Letter to the Editor
Optic perineuritis (OPN) is a clinical entity characterized by painful visual loss and a distinctive neuroimaging sign of optic nerve sheath enhancement (1). It may be a manifestation of a systemic disease as recently reported by McClelland et al (2). OPN may be severe and recurrent and typically requires treatment with systemic corticosteroids. While steroids are thought to be essential in the management of OPN, we recently evaluated a patient with OPN, which resolved completely without treatment.
A 60-year-old African American woman reported a 2-week history of visual loss, severe pain around both eyes, and headache worsened when lying down. She denied nausea, vomiting, photophobia, sonophobia, pulsatile tinnitus, or diplopia. Medical history was significant for well-controlled hypertension and glucose intolerance. Surgical history included cholecystectomy, appendectomy, and hysterectomy. She took only multivitamins, calcium, and omega-3. She denied use of tobacco, alcohol, or recreational drugs.
Visual acuity was 20/25 in both eyes. Examination of the pupils, extraocular movements, and anterior segments was normal. Automated perimetry showed a superior arcuate defect in the right eye, while the left visual field showed generalized depression. Ophthalmoscopy revealed swelling of the right optic disc and a normal left optic disc (Fig. 1).
Orbital magnetic resonance imaging (MRI) demonstrated thin uniform concentric enhancement along the sheath of both optic nerves (Fig. 2). Serum angiotensin-converting enzyme, syphilis serology, and lysozyme measurements were within normal limits as was a chest x-ray. The patient declined a lumbar puncture and a course of systemic corticosteroids.
Six weeks later, without any treatment, her eye pain resolved. Subsequently, her visual fields became normal and she developed mild, bilateral optic disc pallor. Follow-up MRI showed near complete resolution of the bilateral peripheral optic nerve enhancement.
Evaluation of patients with OPN may lead to a specific etiology, but, at times, the cause of the optic neuropathy remains unknown. Documented causes include Wegener granulomatosis (3), sarcoidosis (4), syphilis (5,6), and inflammatory bowel disease (2). Short of comprehensive ophthalmic and systemic evaluations, there is no way to distinguish cases of OPN due to a systemic disorder from those that are idiopathic.
It has been proposed that failure to treat OPN patients with corticosteroids will result in a poor visual outcome (1). Yet, our case suggests that there is a spectrum of severity in OPN, and some patients may experience spontaneous resolution of their optic neuropathy and retain good visual acuity.
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2. McClelland C, Zaveri M, Walsh R, Fleisher J, Galetta S. Optic perineuritis as the presenting feature of Crohn disease. J Neuroophthalmol. 2012;32:345–347.
3. Purvin V, Kawasaki A. Optic perineuritis secondary to Wegener’s granulomatosis. Clin Exp Ophthalmol. 2009;37:712–717.
4. Yu-Wai-Man P, Crompton DE, Graham JY, Black FM, Dayan MR. Optic perineuritis as a rare initial presentation of sarcoidosis. Clin Exp Ophthalmol. 2007;35:682–684.
5. Basta MST, Sankar KN, Dayan M. Unilateral syphilitic perioptic neuritis in a patient coinfected with human immunodeficiency virus type 1. Sex Transm Infect. 2007;83:183–184.
6. Meehan K, Rodman J. Ocular perineuritis secondary to neurosyphilis. Optom Vis Sci. 2010;87:E790–E796.