Kearns-Sayre syndrome with a phenocopy of choroideremia instead of pigmentary retinopathy.

Herzberg, N H; van Schooneveld, M J; Bleeker-Wagemakers, E M; Zwart, R; Cremers, F P; van der Knaap, M S; Bolhuis, P A; de Visser, M · Neurology · 1993

case_report · Level V

Where this comes from

Abstract

Mitochondrial DNA (mtDNA) was deleted in a patient with Kearns-Sayre syndrome (KSS) presenting with a choroideremia-like fundus picture instead of pigmentary retinopathy. No evidence for X-linked choroideremia was present, and because of the strong association between KSS and deleted mtDNA, we suggest that choroideremia is a phenocopy and can be part of KSS.

Medical subject headings