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J Neurol ; 256(9): 1434-7, 2009 Sep.
Article in English | MEDLINE | ID: mdl-19363628

ABSTRACT

Left ventricular hypertrophy (LVH) is a frequent finding in Friedreich's ataxia (FRDA). In previous studies treatment with idebenone, a synthetic analogue of coenzyme Q10, has been associated with a substantial decrease in myocardial hypertrophy, despite great variability in cardiac responsiveness among patients. Here we present the results of a retrospective analysis of a cohort of 35 patients (20 with LVH, 15 without LVH) with confirmed molecular diagnosis of FRDA, treated with idebenone 5 mg/kg/day for up to five years. At the end of the study, we found an increase of interventricular septum and posterior wall thickness in the group without LVH before treatment and no change in the group with LVH before treatment. The neurological picture of the disease significantly deteriorated with time in both groups.


Subject(s)
Antioxidants/therapeutic use , Friedreich Ataxia/drug therapy , Hypertrophy, Left Ventricular/drug therapy , Ubiquinone/analogs & derivatives , Adult , Analysis of Variance , Cohort Studies , Echocardiography , Female , Follow-Up Studies , Friedreich Ataxia/complications , Friedreich Ataxia/pathology , Heart/drug effects , Heart Septum/diagnostic imaging , Heart Septum/drug effects , Heart Septum/pathology , Humans , Hypertrophy, Left Ventricular/complications , Hypertrophy, Left Ventricular/pathology , Male , Myocardium/pathology , Retrospective Studies , Severity of Illness Index , Ubiquinone/therapeutic use
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