Right chorioretinitis and bilateral pseudopapilledema were firstly appreciated in a 9-month-old child with neonatal findings of aseptic chronic meningitis, framed in the context of CINCA syndrome at 1 year. Therapeutical response to various combinations of drugs was inconsistent until 7 years, when anakinra was started with immediate clinical and laboratory improvement. A state of severe retinal dystrophy of post-inflammatory origin became evident on funduscopy, optical coherence tomography and visual electrophysiology tests at the age of 10 years, which remained stationary after 1 year of anakinra treatment.
Rigante, D., Stabile, A., Minnella, A. M., Avallone, L., Ziccardi, L., Bersani, G., Stifano, G., Compagnone, A., Falsini, B., Post-inflammatory retinal dystrophy in CINCA syndrome., <<RHEUMATOLOGY INTERNATIONAL>>, 2010; 2010 (30(3)): 389-393 [http://hdl.handle.net/10807/3682]
Post-inflammatory retinal dystrophy in CINCA syndrome.
Rigante, Donato;Stabile, Achille;Minnella, Angelo Maria;Avallone, Laura;Ziccardi, Lucia;Bersani, Giulia;Stifano, Giovanna;Falsini, Benedetto
2010
Abstract
Right chorioretinitis and bilateral pseudopapilledema were firstly appreciated in a 9-month-old child with neonatal findings of aseptic chronic meningitis, framed in the context of CINCA syndrome at 1 year. Therapeutical response to various combinations of drugs was inconsistent until 7 years, when anakinra was started with immediate clinical and laboratory improvement. A state of severe retinal dystrophy of post-inflammatory origin became evident on funduscopy, optical coherence tomography and visual electrophysiology tests at the age of 10 years, which remained stationary after 1 year of anakinra treatment.
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