Buyse G, Mertens L, Di Salvo G, Matthijs I,
Weidemann F,
Eyskens B, Goossens W, Goemans N, Sutherland GR, Van Hove JL.
Department of Pediatric Neurology, University Hospitals Leuven, Belgium.
Gunnar.Buyse@uz.kuleuven.ac.be
Neurology. 2003 May 27;60(10):1679-81.
Abstract
The authors report 1-year prospective data on
eight patients with Friedreich ataxia. Idebenone did not halt the progression
of ataxia. At the end of therapy, cardiac ultrasound demonstrated
significant reduction of cardiac hypertrophy in six of eight patients.
Cardiac strain and strain rate imaging showed that the reduction of
hypertrophy is preceded by an early and linear improvement in cardiac
function. Idebenone reduced erythrocyte protoporphyrin IX levels in five of
six patients with elevated baseline levels; however, changes did not
consistently relate to cardiac improvement.