The pseudopapilledema of neonatal-onset multisystem inflammatory disease

To report a rare case of neonatal-onset multisystem inflammatory disease with serial photographs to characterize the optic nerve findings. Observational case report. A 6-year-old girl with neonatal-onset multisystem inflammatory disease, who had received systemic corticosteroid therapy for 5 years, had bilateral fibrillar opacities that surrounded the optic disks and extended into the peripapillary nerve fiber layer and vessels. A magnetic resonance imaging examination and lumbar puncture revealed elevated intracranial pressure. The elevated intracranial pressure returned to normal following a corticosteroid taper. Optic disk photographs, taken 4 years earlier, were subsequently obtained. The optic disk appearance had remained unchanged over the 4-year period, consistent with a pseudopapilledema. The optic disk appearance is not consistent with papilledema from increased intracranial pressure. The optic disk findings, in conjunction with the underlying inflammatory syndrome, suggest an infiltrative etiology for the atypical optic nerve findings in neonatal-onset multisystem inflammatory disease.