Retinal Vasculitis as an Initial Presentation of Atypical Neurosarcoidosis With Occult Central Nervous System Inflammation

<b>Purpose:</b> To describe a case of retinal vasculitis as a presenting sign of atypical neurosarcoidosis with occult central nervous system involvement. <b>Methods:</b> A case report and literature review are presented, highlighting the role of the ophthalmic examination and the importance of early neurologic workup for diagnosis and treatment. <b>Results:</b> A 27-year-old woman presented with monocular blurry vision, central scotoma, and headache. Ophthalmic examination demonstrated retinal vasculitis bilaterally, and systemic steroid treatment was initiated. Further neurologic workup with brain magnetic resonance imaging revealed multiple enhancing foci, consistent with features of inflammation. The neurologic disease was recalcitrant, showing no response to multiple steroid-sparing therapies over 2 years. Further workup was pursued, including a brain biopsy showing noncaseating granulomas with small-vessel vasculitis. The ophthalmic and neurologic presentation was consistent with a diagnosis of atypical neurosarcoidosis. Clinical resolution was ultimately achieved after treatment with infliximab. <b>Conclusions:</b> Retinal vasculitis should have a low threshold for initiating early neurologic workup to assess central nervous system involvement.