Abstract
• Purpose: We evaluated patients with Wilson's disease to determine (1) whether presymptomatic patients who have Kayser-Fleischer (KF) rings demonstrate more significant copper imbalance than those who do not have KF rings, and (2) whether presymptomatic patients have smaller KF rings than symptomatic patients with Wilson's disease. • Methods: Thirty two patients with presymptomatic Wilson's disease were retrospectively analyzed. Sixteen of these had received no prior anti-copper therapy and underwent testing for baseline copper metabolism variables (24-hour urine copper, liver copper, and plasma ceruloplasmin). Quantitative measurements of KF rings were made for the group of untreated presymptomatic as well as for a control group of symptomatic Wilson's disease patients. • Results: No significant difference was found between baseline copper variables for presymptomatic patients who had KF rings compared to those who did not. However, KF rings of presymptomatic patients were found to be significantly smaller than KF rings of patients with symptomatic Wilson's disease. • Conclusions: This study suggests a parallel relationship between Kayser-Fleischer ring development and neurologic progression in Wilson's disease. These corneal rings are not only diagnostically useful, but may also be informative regarding the baseline prognosis for development of neurologic decline in presymptomatic patients with Wilson's disease.
| Original language | English (US) |
|---|---|
| Pages (from-to) | S316 |
| Journal | Investigative Ophthalmology and Visual Science |
| Volume | 37 |
| Issue number | 3 |
| State | Published - Feb 15 1996 |
ASJC Scopus subject areas
- Ophthalmology
- Sensory Systems
- Cellular and Molecular Neuroscience