami, Florida (Nikpoor, Gregori).
1. 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.
Corresponding Author: Neda Nikpoor, MD, Department of Ophthalmology, Bascom Palmer Eye Institute, University of Miami Miller School of Medicine, 900 NW 17th St, Miami, FL 33136 ([email protected]).
2. Nguyen MH, Witkin AJ, Reichel E, et al. Microstructural abnormalities in MEWDS demonstrated by ultrahigh resolution optical coherence tomography. Retina. 2007;27(4):414-418. 3. Baglivo E, Safran AB, Borruat FX. Multiple evanescent white dot syndrome after hepatitis B vaccine. Am J Ophthalmol. 1996;122(3):431-432.
4. Borruat FX, Herbort CP, Spertini F, Desarnaulds AB. HLA typing in patients with multiple evanescent white dot syndrome (MEWDS). Ocul Immunol Inflamm. 1998;6(1):39-41. 5. Gass JD. Are acute zonal occult outer retinopathy and the white spot syndromes (AZOOR complex) specific autoimmune diseases? Am J Ophthalmol. 2003;135(3):380-381. 6. Querques G, Bux AV, Forte R, Francesco P, Cristiana I, Noci ND. Multiple evanescent white dot syndrome and multiple sclerosis [in French]. J Fr Ophtalmol. 2011;34(4):252-255.
Conflict of Interest Disclosures: None reported.
886
JAMA Ophthalmology July 2014 Volume 132, Number 7
Copyright 2014 American Medical Association. All rights reserved.
Downloaded From: http://archopht.jamanetwork.com/ by a J H Quillen College User on 05/30/2015
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