Isolated Optic Perineuritis as the Presenting Sign of Sarcoidosis

A 35-year-old healthy African-American woman presented with a 4-month history of gradual loss of vision in the right eye from an optic neuropathy. MRI of the orbits with gadolinium showed isolated thickening and enhancement of the right optic nerve sheath. Chest x-ray and CT-scan of the chest were performed and showed bilateral hilar and mediastinal lymphadenopathy. This was suggestive of sarcoidosis, and the diagnosis was confirmed with histopathology. The patient promptly recovered vision with high-dose corticosteroids; the thickening of the optic nerve sheath also regressed. Isolated optic nerve sheath thickening from sarcoidosis is rare and may mimic compressive optic neuropathies such as optic nerve sheath meningiomas. A systemic evaluation for systemic inflammatory etiologies should be considered in such cases.

Photo Essay Section Editors: Melissa W. Ko, MD Dean M. Cestari, MD Peter Quiros, MD Isolated Optic Perineuritis as the Presenting Sign of Sarcoidosis Jonathan A. Micieli, MD, CM, Catherine J. Streutker, MD, Kieran McIntyre, MD FIG. 1. Optic disc photographs showing normal appearing optic nerves in both eyes (A) and Humphrey 24-2 SITA-Fast visual fields (B) demonstrating generalized depression in the right eye. Axial (D) and coronal (E) MRI of the orbits with fat suppression and postgadolinium showed circumferential thickening of the right optic nerve sheath (white arrows) and to a much lesser extent the left optic nerve sheath. After treatment with intravenous corticosteroids, the visual field defect (C) and optic nerve sheath enhancement (F) resolved. Abstract: A 35-year-old healthy African-American woman presented with a 4-month history of gradual loss of vision in the right eye from an optic neuropathy. MRI of the orbits with gadolinium showed isolated thickening and enhancement of the right optic nerve sheath. Chest x-ray and CT-scan of the chest were performed and showed bilateral hilar and mediastinal lymphadenopathy. This was suggestive of sarcoidosis, and the diagnosis was confirmed with histopathology. The patient promptly recovered vision with high-dose corticosteroids; the Division of Neurology, Department of Medicine, Department of Ophthalmology and Vision Sciences (JAM), University of Toronto, Toronto, Canada; Kensington Vision and Research Centre (JAM), Toronto, Canada; Department of Ophthalmology (JAM), St. Michael's Hospital, Toronto, Canada; Department of Laboratory Medicine (CJS), St. Michael's Hospital, Toronto, Canada; and Division of Respirology (KM), Department of Medicine, University of Toronto, St. Michael's Hospital, Toronto, Canada. The authors report no conflicts of interest. Address correspondence to Jonathan A. Micieli, MD, CM, Kensington Vision and Research Centre, 340 College Street, Suite 501, Toronto, ON M5T 3A9, Canada; E-mail: [e-mail] Micieli et al: J Neuro-Ophthalmol 2020; 40: 255-257 thickening of the optic nerve sheath also regressed. Isolated optic nerve sheath thickening from sarcoidosis is rare and may mimic compressive optic neuropathies such as optic nerve sheath meningiomas. A systemic evaluation for systemic inflammatory etiologies should be considered in such cases. Journal of Neuro-Ophthalmology 2020;40:255-257 doi: 10.1097/WNO.0000000000000822 © 2019 by North American Neuro-Ophthalmology Society A 35-year-old healthy African-American woman presented with a 4-month history of gradual loss of vision in the right eye. There was no pain or pain with eye movements. Her visual acuities were counting fingers at 1 foot in the right eye and 20/20 in the left eye. There was a right afferent pupillary defect, and both optic nerves appeared normal (Fig. 1A). She had normal ocular motility and alignment; there was no proptosis. Humphrey 24-2 SITA-Fast visual fields showed generalized depression in the right eye with 255 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Photo Essay FIG. 2. Chest x-ray (A) and CT scan of the chest (B) demonstrating hilar and mediastinal lymphadenopathy (white thick and thin arrows). Pathology of fine needle core biopsies of mediastinal lymph nodes showed noncaseating granulomas in a background of benign lymphoid cells. Special stains for acid fast bacteria and fungi (ZN and PAS-diastase, not shown) were negative for infectious organisms (C). a mean deviation of 224.75 dB and was normal in the left eye (Fig. 1B). MRI of the brain and orbits with gadolinium revealed circumferential right optic nerve sheath enhancement without optic nerve enhancement; these findings were present to a much lesser extent on the left side (Fig. 1D, E). There were no other changes in the brain. The differential diagnosis included an inflammatory optic perineuritis or less likely an optic nerve sheath meningioma. As part of the workup for systemic inflammatory disease, a chest x-ray and CT-scan of the chest were performed; these showed bilateral hilar and mediastinal lymphadenopathy suggestive of sarcoidosis (Fig. 2A, B). Review of systems revealed no pulmonary symptoms. Fine needle aspirates taken through Olympus endobronchial ultrasound of the mediastinal lymph nodes showed noncaseating granulatomas, consistent with sarcoidosis (Fig. 2C). She was treated with intravenous methylprednisolone 1 g daily for 5 days followed by a slow oral prednisone taper. Her visual acuities returned to 20/20 with complete resolution of the visual field defect after 5 days (Fig. 1C). Repeat MRI after her vision returned to normal showed a decreased thickness of the optic nerve sheath (Fig. 1F). Optic perineuritis refers to an uncommon inflammatory disorder primarily affecting the optic nerves sheath and may be related to systemic granulomatous disease. Approximately, 5%-10% of patients with sarcoidosis have neurological involvement, and the optic nerve is the second most frequently involved cranial nerve after the facial nerve (1). 256 Isolated optic nerve sheath enhancement without optic nerve involvement is rare, and we found 6 such cases in the literature (2-6). Three of these cases were not associated with pain, and 2 patients had a chronic course; however, both the absence of pain and a chronic disease course have not been reported together. Our case is unique in that our patient had both slowly progressive vision loss and absence of pain. This combination was not seen in the previously reported cases and suggests that compression of the optic nerve by the thickened optic nerve sheath was the primary mechanism of vision loss. Sarcoidosis is well known to mimic an optic nerve sheath meningioma or other intraorbital tumors (5). Treatment with corticosteroids likely resulted in resolution of the compression and a rapid improvement in visual symptoms, although an additional inflammatory component was also possible. Also, unique to our case was the available repeat MRI that confirmed the reduced thickness of the optic nerve sheath at the time of visual recovery, providing a clinico-radiological correlation. Sarcoidosis should be considered in the differential diagnosis of optic nerve sheath thickening or enhancement, even in the absence of pain and in the presence of chronic visual symptoms. STATEMENT OF AUTHORSHIP Category 1: a. Conception and design: J. A. Micieli, C. J. Streutker, and K. McIntyre; b. Acquisition of data: J. A. Micieli, C. J. Streutker, and K. McIntyre; c. Analysis and interpretation of data: J. A. Micieli, C. J. Streutker, and K. McIntyre. Category 2: a. Drafting the Micieli et al: J Neuro-Ophthalmol 2020; 40: 255-257 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Photo Essay manuscript: J. A. Micieli; b. Revising it for intellectual content: C. J. Streutker and K. McIntyre. Category 3: a. Final approval of the completed manuscript: J. A. Micieli, C. J. Streutker, and K. McIntyre. REFERENCES 1. Stern BJ, Krumholz A, Johns C. Sarcoidosis and its neurological manifestations. Arch Neurol. 1985;42:909-917. 2. Kidd DP, Burton BJ, Graham EM, Plant GT. Optic neuropathy associated with systemic sarcoidosis. Neurol Neuroimmunol Neuroinflamm. 2016;3:e270. Micieli et al: J Neuro-Ophthalmol 2020; 40: 255-257 3. 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. 4. Jennings JW, Rojiani AM, Brem SS, Murtagh R. 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