Optic Neuropathy and Macular Ischemia Associated with Neurosarcoidosis: A Case Report

Abstract

In this study, we present a case of bilateral optic neuropathy and macular ischemia in the right eye associated with neurosarcoidosis. A 26-year-old woman presented to our clinic with complaints of bilateral blurred vision. Bilateral granulomatous anterior uveitis, vitritis, optic neuropathy, and macular ischemia were detected in the right eye in slit-lamp examination. She also reported complaints of fever, weakness, sweating, arthralgia, and headache for 2 months. She was referred to the pulmonary diseases unit of our hospital due to hilar lymphadenopathy seen in her chest x-ray, and biopsies were taken for diagnostic purposes. Histological analysis of the mediastinal lymph node biopsies revealed chronic, non-caseating, granulomatous inflammation. Furthermore, the patient was referred to a neurologist due to concomitant complaint of intense headaches. She was diagnosed with neurosarcoidosis supported by findings on cranial magnetic resonance imaging and lumbar puncture. She received a 3-day course of high-dose (1 g/day) intravenous steroid treatment (methylprednisolone) followed by a tapering dose of oral prednisone. The patient began receiving oral methotrexate 15 mg/week as a steroid-sparing agent. Significant improvement in neurological and ophthalmological symptoms occurred in the first week of treatment. In this case report, we emphasized that neurosarcoidosis should be included in the differential diagnosis of patients with both bilateral optic neuropathy and macular ischemia. Furthermore, early diagnosis and timely treatment of neurosarcoidosis are important for favorable visual outcomes.

Keywords: Macular ischemia; methotrexate; neurosarcoidosis; optic neuropathy.

Figure 1. Color fundus photograph of a 26-year-old female patient at initial presentation: (A) Optic disc edema, soft exudate in the inferotemporal part of the optic disc, and ischemia in the inferior half of the macula is seen in the right eye; (B) optic disc edema is seen in the left eye

Figure 2. Fundus fluorescein angiography image obtained 2 weeks after initial presentation: (A-D) Optic disc hyperfluorescence beginning in the early phase and increasing in the late phase is seen in both eyes; (D) a hyperfluorescent spot increasing in intensity in the late phase is seen in the superior aspect of the left fovea

Figure 3. Peripheral fundus fluorescein angiography image shows leakage increasing in the late phase in the retinal vessels in the (A) right and (B) left eyes

Figure 4. Fundus photograph taken 3 months after treatment: (A) Complete resolution of the optic disc edema and ischemia in the inferior macula is seen in the right eye; (B) Regression of the optic disc edema is seen in the left eye