In May 2012, a 22-year-old man presented to our clinic with a 2-month history of a visual field (VF) defect over the temporal side of his right eye. He did not have a history of systemic disease. At the first ophthalmic examination, the best corrected visual acuity (BCVA) of each eye was 20/20. Both pupils were 6 mm, round, and reactive to light without a relative afferent pupillary defect. The intraocular pressure was 11 mmHg in the right eye and 8 mm Hg in the left eye. There was no anterior uveitis or conjunctival granulomas in either eye. Funduscopic examination revealed bilateral swelling of the optic discs, a grayish-white mass in the superotemporal peripapillary region between the disc and fovea, and hard exudates around the fovea in the right eye (Fig. 1). Fluorescein angiography (FA) revealed optic disc staining in both eyes (Fig. 2). Automated static perimetry revealed a substantially enlarged blind spot of the right eye, which was compatible with the clinical symptom and fundus examination (Fig. 3).
The complete blood cell count and the serum biochemical profile were unremarkable, and a blood culture for bacterial and fungal pathogens was negative. Acid-fast stain and tuberculosis sputum culture were also negative. The serology test results were negative for human leukocyte antigen B27, antinuclear antibody, rheumatoid factor, reactive plasma reagin, human immunodeficiency virus antibodies, and toxoplasmosis antibodies. Immuno-globulin M antibody levels for cytomegalovirus herpes simplex virus, and varicella-zoster virus were normal. Magnetic resonance imaging of the brain was performed with contrast. There was no space-occupying lesion in the brain or midline shift of the brain supratentorially or infratentorially. Radiographs and computed tomography (CT) images of the chest revealed multiple enlarged and confluent nodes (maximal dimension, >7 cm) at multiple compartments of the bilateral mediastinum, bilateral hilar region, and neck region. This finding was suggestive of sarcoidosis. The differential diagnosis included lymphoma (Fig. 4). Therefore, an ultrasound-guided biopsy of a cervical lymph node was performed. The histopathologic evaluation of the sample revealed granulo-matous tissue without caseous necrosis, which stained negatively with cytokeratin (CK). Therefore, a diagnosis of lymphoma was excluded (Fig. 5). The patient was diagnosed as having neuro-sarcoidosis, based on the presentation, clinical testing, and biopsy results. Treatment was initiated with corticosteroids. After retrobulbar injection of triamcinolone 1.5 mL (10 mg/mL) into the right eye and systemic treatment with oral prednisolone (15 mg 3 times daily; total, 45 mg/d) for 3 months, the patient's optic disc and macular lesions improved (Figs. 6 and 7). Approximately 1 month later, the VF defect resolved (Fig. 8).
Chest computed tomography reveals multiple enlarged nodes in multiple compartments of the bilateral mediastinum and the bilateral hilar region (red arrows).