[Cardiac changes in hereditary spinocerebellar degenerations]

Echocardiographic alterations were compared with the clinical picture in 32 patients with different forms of hereditary spinocerebellar degenerations (HSCD). Three groups of patients were examined. Group I included patients with Friedreich's ataxia (FA), group II consisted of patients with familial cerebellar degeneration, and group III of those with sporadic cerebellar degeneration. Echographic alterations associated with FA were recorded in 71.4% of cases. Cardiomyopathy was confirmed to be a characteristic feature of FA. Echographic alterations in FA were noted to be pleomorphic: apart from typical hypertrophy of the myocardium, a considerable enlargement of the left ventricle was detectable more seldom. In familial cerebellar degeneration, different echocardiographic alterations were recorded in 81.8% of cases, whereas in sporadic cerebellar degeneration, in 78.6% of cases. Dilated cardiomyopathy was revealed in 3 cases (in patients belonging to groups II and III). It is assumed that cardiac pathology may be one of the extraneural manifestations not only in FA but also in other forms of HSCD.