The authors performed ambulatory electro-vectorcardiography polycardiography and echocardiography in 18 patients with typical Friedreich's disease, and 6 patients with atypical forms of hereditary spino-cerebellar ataxia classified on e basis of the degree of neurological involvement, without clinical signs of cardiocirculatory failure. The ECG and VCG recording commonly showed appearances suggestive of myocardial "necrosis" and were of little value in the differential diagnosis between typical and atypical forms of Friedreich's ataxia. This limitation also applied to the kinetocardiogramme which was sometimes pathological confirming the echocardiographic diagnosis of symmetric LV hypertrophy and of septal hypokinesia despite normal ECG and VCG.
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