The pseudopapilledema of neonatal-onset multisystem inflammatory disease.
OBJECTIVE
To report a rare case of neonatal-onset multisystem inflammatory disease with serial photographs to characterize the optic nerve findings.


METHODS
Observational case report.


METHODS
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.


RESULTS
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.


CONCLUSIONS
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.
Hollander
David A
DA
Department of Ophthalmology, The University of California, San Francisco, San Francisco, California, USA.
Hoyt
William F
WF
Howes
Edward L
EL
Fredrick
Douglas R
DR
D017668:Age of Onset; D001327:Autoimmune Diseases / Q000150:complications* / Q000188:drug therapy; D002648:Child; D005260:Female; D005938:Glucocorticoids / Q000009:adverse effects / Q000627:therapeutic use; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007249:Inflammation / Q000150:complications* / Q000188:drug therapy; D019586:Intracranial Hypertension / Q000139:chemically induced; D008279:Magnetic Resonance Imaging; D008727:Methotrexate / Q000627:therapeutic use; D009412:Nerve Fibers / Q000473:pathology*; D009898:Optic Disk / Q000473:pathology*; D010211:Papilledema / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology*; D010781:Photography; D011239:Prednisolone / Q000009:adverse effects / Q000627:therapeutic use
D005938:Glucocorticoids; D007166:Immunosuppressive Agents; D011239:Prednisolone; D008727:Methotrexate