Kraiem Sondos, Abbassi Chedli, Kammoun Sofiane, Tellili Somaya, Hmem Mohamed, Smaali Ibtissem, Khaldi Mohamed Habib, Boudiche Slim, Mejri Mourad, Mokline Amel, Kaies Battikh, Longo Selma, Slimane Mohamed Lotfi
Service Cardiologie Hôpital Habib Thameur, Tunis.
Tunis Med. 2006 Apr;84(4):248-52.
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.
