Conceição I, Carvalho M, Alves M, Luís M L
Serviço de Neurologia, Centro de Estudos Egas Moniz, Hospital de Santa Maria, Lisboa.
Acta Med Port. 1993 Jan;6(1):15-7.
Sixteen (16) patients with Familial Amyloidotic Polyneuropathy-Portuguese Type (FAP) were evaluated longitudinally, using the EMG and Clinical protocols with the aim of establishing the clinical and neurophysiological evolution rate of FAP. We conclude that spontaneous evolution of FAP patients shows a linear progression. Clinical and neurophysiological evolution times are parallel, with the EMG scores being higher than Clinical ones. We propose that Clinical and EMG scores should be used in the evaluation of future therapeutic trials, namely immunodepuration and liver transplantation.
对16例葡萄牙型家族性淀粉样多神经病（FAP）患者进行了纵向评估，采用肌电图（EMG）和临床方案，目的是确定FAP的临床和神经生理演变率。我们得出结论，FAP患者的自然演变呈线性进展。临床和神经生理演变时间是平行的，EMG评分高于临床评分。我们建议在评估未来的治疗试验，即免疫净化和肝移植时，应使用临床和EMG评分。
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