Hypertrophic cardiomyopathy in Freidreich ataxia: about two cases

Freidreich ataxia is the most frequent ataxia of early onset and of autosomal recessive transmission. It is associated with hypertrophic cardiomyopathy in 34 to 77% of cases. The purpose of this article is to describe the cardiac manifestations of two patients affected by this disease. The first case report is about a 34-years-old, bedridden male patient having muscular hypotony and osteotendinous areflexia of the lower limbs. Chest x-ray findings showed a cardiac silhouette distorted by scoliokyphosis. ECG revealed frequent auricular extrasystoles. Cardiac US examination disclosed a pattern of concentric hypertrophic cardiomyopathy with systolic and diastolic dysfonction.