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Case Reports
. 2010 Jan;30(3):389-93.
doi: 10.1007/s00296-009-0939-y. Epub 2009 May 8.

Post-inflammatory retinal dystrophy in CINCA syndrome

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Case Reports

Post-inflammatory retinal dystrophy in CINCA syndrome

Donato Rigante et al. Rheumatol Int. 2010 Jan.

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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