Idiopathic sclerosing orbital inflammation: Presentation of an unusual case with isolated bilateral optic nerve involvement

摘要

We read with great interest the report by Levin et al (1) regarding the role of the optic nerve biopsy in the management of progressive optic neuropathy. We had the opportunity to evaluate a 50-year-old man with progressive deterioration of vision in his left eye for 2 months with minimal periocular pain. Visual acuity was 20/20, right eye, and no light perception, left eye. The right fundus was normal but there was marked optic disc edema in the left eye (Fig. 1). Magnetic resonance imaging (MRI) of the brain and orbits demonstrated enhancement and thickening of the left optic nerve (Fig. 2). An extensive work-up including hematologic tests, lumbar puncture, and computed tomography of chest, abdomen, and pelvis was unremarkable. The patient was given 1 g of methylprednisolone intravenously for 3 days followed by a tapering dose of oral steroids over several months. Although there was improvement in the left optic disc edema, vision remained unchanged. Four months after onset of symptoms, the patient reported vision loss in the right eye. Acuity was 20/40, right eye, and no light perception, left eye. Funduscopy revealed right optic disc edema and left optic atrophy. MRI demonstrated thickening of the right optic with marked enhancement following intravenous contrast. Prednisone was restarted at a dose of 1 mg/kg/d, and there was rapid improvement in the vision in the right eye with resolution of right optic disc edema.