Fading Kayser-Fleischer ring revisited

Abstract

Kayser-Fleischer (KF) ring, caused by deposition of excess copper in the Descemet membrane, is a characteristic ocular manifestation of Wilson disease (WD). Disappearance of KF rings following successful treatment of Wilson disease is typically a slow process that occurs over years. Herein, we describe a 19-year old girl who presented with neuropsychiatric manifestations and was found to have KF rings on slit lamp examination. Subsequent evaluation (brain imaging, liver function tests, serum ceruloplasmin and urinary copper studies) confirmed a diagnosis of Wilson disease with neurological and hepatic involvement. She was treated with d-penicillamine. She had remarkable fading of KF rings within a span of 6 months of copper-chelating therapy, which was also associated with significant improvement in her neurological symptoms. Though KF rings are a harbinger of neurological Wilson's, their disappearance does not always correlate with systemic improvement - an interesting finding in this case.

Keywords: KF ring; Wilson disease.