[Cardiovascular changes in a group of patients with heredoataxia. Clinical, electrocardiographic and echocardiographic findings].

INTRODUCTION: Heredoataxias form a group of degenerative diseases of the nervous system, which are progressive and hand sound in particular families. MATERIAL AND METHODS: Between August 1994 and September 1995. 14 patients diagnosed as having heredoataxia were studied after admission to our hospital. Eight of these had Friedrich's ataxia (group A) and 6 had other types of ataxia (group B). A standard set of investigations were done, including clinical examination, laboratory tests (such as glycaemia and a lipidogram) to evaluate coronary risk factors. A 12 lead electrocardiogram and bidimensional and m mode echocardiogram were also done to detect disorders of conduction, changes in morphology and cardiac function, together with functional cardiovascular and neurological scales. The patients studied were between 14 and 41 years of age. RESULTS: The most frequent cardiovascular symptom was dyspnoea of effort. Changes on the electrocardiogram were seen in 92% of the patients, mainly from Group A (100%) and were due to alterations of ventricular repolarization and non-specific alterations of conduction and of rhythm. Echocardiogram changes were found in 42.8% of the patients, most frequently due to an increase in the myocardial mass of the left ventricle. CONCLUSIONS: Close correlation between age of onset and duration of the illness, and functional cardiovascular impairment was only seen in patients of Group A, in whom there was an earlier onset of the condition.