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Fading Kayser-Fleischer ring revisited.

Maansi Sethi1, Siddharth Madan1, Sarita Beri1

  • 1Department of Ophthalmology, Lady Hardinge Medical College, New Delhi, India.

Saudi Journal of Ophthalmology : Official Journal of the Saudi Ophthalmological Society
|April 8, 2022
PubMed
Summary
This summary is machine-generated.

Kayser-Fleischer rings, a sign of Wilson disease, faded rapidly in a 19-year-old patient within 6 months of copper-chelating therapy. This rapid improvement in ocular copper deposition correlated with neurological symptom recovery.

Keywords:
KF ringWilson disease

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Area of Science:

  • Ophthalmology
  • Neurology
  • Genetics

Background:

  • Wilson disease (WD) is an inherited disorder of copper metabolism.
  • Kayser-Fleischer (KF) rings, copper deposits in Descemet membrane, are a hallmark ocular sign of WD.
  • Typical resolution of KF rings post-treatment is a slow, multi-year process.

Observation:

  • A 19-year-old female presented with neuropsychiatric symptoms and KF rings.
  • Diagnostic workup confirmed Wilson disease with neurological and hepatic involvement.
  • The patient was treated with d-penicillamine, a copper-chelating agent.

Findings:

  • Remarkable fading of KF rings was observed within 6 months of therapy.
  • This rapid ocular improvement coincided with significant recovery of neurological symptoms.
  • The case highlights a faster-than-expected resolution of KF rings.

Implications:

  • The rapid disappearance of KF rings may indicate a positive treatment response in Wilson disease.
  • This case challenges the typical timeline for KF ring resolution.
  • Monitoring KF ring changes can offer insights into Wilson disease treatment efficacy and systemic recovery.