Harding A E, Hewer R L
Q J Med. 1983 Autumn;52(208):489-502.
One hundred and fifteen patients with carefully defined Friedreich's ataxia were assessed clinically and electrocardiographically for evidence of heart disease. Cardiac symptoms, of which dyspnoea and palpitations were the most frequent, occurred in less than 30 per cent. Abnormalities on clinical examination were present in a similar proportion; harsh systolic murmurs, ventricular hypertrophy and added heart sounds were the commonest of these. Cardiac failure and persistent arrhythmias were rare and occurred late in the evolution of the neurological disease. Two patients presented with heart disease before developing neurological symptoms. Cardiac signs and symptoms were uncommon in patients without electrocardiographic abnormalities. About two-thirds of the cases had definitely abnormal ECG recordings. The characteristic finding was of widespread T-wave inversion with ventricular hypertrophy. Serial ECGs, recorded over periods of up to 32 years, were available in 30 cases and showed that abnormalities may develop in patients with Friedreich's ataxia at any time up until 20 years after the onset of neurological symptoms. In four patients initial ECG abnormalities had either improved or disappeared subsequently.
对115例经过严格定义的弗里德赖希共济失调患者进行了临床和心电图评估，以寻找心脏病证据。心脏症状中，呼吸困难和心悸最为常见，发生率不到30%。临床检查异常的比例与之相似；其中最常见的是粗糙的收缩期杂音、心室肥大和额外心音。心力衰竭和持续性心律失常很少见，且在神经系统疾病进展后期出现。两名患者在出现神经系统症状之前就有心脏病表现。在没有心电图异常的患者中，心脏体征和症状并不常见。约三分之二的病例心电图记录明确异常。特征性表现是广泛的T波倒置伴心室肥大。30例患者有长达32年的系列心电图记录，结果显示，弗里德赖希共济失调患者在神经系统症状出现后的20年内任何时候都可能出现异常。4例患者最初的心电图异常随后有所改善或消失。
