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Woman presents with progressive decrease in vision
Examination of the right eye showed optic nerve swelling with disc hemorrhages, fluid extending into the macula, scattered yellowish lesions and peripheral pigmented linear streaks.
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A 48-year-old woman presented to the eye clinic with a progressive decrease in vision in her right eye over the past week. Five days before presentation, she noticed a mild blurring of her right eye, like her glasses were smudged. The next morning, however, she woke up with markedly decreased vision. By the time she came to the office, she also complained of pain with eye movement, photopsias and a headache associated with dull, aching eye pain. She denied any similar previous episodes, recent illnesses, travel, neck stiffness or joint pain.
Her ocular history was unremarkable. Her medical history included insomnia, anxiety and cold sores for which she took Valtrex (valacyclovir hydrochloride, GlaxoSmithKline). She smoked daily, drank alcohol occasionally and worked with inmates. Her family history was unremarkable.
Examination
The patient’s best corrected visual acuity was 20/200 in the right eye and 20/20 in the left eye. Her right pupillary response was sluggish with an afferent pupil defect, and she saw only 8 out of 10 Ishihara color plates on that side. IOP and extraocular movements were normal in both eyes. Humphrey visual field 30-2 testing revealed an increased blind spot in the right eye only. The left eye was normal.
Figure 1. Mosaic color photo of the patient’s right eye demonstrated optic nerve swelling and disc hemorrhages with fluid tracking into the macula, peripheral pigmented linear streaks and multiple white-yellow lesions inferonasally.
Images: Lee G, Baumal C
Figure 2. OCT showed intraretinal and subretinal fluid extending from the optic nerve into the fovea.
Figure 3. OCT through the active lesions revealed collections of material between the RPE and Bruch’s membrane as well as hyperreflectivity of the underlying choroid.
Figure 4. Fundus autofluorescence of the right eye showed hypoautofluorescence of the superotemporal lesions corresponding to the pigmented linear lesions and hyperautofluorescence of the inferonasal lesions corresponding to the clinically more active yellow-white lesions.
Anterior segment exam was unremarkable except for a mild nuclear sclerotic cataract in both eyes. There was no anterior chamber inflammation.
On posterior segment exam, there were 1+ vitreous cells and a few inferiorly located snowballs in the right eye. Fundus exam (Figure 1) demonstrated right optic nerve swelling with disc hemorrhages and fluid extending into the macula. Additionally, there were scattered yellowish lesions inferonasally with various amounts of pigmentary changes and streaks of pigmented spots in the superotemporal periphery running in a circumferential pattern. The left eye was normal.
On Cirrus optical coherence tomography (Carl Zeiss Meditec), there was intraretinal and subretinal fluid extending from the optic nerve to involve the fovea (Figure 2). Sections through the peripheral lesions inferonasally revealed distinct collections of material between the retinal pigment epithelium (RPE) and Bruch’s membrane with underlying choroidal hyperreflectivity (Figure 3). Fluorescein angiography demonstrated no focal areas of leakage, and indocyanine green (ICG) imaging showed blockage in areas of the peripheral lesions. Fundus autofluorescence showed hyperautofluorescent lesions in the areas of the yellowish lesions and hypoautofluorescence around the optic nerve and more pigmented superotemporal lesions (Figure 4).
What is your diagnosis?
Optic neuritis with posterior fundus lesions
The differential diagnosis of optic neuritis with multiple posterior fundus lesions includes infectious entities such as herpes simplex virus, West Nile virus, Epstein-Barr virus, Lyme, toxoplasmosis, Bartonella henselae, syphilis, tuberculosis and ocular histoplasmosis syndrome.
By history, the patient had no definitive exposure to any of the above infectious agents except for tuberculosis (worked with inmates) and herpes simplex (history of cold sores). Herpes viruses typically cause a retinal necrosis and less commonly optic neuritis. West Nile virus may also have a pattern of linear streaks but is less likely associated with vitritis and optic neuritis. In addition, although the retinal findings were most similar to ocular histoplasmosis syndrome (OHS) with the multifocal lesions, vitritis is not usually seen in these patients.
Inflammatory conditions to consider on the differential diagnosis include sarcoidosis and white dot syndromes, particularly multifocal choroiditis and panuveitis (MCP) and punctate inner choroidopathy. An infiltrative entity such as leukemia could potentially present with optic neuritis and multiple lesions, but it is less likely with both acute and chronic lesions, as in this case.
The patient underwent an extensive workup that was negative for syphilis enzyme immunoassay, RPR, ANA, ACE, Lyme, Bartonella henselae and West Nile virus. Her PPD test and chest X-ray were normal. She had positive Epstein-Barr virus and herpes simplex virus IgG titers, which were likely chronic and inactive rather than acute. She also had an MRI with gadolinium that did not demonstrate any enhancing lesions or meningeal involvement. Subsequently, she underwent a lumbar puncture that revealed a normal opening pressure and negative cerebrospinal fluid culture and titers for the above infectious entities.
Given her clinical appearance, the findings on OCT, ICG and autofluorescence, and negative lab work, the patient was diagnosed with MCP.
Discussion
Multifocal choroiditis and panuveitis is a chronic, progressive inflammatory disease typically seen in young myopic women and often results in significant visual loss due to complications, most commonly choroidal neovascularization, involving the posterior pole. Often, it is described as multiple white or yellow lesions at the level of the RPE, which become punched-out appearing over time. The more chronic lesions can also take on a pattern described as linear streaks that run parallel to the equator, as seen in our patient. Linear streaks were once thought to be found only in OHS, but they are found equally in both OHS and MCP and thus cannot be used to distinguish the two. Rather, vitreous inflammation should be used as a distinguishing trait because patients with OHS usually do not have inflammation. Other helpful findings on studies of patients with MCP include blocking at the site of lesions on ICG and hyperautofluorescence in active lesions, which become hypoautofluorescent over time.
Our patient had an interesting presentation with optic nerve involvement causing her vision loss rather than CNV. In the largest report of cases of MCP involving the optic nerve, only 10% to 12% of patients (eight total) were found to have optic neuritis as an ocular finding in their clinical course. In addition, these patients were found to be responsive yet more steroid-dependent than those without optic nerve involvement. Few studies have been done using OCT to monitor or describe the lesions, but our case was consistent with the previous reports describing collections of material between the RPE and Bruch’s membrane with some disruption of the RPE and underlying choroidal hyperreflectivity.
Conclusion
After ruling out infectious etiologies, our patient was started on high-dose oral prednisone with improvement in her ocular pain and headache. One month later, her optic nerve head swelling and macular edema had resolved, but her vision was unchanged. She will continue to be followed in the clinic to monitor control of her inflammation.