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A 45-year-old man with unilateral optic disc edema and vision loss
Digital Journal of Ophthalmology 2019
Volume 25, Number 1
March 29, 2019
DOI: 10.5693/djo.03.2019.02.002
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Benjamin G. Jastrzembski, MD | Division of Ophthalmology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, Massachusetts; Department of Ophthalmology, Hospital for Sick Children, University of Toronto, Toronto, Ontario Nurhan Torun, MD | Division of Ophthalmology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, Massachusetts
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| Differential Diagnosis | The initial presentation of a relative afferent pupillary defect, unilateral disc edema, and stellate macular exudates is suggestive of neuroretinitis. Causes of neuroretinitis include cat-scratch disease, toxoplasmosis, typhus, Lyme disease, syphilis, diffuse unilateral subacute neuroretinitis (DUSN), sarcoidosis, and inflammatory bowel disease. It can also be idiopathic.(1) The ocular symptoms are frequently preceded by a viruslike prodrome. The disc edema typically precedes the stellate maculopathy by 1 week. The macular star begins to fade by 4 weeks and is usually completely resolved by one year. Neuroretinitis is well known, but optic disc inflammation and associated retinal exudates may also be seen in other conditions, such as papilledema or hypertensive retinopathy.(2-5)
In this case, the initial findings prompted laboratory work-up for typical autoimmune or infectious causes of neuroretinitis, which was negative. An MRI of the brain was obtained to evaluate for a compressive lesion or evidence of infectious or autoimmune disease of the brain or orbit. The MRI study demonstrated tram track enhancement of the left optic nerve sheath. This finding shifted the differential diagnosis from neuroretinitis to optic perineuritis or optic nerve sheath meningioma. The subsequent clinical and radiological improvement with steroids was diagnostic of optic perineuritis rather than optic nerve sheath meningioma.
Optic perineuritis is characterized by inflammation of the optic nerve sheath and may be idiopathic or secondary to systemic disease, including Behçet disease, Crohn’s disease, granulomatosis with polyangiitis, giant cell arteritis, sarcoidosis, tuberculosis, syphilis, and metastasis.(6-13) The patient’s extensive studies as outlined above did not reveal an etiology. | |
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