An unusual presentation of neurosarcoidosis: Concurrent optic perineuritis and optic neuritis

Abstract

Neurosarcoidosis is a rare complication of sarcoidosis and typically presents as acute cranial neuropathies. Neurosarcoidosis can rarely cause an inflammatory optic neuropathy, resembles an optic neuritis and even more rarely can cause an optic perineuritis. Although concomitant optic neuritis and optic perineuritis have been reported in other inflammatory conditions, such as myelin oligodendrocyte antibody-associated disease, spatially-distinct optic neuritis, and optic perineuritis has not been previously described in neurosarcoidosis. Here, we present a case of spatially-distinct concomitant optic neuritis and optic perineuritis from neurosarcoidosis in a 51-year-old man initially suspected to harbor metastatic disease based on imaging findings.

Keywords: Neurosarcoidosis; optic neuritis; optic perineuritis; sarcoid.

Figure 1

Left: Color fundus photograph of the right eye showing diffuse optic disc edema, vessel obscuration, disc hemorrhages (arrows), and small flame-shaped retinal hemorrhages (arrowheads). Right: Color fundus photograph of the left eye showing a normal-appearing optic nerve and posterior pole

Figure 2

Left, Coronal MPRAGE T1 image of the dural-based mass (arrow) along the superior left tentorial leaflet, measuring 10 x 6 mm. Right, transverse MPRAGE T1 image of the dural-based enhancing lesion (arrow) along the left paramedian falx, measuring 6 mm × 5 mm

Figure 3

Left, coronal T1 postcontrast imaging showing right optic nerve sheath enhancement surrounding the orbital segment of the optic nerve, with mild optic nerve enhancement (arrow). Right, axial T1 postcontrast imaging demonstrating right optic nerve sheath enhancement and the “tram-track” sign (arrow)

Figure 4

Photomicrographs from the subcarinal lymph node fine-needle aspiration. Left, nonnecrotizing granuloma composed of clusters of epithelioid histiocytes with admixed lymphocytes (hematoxylin-eosin, ×400 magnification). Top right, acid-fast bacilli stain is negative for mycobacteria (×200 magnification). Bottom right, Grocott's Methenamine silver stain is negative for fungal organisms (×200 magnification)

Figure 5

Upper left: Color fundus photograph of the right eye (same photograph as in Figure 1, left) showing diffuse optic disc edema, vessel obscuration, disc hemorrhages, and small flame-shaped retinal hemorrhages. Upper right: Color fundus photograph of the right eye 4 months after discharge from the hospital showing interval resolution of the optic disc edema and the subsequent development of optic atrophy. Lower left: Humphrey visual field from the right eye obtained at the initial evaluation showing complete suppression of the visual field. Lower right: Humphrey visual field obtained at his follow-up evaluation, approximately 4 months after his initial evaluation, showing improvement in his visual field defect